HEMATURIA

1. HEMATURIA

2. Hematuria Transient phenomenon of little significance Sign of serious renal disease

3. Classification of hematuria • Macroscopic - Microscopic • Symptomatic - Symptomless • Transient - Persistent

5. Normal erythrocyte excretion rate * 0 – 425.000/12 h. ( mean – 65.750 ) T.Addis; J.of Clin Invest, 1926 • Upperlimit in children * 500.000 - 2.000.000/24h.

6. Detection & quantificationof 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

7. Microscopic Hematuria • Definition> 3-5 RBC/HPF > 5 RBC/mm³ > 8000 RBC/ml

11. Glomerular versus extraglomerular bleeding

16. Causes of Hematuria • Kidney disease • Lesions along the urinary tract • Conditions unrelated to kidney andurinary tract

17. Hematuria not representing kidney or urinary tract disorder • Following exercise • Febrile disorders • Gastroenteritis with dehydration • Contamination from external genitalia

20. Renal 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

21. Acute nephritic syndrome • Hematuria • Proteinuria • Reduced renal function • Edema • Hypertension

22. Renal 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

23. Causes of urinary tract related Hematuria • Infection • Urolithiasis • Obstruction ( UPJ Stenosis ) • Trauma • Drugs ( Cyclophosphamide ) • Tumors

24. 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

25. Etiologies of isolated Hematuria • 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

26. Hematuria with familial association • Glomerular - Benign Familial Hematuria - Alport syndrome • Non-glomerular - Idiopathic Hypercalciuria -Polycystic Kidney Disease - Urolithiasis - Tumors

27. Idiopathic Hypercalciuria • 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

28. Alport syndrome- hereditary disorder of GBM • X-linked dominant • Autosomal recessive • Autosomal dominant

29. Renal disease • Macro /Microhematuria • Proteinuria • Nephrotic syndrome • Hypertension • Renal failure- males • Progressive or juvenile – 20 y • Nonprogressive – 40 y

30. Hearing defects • Sensorineural bilateral • Never congenital • Boys- 85% . Girls – 18% < 15y • Progression of hearing loss parallels renal impairment

31. Diagnosis of Alport syndrome • Hematuria with or without proteinuria • Hypertension • Renal failure • Ocular defects – anterior lenticonus • Familial hematuria • Sensorineural hearing loss • Progression to renal failure occurring in at least one affected subject

33. ALPORT’S SYNDROME

34. Familial benign essential hematuria • Familial hematuria without proteinuria and without progression to renal failure or hearing defect • Diffuse attenuation of the GBM is usually considered the hallmark of the condition • It’s non pathognomonic of FBEH

35. Continue • Autosomal dominant trait • Normal antigenicity of the GBM

36. Thin basement membrane nephropathy • Hematuria • Proteinuria • Attenuation of the GBM • In children may be Alport • In adults m/p benign disorder

38. HEREDITARY NEPHROPATHY Alport Syndrome Benign Familial Hematuria Overlap 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

39. Evaluation of HematuriaHistory • Detailed review of family history hematuria proteinuria renal insufficiency deafness stones • Precipitating factors infection exercise • Abdominal pain HSP hydronephrosis pyelonephritis urolithiasis

40. Evaluation of HematuriaPhysical Examination • Growth failure • Hypertension • Pallor • Edema • Rash • Abdomen: search for a mass or tenderness • External genitalia: bleeding infection trauma

41. Work-up of a child with Hematuria • 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

42. Isolated 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 yearlyintervals is adequate

43. Isolated hematuriaPossible outcome • Disappearance of hematuria • Hematuria will persist - follow-up should be continued • The hematuria will no longer be “isolated” - further investigation

44. Hematuria Transient phenomenon of little significance Sign of serious renal disease