Acute Poststreptococcal Glomerulonephritis

33
ACUTE NEPHRITIC SYNDROME By Hakimah Khani Binti Suhaimi

Transcript of Acute Poststreptococcal Glomerulonephritis

Page 1: Acute Poststreptococcal Glomerulonephritis

ACUTE NEPHRITIC SYNDROME

By Hakimah Khani Binti Suhaimi

Introduction

bull Synonyms acute nephritis acute nephritic

syndromebull An immunologic mechanism the

result of an immune process that injures the glomeruli of the kidney

bull Clinical features ndash A sudden onset of hematuria ndash Proteinuriandash Edema ndash Oliguria and volume overloadndash Hypertension ndash Azotemia is another common but

inconstant finding

Glomerulo

nephritis

Acute Nephritic syndrome

Acute post strep GN

IgA Nephropath

y

MembranoProliferative

Henoch Schonlein Purpura

Alport Syndrome

SLE nephritis

Other post infectious glomerulonephritis

Nephrotic Syndrome

ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS

Outline

bull Definitionbull Epidemiologybull Etiology and Pathogenesisbull Clinical Featuresbull Complicationbull Investigationbull Diagnosisbull Differential Diagnosisbull Treatment and Managementbull Prognosis

Definition

AGN that follows an infection with a nephritogenic strain of group A beta hemolytic streptococci

The classic example of the acute nephritic syndrome

Nelson Textbook of Pediatrics 7th Edition

Epidemiologybull 121 of the 124 patients had

poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988

bull Globally - incidence has decreased in the past three decades

bull Most commonly ndash sporadicbull Despite that epidemics and clusters of

cases - in some poor or rural communitiescopy 2008 American Society of Nephrology

bull Peak incidence - age 5-12 yo uncommon lt3yo

bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition

Etiology and Pathogenesis

bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1

bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation

bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)

Streptococcal infection

immune complex formation + deposited in GBM

complement system activated

immune injuries

cellular proliferation GBM fracture

capillary lumen narrowed hematuria

glomerular blood flow decreased proteinuria

oliguria GFR distal sodium reabsorption

retention of water amp sodium

blood volume

edema hypertension

Low serum complement

CLINICAL FEATURES

Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma

1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)

2 Proteinuria ndash usu normalize after 4 weeks

3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday

Typical Manifestation (2)3 Gross hematuria (65 of

patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically

4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis

5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 2: Acute Poststreptococcal Glomerulonephritis

Introduction

bull Synonyms acute nephritis acute nephritic

syndromebull An immunologic mechanism the

result of an immune process that injures the glomeruli of the kidney

bull Clinical features ndash A sudden onset of hematuria ndash Proteinuriandash Edema ndash Oliguria and volume overloadndash Hypertension ndash Azotemia is another common but

inconstant finding

Glomerulo

nephritis

Acute Nephritic syndrome

Acute post strep GN

IgA Nephropath

y

MembranoProliferative

Henoch Schonlein Purpura

Alport Syndrome

SLE nephritis

Other post infectious glomerulonephritis

Nephrotic Syndrome

ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS

Outline

bull Definitionbull Epidemiologybull Etiology and Pathogenesisbull Clinical Featuresbull Complicationbull Investigationbull Diagnosisbull Differential Diagnosisbull Treatment and Managementbull Prognosis

Definition

AGN that follows an infection with a nephritogenic strain of group A beta hemolytic streptococci

The classic example of the acute nephritic syndrome

Nelson Textbook of Pediatrics 7th Edition

Epidemiologybull 121 of the 124 patients had

poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988

bull Globally - incidence has decreased in the past three decades

bull Most commonly ndash sporadicbull Despite that epidemics and clusters of

cases - in some poor or rural communitiescopy 2008 American Society of Nephrology

bull Peak incidence - age 5-12 yo uncommon lt3yo

bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition

Etiology and Pathogenesis

bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1

bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation

bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)

Streptococcal infection

immune complex formation + deposited in GBM

complement system activated

immune injuries

cellular proliferation GBM fracture

capillary lumen narrowed hematuria

glomerular blood flow decreased proteinuria

oliguria GFR distal sodium reabsorption

retention of water amp sodium

blood volume

edema hypertension

Low serum complement

CLINICAL FEATURES

Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma

1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)

2 Proteinuria ndash usu normalize after 4 weeks

3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday

Typical Manifestation (2)3 Gross hematuria (65 of

patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically

4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis

5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 3: Acute Poststreptococcal Glomerulonephritis

Glomerulo

nephritis

Acute Nephritic syndrome

Acute post strep GN

IgA Nephropath

y

MembranoProliferative

Henoch Schonlein Purpura

Alport Syndrome

SLE nephritis

Other post infectious glomerulonephritis

Nephrotic Syndrome

ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS

Outline

bull Definitionbull Epidemiologybull Etiology and Pathogenesisbull Clinical Featuresbull Complicationbull Investigationbull Diagnosisbull Differential Diagnosisbull Treatment and Managementbull Prognosis

Definition

AGN that follows an infection with a nephritogenic strain of group A beta hemolytic streptococci

The classic example of the acute nephritic syndrome

Nelson Textbook of Pediatrics 7th Edition

Epidemiologybull 121 of the 124 patients had

poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988

bull Globally - incidence has decreased in the past three decades

bull Most commonly ndash sporadicbull Despite that epidemics and clusters of

cases - in some poor or rural communitiescopy 2008 American Society of Nephrology

bull Peak incidence - age 5-12 yo uncommon lt3yo

bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition

Etiology and Pathogenesis

bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1

bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation

bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)

Streptococcal infection

immune complex formation + deposited in GBM

complement system activated

immune injuries

cellular proliferation GBM fracture

capillary lumen narrowed hematuria

glomerular blood flow decreased proteinuria

oliguria GFR distal sodium reabsorption

retention of water amp sodium

blood volume

edema hypertension

Low serum complement

CLINICAL FEATURES

Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma

1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)

2 Proteinuria ndash usu normalize after 4 weeks

3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday

Typical Manifestation (2)3 Gross hematuria (65 of

patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically

4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis

5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 4: Acute Poststreptococcal Glomerulonephritis

ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS

Outline

bull Definitionbull Epidemiologybull Etiology and Pathogenesisbull Clinical Featuresbull Complicationbull Investigationbull Diagnosisbull Differential Diagnosisbull Treatment and Managementbull Prognosis

Definition

AGN that follows an infection with a nephritogenic strain of group A beta hemolytic streptococci

The classic example of the acute nephritic syndrome

Nelson Textbook of Pediatrics 7th Edition

Epidemiologybull 121 of the 124 patients had

poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988

bull Globally - incidence has decreased in the past three decades

bull Most commonly ndash sporadicbull Despite that epidemics and clusters of

cases - in some poor or rural communitiescopy 2008 American Society of Nephrology

bull Peak incidence - age 5-12 yo uncommon lt3yo

bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition

Etiology and Pathogenesis

bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1

bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation

bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)

Streptococcal infection

immune complex formation + deposited in GBM

complement system activated

immune injuries

cellular proliferation GBM fracture

capillary lumen narrowed hematuria

glomerular blood flow decreased proteinuria

oliguria GFR distal sodium reabsorption

retention of water amp sodium

blood volume

edema hypertension

Low serum complement

CLINICAL FEATURES

Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma

1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)

2 Proteinuria ndash usu normalize after 4 weeks

3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday

Typical Manifestation (2)3 Gross hematuria (65 of

patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically

4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis

5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 5: Acute Poststreptococcal Glomerulonephritis

Outline

bull Definitionbull Epidemiologybull Etiology and Pathogenesisbull Clinical Featuresbull Complicationbull Investigationbull Diagnosisbull Differential Diagnosisbull Treatment and Managementbull Prognosis

Definition

AGN that follows an infection with a nephritogenic strain of group A beta hemolytic streptococci

The classic example of the acute nephritic syndrome

Nelson Textbook of Pediatrics 7th Edition

Epidemiologybull 121 of the 124 patients had

poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988

bull Globally - incidence has decreased in the past three decades

bull Most commonly ndash sporadicbull Despite that epidemics and clusters of

cases - in some poor or rural communitiescopy 2008 American Society of Nephrology

bull Peak incidence - age 5-12 yo uncommon lt3yo

bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition

Etiology and Pathogenesis

bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1

bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation

bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)

Streptococcal infection

immune complex formation + deposited in GBM

complement system activated

immune injuries

cellular proliferation GBM fracture

capillary lumen narrowed hematuria

glomerular blood flow decreased proteinuria

oliguria GFR distal sodium reabsorption

retention of water amp sodium

blood volume

edema hypertension

Low serum complement

CLINICAL FEATURES

Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma

1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)

2 Proteinuria ndash usu normalize after 4 weeks

3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday

Typical Manifestation (2)3 Gross hematuria (65 of

patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically

4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis

5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 6: Acute Poststreptococcal Glomerulonephritis

Definition

AGN that follows an infection with a nephritogenic strain of group A beta hemolytic streptococci

The classic example of the acute nephritic syndrome

Nelson Textbook of Pediatrics 7th Edition

Epidemiologybull 121 of the 124 patients had

poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988

bull Globally - incidence has decreased in the past three decades

bull Most commonly ndash sporadicbull Despite that epidemics and clusters of

cases - in some poor or rural communitiescopy 2008 American Society of Nephrology

bull Peak incidence - age 5-12 yo uncommon lt3yo

bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition

Etiology and Pathogenesis

bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1

bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation

bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)

Streptococcal infection

immune complex formation + deposited in GBM

complement system activated

immune injuries

cellular proliferation GBM fracture

capillary lumen narrowed hematuria

glomerular blood flow decreased proteinuria

oliguria GFR distal sodium reabsorption

retention of water amp sodium

blood volume

edema hypertension

Low serum complement

CLINICAL FEATURES

Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma

1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)

2 Proteinuria ndash usu normalize after 4 weeks

3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday

Typical Manifestation (2)3 Gross hematuria (65 of

patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically

4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis

5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 7: Acute Poststreptococcal Glomerulonephritis

Epidemiologybull 121 of the 124 patients had

poststreptococcal nephritis Department of Pediatrics HUSM July 1987- June 1988

bull Globally - incidence has decreased in the past three decades

bull Most commonly ndash sporadicbull Despite that epidemics and clusters of

cases - in some poor or rural communitiescopy 2008 American Society of Nephrology

bull Peak incidence - age 5-12 yo uncommon lt3yo

bull Male female ratio is 2 1Nelson Textbook of Pediatrics 7th Edition

Etiology and Pathogenesis

bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1

bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation

bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)

Streptococcal infection

immune complex formation + deposited in GBM

complement system activated

immune injuries

cellular proliferation GBM fracture

capillary lumen narrowed hematuria

glomerular blood flow decreased proteinuria

oliguria GFR distal sodium reabsorption

retention of water amp sodium

blood volume

edema hypertension

Low serum complement

CLINICAL FEATURES

Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma

1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)

2 Proteinuria ndash usu normalize after 4 weeks

3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday

Typical Manifestation (2)3 Gross hematuria (65 of

patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically

4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis

5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 8: Acute Poststreptococcal Glomerulonephritis

Etiology and Pathogenesis

bull The child gets gets throat or skin infection by nephritogenic strain of group A beta hemolytic streptococci - serotype 12 4 and 1

bull Antibodies to streptoccocus (eg antistreptolysin O) are formed in his circulation

bull Antigen-antibody circulating immune complexes are subsequently deposited along the glomerular basement membrane (GBM)

Streptococcal infection

immune complex formation + deposited in GBM

complement system activated

immune injuries

cellular proliferation GBM fracture

capillary lumen narrowed hematuria

glomerular blood flow decreased proteinuria

oliguria GFR distal sodium reabsorption

retention of water amp sodium

blood volume

edema hypertension

Low serum complement

CLINICAL FEATURES

Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma

1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)

2 Proteinuria ndash usu normalize after 4 weeks

3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday

Typical Manifestation (2)3 Gross hematuria (65 of

patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically

4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis

5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 9: Acute Poststreptococcal Glomerulonephritis

Streptococcal infection

immune complex formation + deposited in GBM

complement system activated

immune injuries

cellular proliferation GBM fracture

capillary lumen narrowed hematuria

glomerular blood flow decreased proteinuria

oliguria GFR distal sodium reabsorption

retention of water amp sodium

blood volume

edema hypertension

Low serum complement

CLINICAL FEATURES

Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma

1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)

2 Proteinuria ndash usu normalize after 4 weeks

3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday

Typical Manifestation (2)3 Gross hematuria (65 of

patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically

4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis

5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 10: Acute Poststreptococcal Glomerulonephritis

CLINICAL FEATURES

Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma

1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)

2 Proteinuria ndash usu normalize after 4 weeks

3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday

Typical Manifestation (2)3 Gross hematuria (65 of

patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically

4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis

5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 11: Acute Poststreptococcal Glomerulonephritis

Typical Manifestation Develop acute nephritic syndrome 1ndash2 wk after an antecedent streptococcal pharyngitis or 3ndash6 wk after a streptococcal pyoderma

1 Edema75 of the patientsFace periorbital area lower extremities generalized (ascites pleural effusions)

2 Proteinuria ndash usu normalize after 4 weeks

3 Oliguriaschool child lt 400mldaypreschool child lt 300mldayinfant amp toddler lt 200mlday

Typical Manifestation (2)3 Gross hematuria (65 of

patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically

4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis

5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 12: Acute Poststreptococcal Glomerulonephritis

Typical Manifestation (2)3 Gross hematuria (65 of

patients)Smoky tea-colored cola-colored or fresh bloody urineMicroscopical hematuria (almost all patients)The urine appears normal but gt3 RBCsHP are found in centrifuged urine sediment examined microscopically

4 Hypertension (50) ndash mild to moderate typically subsides promptly after diuresis

5 Nonspecific symptoms Such as anorexia vomiting general malaise lethargy abdominal or flank pain low-grade fever and weight gain

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 13: Acute Poststreptococcal Glomerulonephritis

Clinical course

Spontaneous improvement

typically begins within 1 wk with

resolution of edema in 5-10 days

and hypertension in 2-3 wk but

urinalysis may be abnormal

(persistent microscopic

hematuria) for a year

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 14: Acute Poststreptococcal Glomerulonephritis

Complications in severe cases

bull Circulatory hypervolemia Congestive heart failure

bull Encephalopathy

bull Acute renal failure

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 15: Acute Poststreptococcal Glomerulonephritis

Laboratory Investigations1 Urinalysis2 Bacteriological and Serological

test3 Renal function test4 Full blood count5 Serum complement levels6 Kidney ultrasound

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 16: Acute Poststreptococcal Glomerulonephritis

Laboratory Investigations

Urinalysisbull Macroscopic hematuria Rusty or tea-colorbull Microscopy leukocytes red blood cell casts

(pathognomonic) and granular casts bull Proteinuria 2+ (Nephrotic-range proteinuria

occurs in lt5 of patients) bull Pyuria The urine contains large amounts of

fibrin degradation products and fibrinopeptides

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 17: Acute Poststreptococcal Glomerulonephritis

Laboratory Investigations

bull Evidences of streptococcal infection ndash Throat or skin cultures ndash Antistreptolysin O (ASO) titer Pharyngitis (80)

skin infections (lt50) ndash Anti-deoxyribonuclease (DNase) B level

Pharyngitis (98) skin infections (80) bull Renal function Test

ndash The BUN concentration is elevated in 75 of patients and serum creatinine level is increased in one half of the patients but profound decrease in GFR is uncommon in children

ndash Hyperkalemia hypocalcaemia hyponatremia and metabolic acidosis are seen only in severe patients

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 18: Acute Poststreptococcal Glomerulonephritis

Laboratory Investigations

bull Full Blood Countndash A mild normochromic anemia may be

present from hemodilution and low-grade hemolysis

ndash Leococytosis maybe present

bull Activation of complementsndash Serum C3 level decrease (90) return to

normal within 6 weeks ndash Serum C4 levels are typically normal

bull Kidney ultrasoundndash Not necessary if patient has clear cut acute

nephritic syndrome

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 19: Acute Poststreptococcal Glomerulonephritis

Renal Biopsy

bull Patients whose clinical presentation laboratory findings or course is atypical

bull Delay resolutiono oliguria gt 2 weekso Azotaemia gt 3 weekso Gross haematuria gt 3 weekso Persistent proteinuria gt 6 months

Paediatric Protocols 12th Edition

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 20: Acute Poststreptococcal Glomerulonephritis

Light microscopeNot specific for post streptococcal nephritis

bullGlomeruli appear enlarged and hypercellular bullDiffuse mesangial cell proliferation with an increase in mesangial matrix

bullPolymorphonuclear leukocytes are common in glomeruli during the early stage of the disease

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 21: Acute Poststreptococcal Glomerulonephritis

Electron microscope

bullSubepithelial electron-dense deposits or ldquohumpsrdquo are present which are observed on the epithelial side of the glomerular basement membrane (GBM)

bullGaps or discontinuities of GBM which is likely indicative of proteinuria and hematuria

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 22: Acute Poststreptococcal Glomerulonephritis

Immunofluorescence microscopy

bullLumpy-bumpy deposits of immunoglobulin G and complement 3 along the capillary loops and within the mesangium

bullIt is helpful in the differential diagnosis of other entities that may mimic APSGN clinically particularly IgA nephropathy

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 23: Acute Poststreptococcal Glomerulonephritis

Diagnosis

bull Acute onsetbull Symptoms edema oliguria dark

urine hypertensionbull Urinalysis RBCs protein castsbull Evidences of streptococcal

infectionndash Prodromesndash Elavated serum titers of Abs to

streptozymes(ASO)bull Serum C3 - Reduced

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 24: Acute Poststreptococcal Glomerulonephritis

Differential DiagnosisPoints to support Points against

Membrano-proliferative GN

a) Nephritic syndrome

b) Elevated ASO titer

c) Hypocomplement-emia

a) Persistent nephritic syndromeb) Hypocomplementemia (C3) -

gt6-8wc) Marked reduced renal fnd) Dx ndash by renal biopsy ndash ldquotram-

trackingrdquo GBM

IgA nephropathy (Bergerrsquos disease)

a) Hematuriab) Associated with

respiratory illness (following viral syndromes)

a) Recurrentb) No period of latencyc) Hypertension amp edema ndash

uncommond) ASO ndash not elevatede) uarr Serum IgA (15)f) Normal serum complement

valuesg) Focal proliferation diffuse

mesangial IgA deposits

Henoch- Schoumlnlein Purpura

a) Hematuria (80)

b) Mild proteinuriac) Preceding by

URTI (only 50)

a) Characteristic skin rashb) Assoc symptoms Abdominal

pain athritis athralgiac) Normal serum complement

valuescopy Hakimah

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 25: Acute Poststreptococcal Glomerulonephritis

Differential DiagnosisPoints to support Points against

Familial nephritis (Alport Syndrome)

a) Nephritic syndromeb) 1-2 days following

URTI

a) Lack of expected complete resolution

b) Progressive proteinuria after 2nd decade of life

c) Association with non-renal manifestations hearing deficits amp ocular abnormalities

SLE nephritis

a) Occurs in 30ndash70 of children

b) Hematuriac) Mild proteinuriad) Hypocomplement-

emia (C3)

a) Lack of expected complete resolution

b) Association with non-renal manifestations

c) C4 also depressedd) Detection of anti-nuclear

antibodies

Other chronic infections

a) Acute nephritic syndrome

b) Similar histopathologic findings

c) Hypocomplementemia

a) Missing evidence of a prior streptococcal infection

b) Treatment is unresponsive

copy Hakimah

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 26: Acute Poststreptococcal Glomerulonephritis

Treatment

bull Treatment of APSGN is largely that of supportive care

bull Usually patients undergo a spontaneous diuresis within 7 to 10 days after the onset of their illness - strict monitoring ndash nephrotic chart + fluid restriction until diuresis

bull Management is directed at treating the acute effects of renal insufficiency and hypertension

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 27: Acute Poststreptococcal Glomerulonephritis

bull Diuretics

bull DietFluid restriction ndash during oliguric phaseSodium restriction is necessaryProtein restriction is unnecessary

bull AntibioticsA 10-day course of systemic antibiotic therapy with penicillin V is recommended to limit the spread of the nephritogenic organismsAntibiotic therapy does not affect the natural history of glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 28: Acute Poststreptococcal Glomerulonephritis

Treatment for complications

Bed rest

Recheck BP frac12 hour later

Oral nifedipine

Add

Furosemide

Other oral AHT

Significant asymptomatic hypertension

Look for signs and symptoms

Emergency management

indicated

Target BP control

Reduce BP by 25 of target BP

over 3-12 hrs

Next reduction of 75 over 48

hrs

symptomatic severe hypertension or hypertensive emergency encephalopathy

Paediatric Protocols 12th Edition

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 29: Acute Poststreptococcal Glomerulonephritis

Paediatric Protocols 12th Edition

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 30: Acute Poststreptococcal Glomerulonephritis

Treatment for complications

-Give O2

-prop patient up

-ventilatory support if necessary

IV

furosemide

Fluid restriction

-withhold fluid for 24 Hrs if possiible

Consider dialysis if no response to diuretics

Acute pulmon

ary edema

Paediatric Protocols 12th Edition

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 31: Acute Poststreptococcal Glomerulonephritis

Treatment for complications

Monitor BP amp managing

hypertension

Limiting fluid intake in

oliguric state

Correcting electrolyte imbalance

ie life threatening hyperkalemi

a

Optimizing nutritional intake

(since ARF pt usually hypercatabolic)

Implementing acute dialysis

Acute Renal Failure

Paediatric Protocols 12th Edition

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 32: Acute Poststreptococcal Glomerulonephritis

Follow-up

bull for at least 1 yearbull monitor BP at every visitbull do urinalysis and renal func1048991 onto evaluate recoverybull repeat C3 levels 6 weeks later if

not already normalised by time of discharge

Paediatric Protocols 12th Edition

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis
Page 33: Acute Poststreptococcal Glomerulonephritis

Prognosis

bull short term outcome excellent mortality lt05

bull long term outcome 18 of children develop chronic kidney disease

bull following post streptococcal AGN These children should be referred to the paediatric nephrologists for further evaluation and management

Paediatric Protocols 12th Edition

  • ACUTE NEPHRITIC SYNDROME
  • Introduction
  • Slide 3
  • ACUTE POST-STREPTOCOCCAL GLOMERULONEPHRITIS
  • Outline
  • Definition
  • Epidemiology
  • Etiology and Pathogenesis
  • Slide 9
  • Clinical Features
  • Typical Manifestation
  • Typical Manifestation (2)
  • Slide 13
  • Complications in severe cases
  • Laboratory Investigations
  • Laboratory Investigations (2)
  • Laboratory Investigations (3)
  • Laboratory Investigations (4)
  • Renal Biopsy
  • Light microscope Not specific for post streptococcal nephritis
  • Electron microscope
  • Immunofluorescence microscopy
  • Diagnosis
  • Differential Diagnosis
  • Slide 25
  • Treatment
  • Slide 27
  • Treatment for complications
  • Slide 29
  • Treatment for complications (2)
  • Treatment for complications (3)
  • Follow-up
  • Prognosis