Approach to a child with hematuria
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(1) Hematuria refers to the presence of red blood cells in urine. (2) The causes of hematuria can be glomerular or extraglomerular and
Approach to a child with hematuria
- 1. Approach to a child with Hematuria : Sunil Agrawal 1st year MD Pediatrics, IOM
- 2. Contents: • Introduction • Pathophysiology • Causes • History • Examination • Investigations • Management
- 3. Introduction Hematuria means blood in urine. It may be Gross or macroscopic and microscopic hematuria Gross hematuria is seen by naked eyes while microscopic hematuria needs microscope. Hematuria is defined as the presence of at least 5 red blood cells per HPF of centrifuged urine and/or 5 RBC per microliter in uncentrifuged specimen
- 4. • Pathophysiology: Structural disruption in the integrity of glomerular basement membrane caused by inflammatory or immunologic processes Toxic disruptions of the renal tubules Mechanical erosion of mucosal surfaces in the genitourinary tract
- 5. Causes of Hematuria: A) Glomerular hematuria Isolated renal disease Multisystem disease -Postinfectious GN (Post streptococcal) -HSP nephritis - IgA nephropathy( Berger ds) -SLE nephritis - Alport syndrome( hereditary ) -HUS - Thin Glomerular Basement membrane -Wegener granulomatosis disease -Polyarteritis nodosa - Membranoproliferative GN -Goodpasture syndrome - Membranous nephropathy -HIV nephropathy - Focal segmental glomerulosclerosis - Antiglomerular basement membrane -Sickle cell glomerulopathy ds
- 6. B) Extraglomerular hematuria Upper Urinary tract Tubulointerstitial Pyelonephritis Interstitial nephritis ATN Papillary necrosis Nephrocalcinosis Anatomic Hydronephrosis Polycystic kidney disease Tumor (Wilms, Rhabdomyosarcoma, Angiomyolipoma) Trauma
- 7. Vascular: • Arterial/venous thrombosis • Malformation (aneurysms, hemangioma) • Nutcracker syndrome • Hemoglobinopathy (Sickle cell trait/disease) Crystalluria: Calcium, Oxalate, Uric acid Medications: NSAIDs, anticoagulants
- 8. Lower urinary tract Inflammation- infectious and non infectious Cystitis Urethritis Urolithiasis Trauma Coagulopathy Heavy exercise Bladder tumor Factitious syndrome / by proxy
- 9. Common causes of gross hematuria: • Urinary tract infection • Meatal stenosis • Perineal irritation • Trauma • Urolithiasis/hypercalciuria • Coagulopathy • Tumor
- 10. Glomerular cause of gross hematuria IgA nephropathy Alport syndrome Thin glomerular basement membrane disease Post infectious glomerulonephritis HSP nephritis SLE nephritis
- 11. Causes of Hematuria in the Newborn: • Renal vein thrombosis (Asphyxia, dehydration, shock) • Renal artery thrombosis • Autosomal recessive polycystic kidney disease • Obstructive uropathy • Urinary tract infection • Bleeding and clotting disorders • Trauma, bladder catheterization • Cortical necrosis (Hypoxic/ischemic perinatal insult) • Nephrocalcinosis (Frusemide in premature)
- 12. History: • Age: 2-5yrs: Wilms tumor 5-12yrs: PSGN • Sex: F>>M in >1-2yrs: UTI F>>M: SLE nephritis M>F : X-linked form of Alport syndrome • Race: whites: Idiopathic hypercalciuria blacks: Sickle cell disease
- 13. Colour of urine: Colour Causes Dark yellow Normal concentrated urine Dark brown or Bile pigments black Homogentisic acid, melanin, tyrosinosis, methemoglobinuria Cola coloured Glomerular hematuria Red or pink Extraglomerular hematuria, urine Hemoglobin, Myoglobin, Porphyrins, Chloroquine, Deferoxamine, Beets, blackberries, Rifampin, Red dyes in food, Urates
- 14. 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 urethra (Urethrorrhagia – spotting in underwear); Terminal (with suprapubic pain, disturbance of micturition) – from bladder
- 15. Associated Symptoms: • Fever: Infections, SLE, AGN • Facial puffiness, Oedema of legs, weight gain, Shortness of breath: Acute Glomerulonephritis • Hypertension (Headache, visual changes, epistaxis, seizures): AGN, ARF • Abdominal pain: Urolithiasis (Loin to groin), UTI, clots, Nutcracker syndrome • Painless: Glomerular • Abdominal mass: Hydronephrosis, PKD, Wilm’s tumour • Joint pain (HSP, SLE) • Rashes (HSP, SLE, PAN) • Neurologic – SLE, HUS (seizures, irritability) • Jaundice: Hemolysis, Obstructive jaundice
- 16. • H/o exercise, menstruation, recent bladder catheterization or passage of a calculus • Recent upper respiratory (1-2wks back), skin infection (3-6 wks): PSGN • GI infection: HUS, HSP nephritis • Gross hematuria precipitated by URI: Alport syndrome, IgA Nephropathy • H/o bleeding from other sites: Bleeding disorders, Hemoptysis in Good Pasture syndrome • H/o Trauma, abdominal surgery, Child abuse (Social factors - Munchausen), crush injury • H/o ingestion of drugs (ATT – Rifampicin, Ibuprofen, Chloroquine, Metronidazole, Iron), i.v. contrast agents (Toxic nephropathy, RVT)
- 17. • H/o Vision or hearing defects: Alport syndrome • Family h/o: Hereditary glomerular diseases (Alport syndrome, Thin glomerular Basement Membrane Disease, IgA Nephropathy), Urolithiasis, Hypercalciuria, Sickle cell disease/trait • H/o consanguinity or affected siblings in ARPKD, Metabolic disorders
- 18. Examination: • Vitals: – BP: in AGN, PKD – Temperature • Oedema: in AGN • Pallor: Bleeding disorders, HUS, SLE, CRF • JVP: Raised in CHF • Per abdomen: Mass – Kidney: Hydronephrosis (Urinary tract obstruction), Wilms tumour; B/L in ARPKD, hydronephrosis – Bladder palpable: Distal obstruction – Tenderness: HSP
- 19. • Skin lesions Purpura(HSP) Butterfly rash (SLE) Bruises (Trauma, Child abuse) • Abnormal external genitalia e.g. ambiguous genitalia in WAGR syndrome (Wilms, aniridia, genital anomalies, mental retardation), Wilms tumour – hypospadias, cryptorchidism, Genital trauma • Signs of Congestive cardiac failure, HTNsive encephalopathy: AGN • Joint swelling, tenderness: HSP, SLE • Ophthalmologic: Alport syndrome (Anterior lenticonus, macular flecks, recurrent corneal erosions), Aniridia (Wilms) • Hearing assessment: Alport (B/L SNHL)
- 20. General Approach to Investigate the child with Hematuria
- 21. Investigations: • Urine dipstick test: Based on the peroxidase-like activity of hemoglobin • It can detect trace amounts of hemoglobin and myoglobin. • Can detect 5-10 intact RBC per mm3 of unspun urine • False +ve: Urine pH >9, H2O2 • False –ve: High ascorbic acid, formalin • Also for urine albumin
- 22. Investigations: Urine microscopy: Presence of RBCs and casts (> 5 RBCs per HPF) in centrifuged urine Glomerular Non - glomerular 1. Brown, cola coloured or smoky 1. Bright red, pink 2. RBC casts 2.Terminal hematuria/ Passage of clots 3. Proteinuria 2+ or more 3. Proteinuria of < 2+ 4. Deformed urinary RBCs 4. Normal morphology of RBCs
- 23. Study on Evaluation of hematuria using the urinary albumin- to-total-protein ratio to differentiate glomerular and nonglomerular bleeding •N. Ohisa · R. Matsuki · H. Suzuki · H. Miura · Y. Ohisa · K. Yoshida Department of Clinical Laboratory, Tohoku University Hospital, Sendai, Japan •microscopy-based differentiation is not only tedious but the sensitivity and specificity may vary from one examiner to another. Furthermore, once the specimen has been frozen, differentiation becomes difficult. •A total of 143 random urine specimens from patients seen at the Division of Nephrology, Endocrinology and Vascular Disease and the Urology Department at Tohoku University Hospital were included in the study. The inclusion criterion for the study was a diagnosis of glomerular disease based on clinical features or histopathology with hematuria (5 or more RBCs per high-power fi eld). Of the 104 patients diagnosed with glomerular disease and 39 specimens from patients with nonglomerular disease, used as controls in the study.
- 24. • With respect to the albumin-to-total-protein ratio, the mean value in the glomerular disease group was 0.72 •} 0.10, whereas the mean in the nonglomerular disease group was 0.35 •} 0.17 (P < 0.001). The distribution of the albumin-to-total- protein ratio in the glomerular and nonglomerular disease groups was clearly differentiated. Sensitivity and specificity as a function of the albumin-to-total- protein ratio. At a ratio cutoff of 0.59, sensitivity and specificity was maximized at 96.2% and 100%, respectively.
- 25. Distribution of urinary albumin-to-total-protein ratio in glomerular and nonglomerular hematuria. The mean albumin-to-totalprotein ratio in the glomerular disease group was signifi cantly higher than that in the nonglomerular disease group. A cutoff of 0.59 excluded all nonglomerular disease cases, while detecting all but three glomerular disease cases. Gl, glomerular disease; non-Gl, nonglomerular disease; vertical axis, albumin-to-total-protein ratio; horizontal axis, glomerular disease, nonglomerular disease
- 26. Urine albumin-to-total-protein ratio sensitivities and specifi ities in differentiating glomerular and nonglomerular hematuria. At a ratio of 0.59, sensitivity and specificity were 96.2% and 100%, respectively.
- 27. Cola/ brown urine? Proteinuria(>30mg/dl)? RBC cast? Acute nephritic syndrome? Yes NO Glomerular hematuria Extraglomerular hematuria • CBC Step 1 • Electrolytes,Ca • Urine culture • BUN/Cr Step 2 • Serum protein/albumin • Urine Ca/Cr • Cholesterol • Renal/ bladder USG • C3/C4 Step 3 • ASO/Anti-DNase B • Urinalysis: siblings, parents • ANA • Serum electrolytes, Cr, Ca • Antineutrophil antibody • If crystalluria, urolitiasis or nephrocalcinosis: • Throat/ skin culture 24-hr urine for Ca, Cr, Uric acid, oxalate • 24-hour urine total protein creatinine clearance • If hydronephrosis/pyelocaliectasis: cystogram, renal scan
- 28. Investigations: • 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 >0.21
- 29. Investigations: • Imaging Studies – Renal and bladder sonography: Urinary tract anomalies, such as hydronephrosis, hydroureter, nephrocalcinosis, tumor, and urolithiasis, Renal parenchymal disease – X-Ray KUB: calculi – Doppler study of renal vessels and IVC: Renal vein thrombosis – Intravenous urography – Spiral CT scan - Urolithiasis, Wilms tumor and polycystic kidney disease, Renal trauma – Micturating cystourethrograms - Urethral and bladder abnormalities (eg, cystitis), in recurrent UTI to r/o VUR, anomalies – Radionuclide studies – Renal function and perfusion – Angiogram – Chest X-Ray (Pulmonary oedema, CHF)
- 30. Investigations: • Renal biopsy: Relative indications - • Significant proteinuria (3+ or more) or nephrotic syndrome + • Recurrent persistent hematuria (Microscopic >2yrs) • Abnormal renal function, Persistent HTN • Hematuria, Proteinuria, diminished renal function, low C3 level persist beyond 2 mo of onset of AGN • Absence of evidence of streptococcal infection • Serologic abnormalities (abnormal ANA or dsDNA levels) • A family history of end stage renal disease or evidence of Chronic renal disease in patient
- 31. Dx Histology Clinical Lab IgA IgA deposition in the Gross, No specific Nephro- mesangium, glomerular intermittent, changes, pathy sclerosis, proliferative changes, painless although crescents in severe cases hematuria increased serum IgA levels observed in some patients HSP Same as IgA nephropathy Purpura, joint No specific pains, laboratory data abdominal pain, hematuria, etc SLE Mild glomerulitis, proliferative Hematuria, Abnormal C3, C4, changes, immune complex proteinuria, ANA, dsDNA, deposition, crescents, hypertension, anemia, immunoglobulin deposition joint pains, thrombocytopeni rashes, etc a, etc
- 32. Dx Histology Clinical Lab Alport Some thinning of basement Sensorineural No Syndrome membranes, "basket weave" hearing loss, corneal specific changes in the glomerular abnormalities, changes basement hematuria, renal membrane on electron failure microscopy Thin Average glomerular basement Persistent No Basement membranes thin (reported to microscopic or gross specific Membrane be 100-200 nm) hematuria, changes disease significant family history Mesangio- Glomerular lobulations, Hematuria, C3 levels proliferative thickening of the mesangial proteinuria, may be GN matrix and glomerular hypertension low basement membranes, crescents, etc
- 33. Investigations: • Cystourethroscopy: Terminal hematuria, disturbances of micturition, suprapubic pain (Only if strong suspicion of bladder ulceration, tumours) • Screening of first degree relatives in persistent hematuria
- 34. Summary
- 38. For Asymptomatic, isolated microscopic hematuria: Isolated microscopic hematuria Repeat urinalysis weekly x 2 Negative F/U Urine R/M with (No exercise x 48hrs) examination Persistent hematuria Test parents for Positive Benign Familial hematuria Hematuria No Yes Family h/o calculi Urine Ca/Cr ratio No Normal +Hearing test, +USG, Yearly Urine R/M, +X-Ray KUB examination, BP
- 39. Management: • According to cause: – 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 cause (Steroids, cyclophosphamide)
- 40. Management: – 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 tumour
- 41. References: • Nelson Textbook of Pediatrics, 19th Ed • Nelson Essentials of Pediatrics, 6th Ed • O.P. Ghai Essential pediatrics, 7th Ed • Pediatric Nephrology, Shrivastava, Bagga, 4th Ed • Japanese Society of Nephrology 2007 • Indian J Pediatrics 1999; 66 : 207-214 • Various Websites