Hematuria

Transient phenomenon Sign of serious

of little significance renal disease
Classification 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 & quantification
of 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 Hematuria

Definition > 3-5 RBC/HPF

> 5 RBC/mm³

> 8000 RBC/ml

 Glomerular versus extraglomerular
bleeding
Urinary finding Glomerular Extraglomerular
Red cell casts May be present Absent

Red cell Dysmorphic Uniform

morphology
Proteinuria May be present Absent

Clots Absent May be present

Color May be red or May be red
brown
Causes of Hematuria

Kidney disease

Lesions along the urinary tract

Conditions unrelated to kidney and

urinary tract
 Hematuria not representing
kidney or urinary tract disorder
Following exercise

Febrile disorders

Gastroenteritis with dehydration

Contamination from external

genitalia
 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
Acute nephritic syndrome

Hematuria
Proteinuria
Reduced renal function
Edema
Hypertension
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
Causes of urinary tract
related Hematuria

Infection
Urolithiasis
Obstruction ( UPJ Stenosis )
Trauma
Drugs ( Cyclophosphamide )
Tumors
 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
 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
Hematuria with familial
association
Glomerular
- Benign Familial Hematuria
- Alport syndrome
Non-glomerular
- Idiopathic Hypercalciuria
- Polycystic Kidney Disease
- Urolithiasis
- Tumors
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
 Alport syndrome
- hereditary disorder of
GBM
X-linked dominant

Autosomal recessive

Autosomal dominant
 Renal disease
Macro /Microhematuria
Proteinuria
Nephrotic syndrome
Hypertension
Renal failure- males
Progressive or juvenile – 20 y
Nonprogressive – 40 y
 Hearing defects
Sensorineural bilateral

Never congenital

Boys- 85% . Girls – 18% < 15y

Progression of hearing loss parallels

renal impairment
 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
ALPORT’S SYNDROME
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
 Continue

Autosomal dominant trait

Normal antigenicity of the GBM

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

Benign Familial Alport Syndrome

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
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
Evaluation of Hematuria
Physical Examination
Growth failure
Hypertension
Pallor
Edema
Rash
Abdomen: search for a mass or tenderness
External genitalia: bleeding
infection
trauma
 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
 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
 Isolated hematuria
Possible outcome
Disappearance of hematuria

Hematuria will persist -

follow-up should be continued

The hematuria will no longer be “isolated” -

further investigation
 Hematuria

Transient phenomenon Sign of serious

of little significance renal disease