HEMATURIA

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HEMATURIA
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Hematuria
Transient phenomenon of little significance
Sign of serious renal disease
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Classification 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 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

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Microscopic Hematuria

• Definition gt 3-5 RBC/HPF
• gt 5 RBC/mm³
• gt 8000 RBC/ml

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Glomerular versus extraglomerular 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 Hematuria

• Kidney disease
• Lesions along the urinary tract
• Conditions unrelated to kidney and urinary tract

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Hematuria not representing kidney or urinary
tract disorder

• Following exercise
• Febrile disorders
• Gastroenteritis with dehydration
• Contamination from external genitalia

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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

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Acute nephritic syndrome

• Hematuria
• Proteinuria
• Reduced renal function
• Edema
• Hypertension

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Renal causes of Hematuria

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

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Causes of urinary tract related Hematuria

• Infection
• Urolithiasis
• Obstruction ( UPJ Stenosis )
• Trauma
• Drugs ( Cyclophosphamide )
• Tumors

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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

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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

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Hematuria with familial association

• Glomerular
• - Benign Familial Hematuria
• - Alport syndrome
• Non-glomerular
• - Idiopathic Hypercalciuria
• - Polycystic Kidney Disease
• - Urolithiasis
• - Tumors

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Idiopathic Hypercalciuria

• Definition Calcium excretion gt 4 mg/kg/day
• Urinary Ca/Creatinine gt
  0.2
• Possible mechanism of hematuria microcrystals
  damaging the tubular or mucosal epithelia.
  Resolution of hematuria with anticalciuric
  therapy

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Alport syndrome- hereditary disorder of GBM

• X-linked dominant
• Autosomal recessive
• Autosomal dominant

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Renal disease

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

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Hearing defects

• Sensorineural bilateral
• Never congenital
• Boys- 85 . Girls 18 lt 15y
• Progression of hearing loss parallels renal
  impairment

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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

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ALPORTS SYNDROME
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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
• Its non pathognomonic of FBEH

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Continue

• Autosomal dominant trait
• Normal antigenicity of the GBM

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Thin basement membrane nephropathy

• Hematuria
• Proteinuria
• Attenuation of the GBM
• In children may be Alport
• In adults m/p benign disorder

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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
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Evaluation of Hematuria History

• 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 HematuriaPhysical Examination

• Growth failure
• Hypertension
• Pallor
• Edema
• Rash
• Abdomen search for a mass or tenderness
• External genitalia bleeding
• infection
• trauma

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Work-up of a child with Hematuria

• Phase I Urinalysis ( sediment examination )
• RBCs 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 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 hematuria
Possible 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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Hematuria
Transient phenomenon of little significance
Sign of serious renal disease