HematuriaHematuriaHematuriaHematuria

Transient phenomenonof little significance

Transient phenomenonof little significance

Sign of seriousrenal disease

Sign of seriousrenal disease

Classification of hematuriaClassification of hematuria

Macroscopic - Microscopic

Symptomatic - Symptomless

Transient - Persistent

Normal erythrocyte excretion rate * 0 – 425.000/12 h. ( mean – 65.750 )

T.Addis; J.of Clin Invest, 1926

Upper limit in children * 500.000 - 2.000.000/24h.

Detection & quantificationDetection & quantificationof hematuriaof hematuria

Detection & quantificationDetection & quantificationof hematuriaof hematuria

Screening test ( dipsticks ) - 0.02-0.03 mg/dL of Hb, myoglobin - ~5-20 RBC/mm³

Semiquantitative estimation - centrifugation of 10-15 ml of urine - resuspention of sediment in 1 ml of residual urine - high-power microscopy

Counting chamber - the number of cells in 1 microliter of unspun urine

Microscopic HematuriaMicroscopic HematuriaMicroscopic HematuriaMicroscopic Hematuria

Definition > 3-5 RBC/HPF

> 5 RBC/mm³

> 8000 RBC/ml

Definition > 3-5 RBC/HPF

> 5 RBC/mm³

> 8000 RBC/ml

Glomerular versus Glomerular versus extraglomerular bleedingextraglomerular bleeding

Urinary finding Glomerular Extraglomerular

Red cell casts May be present Absent

Red cell morphology

Dysmorphic Uniform

Proteinuria May be present Absent

Clots Absent May be present

Color May be red or brown

May be red

Causes of HematuriaCauses of HematuriaCauses of HematuriaCauses of Hematuria

Kidney disease

Lesions along the urinary tract

Conditions unrelated to kidney and urinary tract

Hematuria not representing Hematuria not representing kidney or urinary tract kidney or urinary tract

disorderdisorder

Hematuria not representing Hematuria not representing kidney or urinary tract kidney or urinary tract

disorderdisorderFollowing exercise

Febrile disorders

Gastroenteritis with dehydration

Contamination from external genitalia

Renal causes of HematuriaRenal causes of HematuriaRenal causes of HematuriaRenal causes of Hematuria

Glomerular - Acute Postinfectious Glomerulonephritis - IgA Nephropathy - Hereditary Nephritis ( Alport syndrome) - Benign Recurrent or Persistent Hematuria ( Thin Membrane Disease ) 1.Sporadic 2.Familial - Membranoproliferative Glomerulonephritis - Crescentic Glomerulonephritis - Lupus Nephritis - Nephritis of Henoch-Shönlein Purpura - Focal Glomerulosclerosis - Hemolityc-Uremic Syndrome

Acute nephritic syndromeAcute nephritic syndrome Acute nephritic syndromeAcute nephritic syndrome

Hematuria Proteinuria

Reduced renal functionEdemaHypertension

Renal causes of HematuriaRenal causes of HematuriaRenal causes of HematuriaRenal causes of Hematuria

Non-glomerular - Infection ( Pyelonephritis ) - Interstitial Nephritis - Metabolic ( Uric Acid, Nephrocalcinosis ) - Renal Malformation ( Cystic Kidney ) - Tumors ( Wilm’s, Acute Leukemia) - Idiopatic Hypercalciuria - Trauma

Causes of urinary tract Causes of urinary tract related Hematuriarelated Hematuria

Causes of urinary tract Causes of urinary tract related Hematuriarelated Hematuria

InfectionUrolithiasisObstruction ( UPJ Stenosis )TraumaDrugs ( Cyclophosphamide )Tumors

Isolated HematuriaIsolated Hematuria(microscopic)(microscopic)

Isolated HematuriaIsolated Hematuria(microscopic)(microscopic)

No other urinary abnormalitiesNo renal insufficiencyNo evidence for systemic disease

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

Etiologies of isolated Etiologies of isolated HematuriaHematuria

Etiologies of isolated Etiologies of isolated HematuriaHematuria

Glomerular - Benign Recurrent or Persistent Hematuria 1.Sporadic 2.Familial - IgA Nephropathy - Alport syndrome - PSAGN

Non-glomerular - Idiopathic Hypercalciuria - Cystic Kidneys - Urinary Tract obstruction - Tumors - Trauma

Hematuria with familial Hematuria with familial associationassociation

Hematuria with familial Hematuria with familial associationassociation

Glomerular - Benign Familial Hematuria - Alport syndrome

Non-glomerular - Idiopathic Hypercalciuria - Polycystic Kidney Disease - Urolithiasis - Tumors

Idiopathic Idiopathic HypercalciuriaHypercalciuria

Idiopathic Idiopathic HypercalciuriaHypercalciuria

Definition: Calcium excretion > 4 mg/kg/day Urinary Ca/Creatinine > 0.2

Possible mechanism of hematuria: microcrystals damaging the tubular or mucosal epithelia. Resolution of hematuria with anticalciuric therapy

Alport syndromeAlport syndrome- hereditary disorder of - hereditary disorder of

GBMGBM

X-linked dominant

Autosomal recessive

Autosomal dominant

Renal diseaseRenal disease

Macro /MicrohematuriaProteinuriaNephrotic syndromeHypertensionRenal failure- malesProgressive or juvenile – 20 yNonprogressive – 40 y

Hearing defectsHearing defects

Sensorineural bilateral

Never congenital

Boys- 85% . Girls – 18% < 15y

Progression of hearing loss parallels renal impairment

Diagnosis of Alport Diagnosis of Alport syndromesyndrome

Hematuria with or without proteinuriaHypertensionRenal failureOcular defects – anterior lenticonusFamilial hematuriaSensorineural hearing lossProgression to renal failure occurring in at least one affected subject

ALPORT’S SYNDROME

Familial benign Familial benign essential hematuriaessential hematuria

Familial hematuria without proteinuria and without progression to renal failure or hearing defectDiffuse attenuation of the GBM is usually considered the hallmark of the conditionIt’s non pathognomonic of FBEH

ContinueContinue

Autosomal dominant trait

Normal antigenicity of the GBM

Thin basement Thin basement membrane nephropathymembrane nephropathy

HematuriaProteinuriaAttenuation of the GBMIn children may be AlportIn adults m/p benign disorder

HEREDITARY NEPHROPATHYHEREDITARY NEPHROPATHYHEREDITARY NEPHROPATHYHEREDITARY NEPHROPATHY

Benign Familial Hematuria

Alport Syndrome

Overlap of histological findingsOverlap of histological findings

The prognosis appears to be depend more on the degree of clinical expression in other members of the family and less

on the histological findings

Evaluation of HematuriaEvaluation of Hematuria HistoryHistory

Evaluation of HematuriaEvaluation of Hematuria HistoryHistory

Detailed review of family history hematuria proteinuria renal insufficiency deafness stones

Precipitating factors infection exercise

Abdominal pain HSP hydronephrosis pyelonephritis urolithiasis

Evaluation of HematuriaEvaluation of HematuriaPhysical ExaminationPhysical Examination

Evaluation of HematuriaEvaluation of HematuriaPhysical ExaminationPhysical Examination

Growth failureHypertensionPallorEdemaRashAbdomen: search for a mass or tendernessExternal genitalia: bleeding

infection trauma

Work-up of a child with Work-up of a child with HematuriaHematuria

Work-up of a child with Work-up of a child with HematuriaHematuria

Phase I: Urinalysis ( sediment examination ) RBC’s morphology Urine culture BUN, Creatinine, Proteins, Electrolytes Antibodies against strept. & other antigens Complement, ANF, Immunoglobulins Renal US Urinalysis of 1st degree relatives 24h urine collection: Ca, Creat.,Protein, UA

Phase II: Hearing test Cystoscopy Renal biopsy

Isolated hematuriaIsolated hematuriaIsolated hematuriaIsolated hematuria

The child needs to be monitored for the appearance of new clinical signs:

hypertension proteinuria changes in the pattern or severity of hematuria

If there is no change in the first year,observation at yearly intervals is adequate

Isolated hematuriaIsolated hematuria Possible outcomePossible outcome

Isolated hematuriaIsolated hematuria Possible outcomePossible outcome

Disappearance of hematuria

Hematuria will persist - follow-up should be continued

The hematuria will no longer be “isolated” - further investigation

HematuriaHematuriaHematuriaHematuria

Transient phenomenonof little significance

Transient phenomenonof little significance

Sign of seriousrenal diseaseSign of seriousrenal disease