hematuriahematuria transient phenomenon of little significance transient phenomenon of little...
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HematuriaHematuriaHematuriaHematuria
Transient phenomenonof little significance
Transient phenomenonof little significance
Sign of seriousrenal disease
Sign of seriousrenal disease
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Classification of hematuriaClassification of hematuria
Macroscopic - Microscopic
Symptomatic - Symptomless
Transient - Persistent
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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.
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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
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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
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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
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Causes of HematuriaCauses of HematuriaCauses of HematuriaCauses of Hematuria
Kidney disease
Lesions along the urinary tract
Conditions unrelated to kidney and urinary tract
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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
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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
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Acute nephritic syndromeAcute nephritic syndrome Acute nephritic syndromeAcute nephritic syndrome
Hematuria Proteinuria
Reduced renal functionEdemaHypertension
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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
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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
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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
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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
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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
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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
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Alport syndromeAlport syndrome- hereditary disorder of - hereditary disorder of
GBMGBM
X-linked dominant
Autosomal recessive
Autosomal dominant
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Renal diseaseRenal disease
Macro /MicrohematuriaProteinuriaNephrotic syndromeHypertensionRenal failure- malesProgressive or juvenile – 20 yNonprogressive – 40 y
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Hearing defectsHearing defects
Sensorineural bilateral
Never congenital
Boys- 85% . Girls – 18% < 15y
Progression of hearing loss parallels renal impairment
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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
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ALPORT’S SYNDROME
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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
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ContinueContinue
Autosomal dominant trait
Normal antigenicity of the GBM
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Thin basement Thin basement membrane nephropathymembrane nephropathy
HematuriaProteinuriaAttenuation of the GBMIn children may be AlportIn adults m/p benign disorder
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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
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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
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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
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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
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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
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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
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HematuriaHematuriaHematuriaHematuria
Transient phenomenonof little significance
Transient phenomenonof little significance
Sign of seriousrenal diseaseSign of seriousrenal disease