Introduction
Hematuria means blood in the urine. Significant hematuria is defined as more than 5 red blood cells/HPF of standard urinary sediment. The site of bleeding can be from the kidneys/pelvicalyceal system/ureters/urinary bladder or urethra. Rarely hematuria is due to coagulation disorder.
The color of the urine can give clues to the site of hematuria eg. Cola colored urine - glomerular bleeding especially with proteinuria >2+ and puffiness of eyes. Bright red color especially if at the end of urination is due to bleeding from the urinary bladder or urethra. Every pink/red/brown colored urine need not be due to blood in the urine. Ingestion of colored sweets, beetroot, drugs, hemoglobin or myoglobin can cause red urine and history is very important before investigating further.
Intermittent Hematuria In an Active Child
Always rule out exercise-induced hematuria (after active sports or school activity, RBCs appear in urine and disappear if the child is rested. IVP/Cystoscopy -are rarely required for the diagnosis of hematuria Microscopic hematuria of long-standing usually does not cause anemia, hence does not need hematinics.
Presentation
Clinical Clues From History And Physical Examination
Dysuria, loin pain, colic | Hypercalcuria, stone
|
joint pain, skin rash
| SLE, HSP |
Bleeding tendency
| Coagulation disorders, platelet deficiency
|
Family history of hematuria
| Benign familial, Alport's syndrome, stones |
Purpura
| HSP |
Edema, Hypertension | Glomerulonephritis |
Anemia, Splenomegaly | Sickle cell disease
|
Renal angle tenderness
| Urinary tract infection
|
Renal mass
| Wilm's tumor, Polycystic kidney disease, hydronephrosis
|
Deafness
| Alport's syndrome |
Painful hematuria
| Stones, UTI, hypercalciuria |
Salient Features of Selected Diseases With Hematuria
Alport's Syndrome
It is a triad of persistent hematuria, deafness, familial disease along eye anomalies which progresses to chronic renal failure especially in males. It is inherited as a sex-linked recessive/autosomal recessive/autosomal dominant condition. No treatment is available except renal transplant when the child progresses to ESRD.
IgA nephropathy
It is a non-familial disease of unknown etiology. It presents with persistent microscopic and rarely macro hematuria. In the majority of cases, it does not progress to ESRD in pediatric age. But 15% of cases of IgA nephropathy in adults end in ESRD. Rarely nephrotic syndrome and hypertension are encountered after many years of onset.
Familial benign hematuria
It also presents with persistent microscopic hematuria and needs to be differentiated from Alport's syndrome since the prognosis is good in Familial benign hematuria whilst chronic renal failure occurs in Alport's syndrome. The final diagnosis is based on audiometry and kidney biopsy on EM.
Urine
- color
- dipstick to detect blood/benzedine test (+ve with Hb/myoglobin and proteinuria)
- microscopy of urine for RBC's
- Wright's stain/phase contrast microscopy/Coulter counter can detect source of bleeding from glomerular or non glomerular source
Investigations should be phased in such a way as to rule out common causes.
Phase 1
- Acute post infections glomerulonephritis- ASO titre, serum C3 level, HBsAg
- Acute pyelonephritis - Urine Culture, Colony Count
- Hypercalciuria - Urinary Calcium/Creatinine ratio, 24 hours urinary calcium excretion
- Ultrasonography to rule out stones, tumors, hydronephrosis, cystic kidney disease
If urine examination reveals <5 RBCs/HPF on monthly examination, then urine examination should be repeated at least 3 times before it is considered normal.
Phase 2
If hematuria is persistent in school age, then the following should be done:
- Family members should be screened for hematuria
- ANA, Anti dsDNA, C3 levels
- Renal function tests
- Audiometry for nerve deafness
- Renal biopsy to rule out IgA nephropathy. If biopsy is normal then in case of positive family history, it is familial benign nephropathy
Indications for renal biopsy (Light immunoflourescent and electron microscopy)
- Asymptomatic hematuria for more than 1 year
- Hematuria with positive family history
- Hematuria + moderate to severe proteinuria, hypertension or elevated BUN, S.creatinine lasting for 2-3 weeks (atypical features in acute post streptococcal GN)
- Deafness
+ve serology for hepatitis B or SLE
* Alport's syndrome can be diagnosed only if EM is done. IgA nephropathy can be diagnosed only if IF is done. If EM and IF is not possible, light microscopy alone can give a final diagnosis in all condition except Alport's syndrome and IgA nephropathy