• Hematuria is defined as the presence of at least 5 red blood cells per microliter of urine.
• The prevalence of microscopic hematuria among school-aged children ranges from 0.5% to 2.0%.

Causes of Hematuria:

1. Glomerular hematuria

   Isolated renal disease
   -Postinfectious GN (Post streptococcal)
   – IgA nephropathy( Berger ds)
   – Alport syndrome( hereditary )
   – Thin Glomerular Basement membrane disease
   – Membranoproliferative GN
   – Membranous nephropathy
   – Focal segmental glomerulosclerosis
   – Antiglomerular basement membrane ds
   Multisystem disease
   -HSP nephritis
   -SLE nephritis
   -HUS
   -Wegener granulomatosis
   -Polyarteritis nodosa
   -Goodpasture syndrome
   -HIV nephropathy
   -Sickle cell glomerulopathy

2. Extraglomerular hematuria

   • Upper Urinary tract
     Tubulointerstitial:
     • Pyelonephritis
     • Interstitial nephritis
     • ATN
     • Papillary necrosis
     • Nephrocalcinosis

     Anatomic:

     • Hydronephrosis
     • Polycystic kidney disease
     • Tumor (Wilms, Rhabdomyosarcoma, Angiomyolipoma)
     • Trauma

     Vascular:

     • Arterial/venous thrombosis
     • Malformation (aneurysms, hemangioma)
     • Hemoglobinopathy (Sickle cell disease)
     • Crystalluria: Calcium, Oxalate, Uric acid
     • Medications: NSAIDs, anticoagulants
   • Lower urinary tract
     • Inflammation- infectious and noninfectious
     • Cystitis
     • Urethritis
     • Urolithiasis
     • Trauma
     • Coagulopathy
     • Heavy exercise
   • Bladder tumor
     Factitious syndrome / by proxy

Common causes of gross hematuria:

• Urinary tract infection
• Meatal stenosis
• Perineal irritation
• Trauma
• Urolithiasis
• Hypercalciuria
• Coagulopathy
• Tumor
• Glomerular
  • IgA nephropathy
  • Alport syndrome
  • Thin glomerular basement membrane disease
  • Post-infectious glomerulonephritis
  • HSP nephritis
  • SLE nephritis

Causes of Hematuria in the Newborn:

• Renal vein thrombosis
• Renal artery thrombosis
• Autosomal recessive polycystic kidney disease
• Obstructive uropathy
• Urinary tract infection
• Bleeding and clotting disorders
• Trauma, bladder catheterization
• Cortical necrosis
• Nephrocalcinosis

History:

• Age: 2-5yrs: Wilms tumor 5-12yrs: PSGN
• Sex: F>M UTI, SLE nephritis. Alport syndrome, IgA Nephropathy
• Race: whites: Idiopathic hypercalciuria blacks: Sickle cell disease

Colour of urine:

Characteristics of urine:

• Amount of urine: Reduced in AGN, ARF
• Clots in urine: Extraglomerular
• Frequency, Dysuria, recent enuresis: UTI
• Frothy urine: Suggests Proteinuria seen in Glomerular diseases
• Timing:
• Initial stream – from the urethra (Urethrorrhagia – spotting in underwear);
• Terminal (with suprapubic pain, disturbance of micturition) – from the bladder
• The recurrent episode of Gross Hematuria
• IgA Nephropathy
• Alport Syndrome
• Thin Glomerular basement disease
• Hypercalciuria
• Urolithiasis

Associated Symptoms:

• Fever: Infections, SLE, AGN
• Facial puffiness, Oedema of legs, weight gain, Cough, Shortness of breath: Acute Glomerulonephritis
• Hypertension (Headache, visual changes, epistaxis, seizures): AGN, ARF
• Abdominal pain: Urolithiasis (Loin to groin), UTI, clots, Nutcracker syndrome, Idiopathic hypercalciuria
• Painless: Glomerular
• Abdominal mass: Hydronephrosis, PCKD, Wilm’s tumor
• Joint pain: HSP, SLE
• Rashes: HSP, SLE, PAN
• Neurologic: SLE, HUS (seizures, irritability)
• Cutaneous Abnormalities Tuberous, Sclerosis, Von Hippel Lindau Syndrome
• Jaundice: Hemolysis, Obstructive jaundice
• H/o exercise, menstruation, recent bladder catheterization or passage of a calculus
• Recent upper respiratory
• 1-2wks back PSGN(Hematuria can last up to 4-6wk
• 1-2 days IgA Nephropathy(Hematuria lasts up to 5 days)
• 1-2 days (Alport Syndrome)
• Skin infection (3-6 wks): PSGN
• GI infection: HUS, HSP nephritis, IgA Nephropathy
• H/o bleeding from other sites: Bleeding disorders, Hemoptysis in Good Pasture syndrome
• H/o Trauma, Unexplained perineal Bruising (Child abuse), crush injury(Rhabdomyolysis)
• H/o ingestion of drugs (ATT – Rifampicin, Ibuprofen, Chloroquine, Metronidazole, Iron), i.v. contrast agents (Toxic nephropathy)
• H/O Black colored Stool HSP/HUS
• H/o Vision or hearing defects: Alport syndrome
• Family h/o: Hereditary Nephritis(Alport syndrome), Thin glomerular Basement Membrane Disease, SLE Nephritis, IgA Nephropathy), Urolithiasis, PCKD, Sickle cell disease/trait
• H/o consanguinity or affected siblings in ARPKD, Metabolic disorders

Examination:

• Vitals:
  • Pulse
  • BP: in AGN, PKD
  • Temperature
• Edema: in AGN
• Pallor: HSP, HUS, SLE, CRF, Goodpasture Syndrome
• JVP: Raised in CHF
• Anthropometry
• Throat: pharyngitis
• Face
  • Malar flush
• Skin lesions: Purpura(HSP)
  Bruises (Trauma, Child abuse)
• Malformations: VATER (vertebral body anomalies, anal atresia, tracheo-oesophageal fistula, renal dysplasia
• Joint swelling, tenderness: HSP, SLE
• Ophthalmologic: Alport syndrome (Anterior lenticonus, macular flecks, recurrent corneal erosions), Aniridia (Wilms)
• Hearing assessment: Alport (B/L SNHL)
• Abdominal examination
  • Bladder distention in posterior urethral valves,
  • Abdominal masses- hydronephrosis, tumor, polycystic kidney
  • Bruit
  • Renal angle tenderness
  • Examination of the urethra, external genitalia

Abnormal external genitalia e.g. ambiguous genitalia in WAGR syndrome (Wilms, aniridia, genital anomalies, mental retardation)

• Chest/ CVS examination-
  • Cardiomegaly
  • Murmur (anemia, IE)
  • Pericarditis(CRF)
  • Lung field( Basal crepts-fluid overload)

Investigations:

Urine dipstick test

• It can detect trace amounts of hemoglobin and myoglobin.
• Can detect 5-10 intact RBC
• False +ve: Urine pH >9, H2O2
• False –ve: High ascorbic acid, formic acid
• Also for urine albumin

Urine microscopy: Presence of RBCs and casts (> 5 RBCs per HPF) in centrifuged urine

• Urine C/S
• RFT: Blood urea nitrogen/serum creatinine, Na/K (↓Na in AGN, ↑K in ARF)
• Complete blood counts (CBC): Hb – ↓ in bleeding, HUS, SLE, CRF; Abnormal TC, DC in infections, HUS, ↓ in SLE
• Platelet counts and Coagulation studies: (history suggestive of bleeding disorder, HUS), Sickle cell (Hemoglobin electrophoresis)
• PBS: Microangiopathic hemolytic anemia
• ESR, CRP – Infections
• 24 hr urinary protein, Spot urinary protein: Creatinine ratio, Serum albumin, and cholesterol if associated proteinuria (Nephrotic syndrome)
• Urine calcium: Hypercalciuria is a relatively common finding in children.
  • 24-hour urinary calcium (>4 mg/kg/d), or
  • Spot urine calcium-creatinine ratio >0.2
• Imaging Studies
  • Renal and bladder sonography: Urinary tract anomalies, such as hydronephrosis, hydroureter, nephrocalcinosis, tumor, and urolithiasis, Renal parenchymal disease
  • X-Ray KUB: calculi
  • Micturating cystourethrograms – Urethral and bladder abnormalities (eg, cystitis), in recurrent UTI to r/o VUR, anomalies
  • Intravenous urography
  • Radionuclide studies – Renal function and perfusion
  • Doppler study of renal vessels and IVC: Renal vein thrombosis
  • Spiral CT scan – Urolithiasis, Wilms tumor and polycystic kidney disease, Renal trauma
  • Angiogram
  • Chest X-Ray (Pulmonary edema, CHF)
• Renal biopsy is indicated for
  • children with persistent microscopic hematuria
  • children with recurrent gross hematuria associated with decreased renal

For Asymptomatic, isolated microscopic hematuria:

• The recurrent episode of Gross Hematuria
• IgA Nephropathy
• Alport Syndrome
• Thin Glomerular basement disease
• Hypercalciuria
• Urolithiasis

Management:

According to causes:

• Reassurance and F/U
• Treat cystitis, pyelonephritis, AGN: Antibiotics
• Supportive treatment: Diuretics, Fluid and salt restriction, Antihypertensives
• Monitoring – BP, I/O, weight, Urine R/M
• Treat Hyperkalemia, ARF, CHF, acidosis, fluid overload, HTN, and its complications
• ACE inhibitors useful in proteinuria
• Immunosuppressive therapy: Depending on a cause (Steroids, cyclophosphamide)
• Idiopathic Hypercalciuria: Hydrochlorothiazide, Potassium Citrate, Sodium Restriction
• Calculi: Plenty of water
• ESRD: Dialysis, Renal transplantation
• Correct thrombocytopenia, anemia, coagulation factor deficiency
• Renal vein thrombosis: Anticoagulant therapy or thrombectomy may be needed
• Surgical correction: Calculi, PUJ obstruction, Posterior urethral valves, Wilms tumor

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