Glomerular Syndromes PK
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Transcript of Glomerular Syndromes PK
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Pathology of Common
GLOMERULAR SYNDROMES
Dr Purushotham krishnappa
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Objectives
To learn basic renal terminologies
Understand the common clinical featuresof common glomerular diseases Nephrotic syndrome
Nephritic syndrome
Rapidly progressive glomerulonephritis
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Basic terminology and concepts
Azotemia
Biochemical abnormality
Raised blood urea nitrogen and creatinineDecreased GFR
The cause may be:RenalExtrarenalPre-renal: hypoperfusion
Post-renal: obstruction
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Basic terminology and concepts
Uraemia
Azotaemia plusclinical signs and symptomsMetabolicEndocrineGastrointestinal uraemic gastroenteritis
Peripheral nerves peripheral neuropathyHeart uraemic fibrinous pericarditis.
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Clinical syndromes
Clinical syndromes are convenient starting pointstoidentify and evaluate kidney diseases.
Syndromes related to injury to the glomerular capillary
wall are quitespecificfor glomerular diseases.
Clinical presentations of diseases of tubules, interstitiumand blood vessels are less specific.
They often present as non-specific acute or chronicrenal insufficiency.
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Clinical syndromes
Point to remember:
Some diseases may have more than 1 clinicalpresentation with different syndromes or a mixture ofsyndromes, e.g. SLETherefore, clinical syndromes alone may not besufficient.
A renal biopsy - based diagnosis may be required.
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Clinical syndromes forglomerular diseases
1. Nephrotic syndrome
2. Acute nephritis3. Asymptomatic haematuria
4. Asymptomatic proteinuria.
5. Rapidly progressive glomerulonephritis6. Acute renal failure
7. Chronic renal failure
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Nephrotic Syndrome
1. Heavy proteinuria(Nephrotic range ismore than 3.5 gm /day)
2. Hypoalbuminaemia(less than 3 gm/dl)
3. Severe oedema-generalised4. Hyperlipidemia
Classic example: minimal change disease
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Causes of Nephrotic Syndrome
Primary Glomerular Disease
Membranous glomerulopathy - Adults Minimal change disease - Children Focal segmental glomerulosclerosis Membranoproliferative glomerulonephritides Other proliferative glomerulonephritis (focal, "pure mesangial," IgA
nephropathy)System ic Diseases
Diabetes mellitus Amyloidosis
Systemic lupus erythematosus Drugs (nonsteroidal anti-inflammatory, penicillamine, "street heroin") Infections (malaria, syphilis, hepatitis B and C, acquired
immunodeficiency syndrome) Malignant disease (carcinoma, lymphoma) Miscellaneous (bee-sting allergy, hereditary nephritis)
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Causes and conditions associatedwith nephrotic syndrome
Frequency:Children (unselected):
Minimal change disease 88%
Focal segmental glomerulosclerosis 5%Membranoproliferative glomerulonephritis 1%
Adults:
Minimal change 15%
Focal segmental glomerulosclerosis 35%
Membranous glomerulopathy 33%
Membranoproliferative glomerulonephritis 2%
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Nephrotic syndrome
Associated with a myriad of diseases.
A few forms of glomerular diseasesaccount of most of the cases of nephroticsyndrome.
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Hypoproteinemia
Albumin
Immunoglobulins
Metal binding proteins Erythropoietin urinary
loss
Transferrin Complement deficiency
Coagulation components
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Hyperlipidemia
Hypercholesterolemia
Hypertriglyceridemia
Low-density lipoproteins (LDL) Very low- density lipoproteins (VLDL)
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Mechanisms of Hyperlipidemia
Increased hepatic synthesis of LDL, VLDL andlipoprotein (a) in response to hypoalbuminemia
Urinary loss of HDL
Enzymatic changes with abnormal lipidbiosythesis and degradation
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Edema
Lower colloid osmotic pressure?
15mmHg H2Ocolloid osmotic pressure26 mmHg
Edema
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Diagnosis
Diagnosis:
NS?
Primary or secondary?Complications?
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Differential diagnosis
Primary Secondary
children minimal change allergic purpuranephritis
Teenager mesangial proliferative FSGSnephritis
Middle age mesengial capillary SLE LN
nephritisold age membranous myeloma,
amyloidosis
nephropathy
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Complications
Acute renal failure( ARF)
Hypoalbuminemia Hypovolemia pre-renal
azotemia
Dyslipidemia
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Treatment
Support care
Rest in bed; limitation of proteinintake(0.8-1.0g/kg/d); limitation of saltintake (
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Treatment
FSGS: sensitive to steroids in 30-50% ofpatients; slow response to therapy;steroids therapy (onset) for 3-4 months; if
not response until 6 month (resistant),then try cyclosporine.
Mesangial proliferative GN: no evidence
show that adults will response to steroids;aspirin
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Acute Nephritis
Acute onset Gross hematuria Hypertension Mild to moderate proteinuria
Classic example: acute post-s treptococcalglomerulonephr i t is
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Asymptomatic hematuria or
proteinuria
Haematuria
Subnephrotic proteinuria
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Rapidly Progressive
Glomerulonephritis
Characterised by:
Acute nephritis Proteinuria
Acute renal failure
Classic example: Crescentic glomerulonephritis
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Histologic Alteration of
glomerulus - 1
Hypercellularity
(proliferative)
Increase in number of cells
in glomerular tuft.Due to:
Cellular proliferation ofmesangial or endothelial
cells (endocapillary) Leukocyte infiltration
Crescent formation(extracapillary)
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Histologic Alteration of
glomerulus - 2Basement membranethickening
Thickening of capillary wall by lightmicroscopy
On electron microscopy -deposition of amorphous materialoften immune complexes onendothelial/epithelial side ofbasement membrane e.g.membranous g lomeru lopathy
OR Thickening of basement
membrane proper e.g diabet icg lomerulosc leros is
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Histologic Alteration of
glomerulus- 3
Hyalinisation or
sclerosis of
glomerulus
Amorphous substance plasma proteins
Collagen
Capillary luminaobliterated
End result of variousglomerular damage
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Histologic Alteration of
glomerulus
1. Hypercellularity (proliferative)
2. Basement membrane thickening
3. Hyalinisation or sclerosis ofglomerulus
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Glomerular Pathology
Terminology
Diffuse- all glomeruli affected
Global- the whole glomerulus affected
Focalonly a portion of glomeruli affected
Segmentalonly a part (segment) of each glomerulus affected
Mesangialmesangium affected.
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Summary of Glomerular SyndromesAcute nephritic syndrome Hematuria, azotemia, variable
proteinuria, oliguria, edema, andhypertension
Rapidly progressiveglomerulonephritis
Acute nephritis, proteinuria, andacute renal failure
Nephrotic syndrome >3.5 gm proteinuria,hypoalbuminemia, hyperlipidemia,lipiduria
Chronic renal failure Azotemia uremia progressing foryears
Asymptomatic hematuria orproteinuria
Glomerular hematuria; subnephroticproteinuria