DR. MUHAMMAD RAFIQUE

ASSISTANT PROF. PAEDITRICS

Hematuria+Acute Nephritis

DefinitionMacroscopic (gross) Hematuria:any discolored urine visible to the human eye/>10 RBCs/HPF

Microscopic Hematuria:>5 RBC under microscope/ HPF in 3 of 3 consecutive centrifuged Specimen of urine at least

1 week apart.

HematuriaHematuria

Transient

Phenomenon of little significance

Transient

Phenomenon of little significance

Persistent

sign of seriousrenal disease

Persistent

sign of seriousrenal disease

Classification of hematuria

Macroscopic - Microscopic

Symptomatic - Symptomless.

Transient - Persistent

SHOULD WE CONSIDER ANYCHANGES IN THE URINE COLOUR , RED BROWN AS ?

Differential diagnosis of dark urine

Haemoglobinuria: dipstick positive but no red cells on microscopy

MyoglobinuriaFood, e.g. beet rootDrugs, e.g. rifampicin, nitrofurantoin,

sennaPorphyria: urine darkens on standingBilirubinuria: obstructive billiary disease

Causes of HematuriaInfection: cystitis, tuberculosis.Tumour: Wilm’s tumourTrauma: renal tract trauma e.g. accidents, catheterInflammation: glomerulonephritis, HSP,

IgA nephropathy, polyarteritis nodosa.Structural: calculi (renal, bladder, ureteric).Haematological: SCD, coagulation disorders.Toxins: sulphonamides, cyclophosphamide, NSAID.Others: genital bleeding, child abuse, menstruation. Factitious: (including Munchausen by proxy).

Sites of origin of hematuria

Renal HematuriaUpper urinary tract

glomeruluscollecting tubules

interstitium

Lower urinary tractcalyxpelvisureterbladderurethra

Glomerular Hematuria

RENAL

IgAnephropathy

Post infectious

MPGN

MULTI-SYSTEM

HSP nephrititisSLE nephritisHUSSickle cell Disease

Extraglomerular Hematuria (RENAL)

UPPER UR TRACT

pyelonephritisATNpapillary necrosis

nephrocalcinosisThrombosis.MalformationTumor

LOWER UR TRACT

cystitisurethritisurolithiasistraumacoagulopathyheavy exercise

EXTRA RENAL

Bleeding disorder.

Mention bleeding disorders presented as haematuria ?

HaemophiliaITP

Isolated Hematuria (microscopic)Isolated Hematuria (microscopic)

No other urinary abnormalities.No renal insufficiency.No evidence for systemic disease.

Incidence ( school-aged children ) 4-6% - single urine examination 0.5-1% - repeated testing over 6-12 months

Isolated hematuriaIsolated hematuriaThe child needs to be monitored for new signs:

Hypertension Proteinuria Changes in the pattern or severity of hematuria No change in the first year, observation at yearly intervals adequate (check for serum creatinine) .BP and urine analysis every 3 mo, until the hematuria resolve.

ISOLATED GROSS RECURRENT HEMATURIA

IG-A NEPHROPATHYALPORT’S SYNDROMEHYPERCALCIURIA

ASYMPTOMATIC PATIENT

SYMPTOMATIC / ASYMPTOMATIC

SYMPTOMATIC• Renal symptoms–UTI–Nephrolithiasis

• Systemic symptoms

–HSP–SLE

ASYMPTOMATIC• Hypercalciuria• Cystic kidney disease

• Vascular -thrombosis

• Obstruction• Trauma • Tumor • Exercise• Coagulation disorder

• Hemoglobinopathy• Benign F. Hematuria

• IgA nephropathy• Acute PSGN

Hematuria……

Patient comes to you, complaining that his/her urine is reddish in color...

What is your first step?

HematuriaIs it really blood?Urine Dipstick

detects - Hb. - Myoglobin - Protein

Evaluation of HematuriaHistory

Evaluation of HematuriaHistory

Detailed review of F/Hx.• Hematuria• Proteinuria• Renal insufficiency• Deafness• Stones

Precipitating factors: infection, exercise Abdominal plain radiograph for:• HSP• Hydronephrosis• Pyelonephritis• Urolithiasis

Evaluation of Hematuria…Physical Examination

Evaluation of Hematuria…Physical Examination

Growth failure.Hypertension.Pallor.Edema.Rash.Abdomen: mass or tenderness.External genitalia:

- Bleeding - Infection -Trauma

Do urine analysis

After confirming hematuria .

? isIs it glomerular or not ?Is it from upper / lower utract?

Glomerular versus extraglomerular bleeding

Urinary findingUrinary findingGlomerularGlomerularExtraglomerulExtraglomerularar

- Red cell casts- Red cell castsMay be presentMay be presentAbsentAbsent

- Red cell- Red cell

morphologymorphologyDysmorphicDysmorphicUniformUniform

- Proteinuria- ProteinuriaMay be presentMay be presentAbsentAbsent

- Blood Clots- Blood ClotsAbsentAbsentMay be May be presentpresent

- Urine Color- Urine ColorMay be May be red/brown, cola red/brown, cola likelike

May be redMay be red

INVESTIGATIONSGLOMERULAR CBC Electrolyte,

Ca BUN C3 / C4 Cholesterol ASO titer ANA, ANCA Throat

culture Skin culture 24 hr urinary protein

EXTRA GLOMERULARSTEP1 : Urine cultureSTEP 2 : Urine -ca./cr.Hb. Electrophoresis for

SCDUSG - renal/bladderSTEP 3:Serum electrolytes , ca.

cr.If nephrocalcinosis,

urolithiasis -24 hr urine for: ca; cr; uric acid ,

oxalatesIf hydronephrosis: -cystogram, renal scan

When to refer to nephrologist ?

• Patient with nephritis.• Hypertension.• Renal insufficiency.• Urolithiasis ,nephrocalcinosis• F/Hx. of renal disease• Persistent asymptomatic hematuria > year

Nephritis• Nephritis often caused by infections, toxins

and auto-immune diseases. -Autoimmune Diseases - IgA nephropathy

-Hereditary Nephritis –Alport’s Syndrome

-Infection-related Glomerular Disease, bacterial endocarditis -HIV

Nephritis• Most common cause of glomerular injury. • It is a disturbance of the glomerular structure

with inflammatory cell proliferation. This can leads to: reduced glomerular blood flow(GFR) –(oliguria).

retention of waste products-(uremia/azotemia).

leakage of RBCs from damaged glomerulous -(hematuria).

Low renal blood flow activates the renin-angiotensin-aldosterone system leading to (hypertension).

Types of nephritis Glomerulonephritis: inflammation of glomeruli.

Interstitial nephritis: inflammation of the spaces between renal tubules.

pyelonephritis: when UTI has reached the pyelum (pelvis) of the kidney.

Lupus nephritis: a disease of the immune system

A 7 year-old boy presents with coca-colored urine, periorbital edema, and headache.

His blood pressure is 150/100.

Etiology of PSGN Follow1-3 weeks infection by group-A beta

hemolytic streptococci (M and T serotypes).

M types 1, 2, 4, 12, 18, 25, 49, 55, 57, and 60 may have nephritogenic potential. These may be caused by skin or throat infections.

The risk of developing nephritis infection by M type 49 is 5% if it is present in the throat, 25% if infection by the same organism in the skin .

Clinical picture of PSGNlatent period 1-3 weeks between onset of

strep. infection and development of clinical nephritis

Edema is the most frequent symptom.Gross hematuria at onset in 30-50% .

Urine is smoky, cola/tea colored, or rusty. The color is dependent on amount of blood present and the pH of the urine.

Observant parents may note oliguriaHypertension.

Diagnosis of PSGN

Investigations for recent Group-A Strep. Infection.Throat or skin cultureASO titerAnti –DNase B (skin)Streptozyme test

Decreased complement levels -usually C3 complement returns normal in 4-6 weeks

Urine analysis in PSGNUrine output mostly reduced.Hematuria - most consistent

abnormality.Hyaline and/or epithelial cellular castsRBC casts have been found in 60-85%

of hospitalized children with PSGN.

Urine color in PSGN

Urine microscopy

-Erythrocyte cast. Arrows:erythrocytes embedded in the matrix of the cast

-Their presence indicatesglomerular hematuria

RBCs casts formation

HISTOLOGICAL FINDINGS

light microscopy: the glomerular tufts appear enlarged and swollen, often filling Bowman space.

Immunofluorescent microscopy: Granular deposits of immunoglobulin G (IgG) and C3 along the capillary walls.

Electron microscopy: electron-dense deposits (humps) in the subepithelial space.

PSGN

Admission criteria for PSGN?

Hypertension.Increased S. creatinine.Electrolyte disturbance (high K+)

Duration of Hematuria/Proteinuria?

Gross Hematuria: Few weeksMicroscopic Hematuria:18 monthsProteinuria: Decreasing over 18 months

Compilications of PSGNHypertensive encephalopathy

Heart Failure.

K, po4, ca

Acidosis Seizure Renal failure.

Treatment of PSGN

Symptomatic. - Antibiotics - Antihypertensives - DiureticsComplete recovery in 95 %Recurrence is extremely rare.

Question?

14 yr female with hematuria.

More “tired” latelyWhat is your diagnosis?

What labs. will you order?

SLE Nephritis

C3 and C4 will be low.Anti DNAse-B +veSometimes will only manifest as

kidney disease.

When to consider renal biopsy in GN ?

Acute renal failure.Nephrotic SyndromeAbsence of evidence of strep. infectionAbsence of hypocomplementemia. Persistent proteinuria, hematuria

>6mo.Persistently low C3 for>3 months.

Thank you for your attention!