PEDBASE.org - The Pediatric Database - Detailed information of HEMATURIA

HEMATURIA

DEFINITION:

The excretion of 3 or more RBC's in a high powered field in 2 or more consecutive urine samples (freshly voided, clean-catch concentrated urine).

EPIDEMIOLOGY:

incidence: .17 - 0.3%
- prevalence: .4 - 2.1% (microscopic hematuria)
risk factors:
- see differential diagnosis

DIFFERENTIAL DIAGNOSIS:

1. Renal

1. Trauma

blunt, calculi, hydronephrosis

2. Toxic

anticoagulants, acetazolamine, aminophylline, bacitracin, cephalosporin, cyclophosphamide, kanamycin, methicillin, PAS, phensuximide, phenytoin, salicylate, sulphonamides

3. Infectious

UTI - bacterial, viral, TB

4. Hemorrhagic

angioma, coagulopathies, infarct (emboli), malformations, papillary necrosis, telangiectasia, varices

5. Neoplastic

Wilms' Tumor

6. Intrinsic

1. Glomerular

1. Hereditary

Alport Syndrome*
Benign Familial Hematuria*
Thin Basement Membrane Disease*
Nail-Patella Nephritis

2. Non-Hereditary

1. Acute Glomerulonephritis (GN)

Poststreptococcal GN*
Hemolytic Uremic Syndrome
Henoch-Schoenlein Purpura

2. Chronic Glomerulonephritis

1. Primary Glomerulonephritis

Berger (IgA) Nephropathy*
Focal Segmental Glomerulosclerosis
Mesangioproliferative GN
Membranoproliferative GN

2. Secondary Glomerulonephritis

Goodpastures Nephropathy
SLE Nephropathy
Wegeners Nephropathy
Paraproteinemia
- Amyloid Nephropathy
- Essential Cryoimmunoglobulinemia

2. Tubulointerstitial

1. Trauma

2. Toxic

Heavy Metals (mercury, lead)

3. Infectious

viral (CMV, EBV)

4. Hereditary

Polycystic Kidney Disease
- Autosomal Recessive (Infantile Form)
- Autosomal Dominant (Adult Form)

5. Vascular

Sickle Cell Nephropathy
Others
- Arteriolor Nephrosclerosis
- Atheroembolic Nephropathy

6. Immune

SLE Nephropathy
Sjogren Disease
- most common causes of renal hematuria in childhood

2. Post Renal (Anatomical)

1. Ureter

1. Congenital Anomalies*

UVJ Obstruction, UPJ Obstruction, Vesicoureteral Reflux

2. Others

stone, trauma, tumor, uretitis

2. Bladder

cystitis (infectious [UTI]*, chemical), diverticuli, foreign bodies (stones), obstruction, polypi, trauma, tumor

3. Urethra

carbuncle, foreign body, meatal ulcer, urethritis, trauma, tumor

4. Prostate

inflammatory

5. Penis

excoriation, foreign body, trauma
- most common causes of post-renal hematuria in childhood

CLINICAL FEATURES

1. History of Presenting Illness

at the end of the history, one should be able to discern:
- glomerular vs non-glomerular hematuria (if glomerular then)
- hereditary vs non-hereditary (if non-herediatary then)
- acute GN vs chronic GN (if chronic then)
- primary GN vs secondary GN

onset
- when began with conditions identified around the initial presentation, i.e., trauma, drug ingestion, etc
- record of previous urinalyses
precipitation/palliation
- identification of triggering agents
  - other streptococcal infections - impetigo, pharyngitis URT, in other family members and close contacts - exposure to other infections (diarrhea), drugs, foods (hamburger), allergens, vaccinations, chemicals, etc
quality
- real vs factitious
  - the following will give the appearance of hematuria
    - foods: beets, blackberries, food dyes
    - drugs: rifampin, laxatives (phenolphthalein) deferoxamine
    - chemicals: pyridines, urates
- glomerular vs non-glomerular hematuria
  - urine colour (pink, red, coke, burgundy, dark brown)
- gross vs microscopic hematuria
  - urine colour +/- clots
radiation
- other sites of bleeding
severity
- signs and symptoms of acute/chronic anemia
timing
- acute vs acute-on-chronic
- duration of hematuria
- frequency per day, week, month and is the frequency of hematuria increasing over time
associated symptoms
- past medical history
- functional inquiry
  - see Specific Entities below and files in the Database on the individual disorders listed in the differential diagnosis of hematuria
  - helps differentiate acute GN from chronic GN and primary GN from secondary GN

2. Family History

helps to differentiate hereditary from non-hereditary forms

1. Hematuria

family members must have had previous urinalyes to ascertain this

2. Renal Disease

stones, Polycystic Kidney Disease, Wilms' Tumor
renal dialysis
kidney transplantation

3. Others

bleeding disorders
hearing/ocular impairment (Alport Syndrome)

3. Physical Examination

1. Vitals

hypertension, fever

2. Glomerular Hematuria

presents in 1 of 3 ways:

1. Microscopic Hematuria +/- Proteinuria

2. Macroscopic Hematuria + Proteinuria

brown or cola-coloured urine

3. Nephritic Syndrome

oliguria, anuria
edema - peripheral or periorbital
hypertension with headaches, nausea/vomiting, seizures

3. Non-Glomerular Hematuria

bright red blood +/- clots
varying degrees of hematuria during stages of urination
dysuria, urgency, frequency, flank pain, polyuria
passage of stones

4. Specific Entities

1. Alport Syndrome

hematuria - microscopic +/- gross
associated symptoms
- HEMATURIA, ocular anomalies (keratoconus, cataracts, lenticonus, nystagmus)
- Nephritic and/or Nephrotic Syndrome

2. Benign Familial Hematuria

hematuria - microscopic +/- gross
associated symptoms
- positive family history (autosomal dominant)

3. Thin Basement Membrane Disease

hematuria - microscopic +/- proteinuria
associated symptoms
- HEMATURIA
- Nephritic Syndrome

4. Nail-Patella Disease

hematuria - microscopic +/- proteinuria
associated symptoms
- dysplastic nails, hypoplastic or absent patellae
- Nephritic and/or Nephrotic Syndromes

5. Poststreptococcal GN

hematuria - microscopic +/- gross
associated symptoms
- prodrome of pharyngitis, URTI, impetigo
- Nephritic and/or Nephrotic Syndrome

6. Hemolytic Uremic Syndrome

hematuria - microscopic +/- proteinuria
associated symptoms
- prodrome of bloody diarrhea
- anemia, thrombocytopenia (petechiae)
- Nephritic and/or Nephrotic Syndrome

7. Henoch-Schoenlein Purpura

hematuria - microscopic +/- proteinuria
associated symptoms
- purpuric rash, arthritis, abdominal pain
- Nephritic and/or Nephrotic Syndrome

8. Goodpastures Disease

hematuria - microscopic +/- proteinuria
associated symptoms
- hemoptysis
- Nephritic and/or Nephrotic Syndrome

9. Wegeners Nephropathy

hematuria
associated symptoms
- otitis media, adenitis, sinusitis, respiratory distress

INVESTIGATIONS:

1. Hematuria (Diagnosis)

1. Hematuria - Dipstick

detects hematuria, hemoglobinuria, myoglobinuria
principle: orthotolodine-peroxide oxidizes blue in the presence of Hb and Mb peroxidase-like activity
positive if 2 or more RBC/hpf
FP: ascorbic acid FN: ? urinary tract infections

2. RBC Morphology - Microscopy

if +dipstick, urine examined for RBC's
positive for hematuria if 3 or greater RBC's/hpf
examine 0.5cc of a resuspended pellet from a 10cc centrifuged sample
Light Microscopy
- glomerular
  - RBC casts
  - damaged RBC's - dysmorphic, distorted, pitted, small
- non-glomerular
  - lack of RBC cases
  - undamaged RBC's
- with Wright Stain (to view WBC's)

2. First Line Investigations

1. Urine

1. Urinalysis

proteinuria, WBC, bacteria, crystals, pH
test patient and all family members

2. Urine Culture and Sensitivity (C&S)

3. Microscopy

RBC morphology and casts

2. Serum

Renal Function Tests
- serum creatinine, BUN, uric acid
- Ca/Cr ratio (hypercalcuria)
CBC with smear

3. Second Line Investigations

1. Serology

complement (C3, C4), ANA, anti-DNA Ab, ASO titre

2. Imaging Studies

1. Renal Ultrasound

indicated for:
- gross hematuria
- suspected post renal anomalies
- to rule out congenital anomalies and Wilms' Tumor in young patients (less than 5 years of age)

2. Voiding Cystourethrogram (VCUG)

for suspected vesicoureteral reflux (VUR)

3. IVP

for imaging of collecting system, to detect stones and/or obstructive uropathy

3. Others

throat swab

MANAGEMENT:

1. Supportive

treat anemia
treat underlying disorder
treat complications

2. Acute Nephritic Syndrome

fluid restriction
control hypertension
manage hyperkalemia
manage pulmonary edema

Pediatric Database - HEMATURIA

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