seminar on Haematuria

seminar on Haematuria

1. WELCOME To Seminar Dr Mehadi Hasan Rimon Resident , Year-1(neonatology ) Dr. Ishrat Zahan Nigar Resident, Year-2( Pediatric Neurology ) Dr. Zannatul Ferdous Resident , Year-2 (Pediatric Neurology)

2. Case Scenerio Redwan , 6 years old boy, 3 rd issue of his non consanguineous parents, got admitted with the complaints of generalized swelling of body for 7days along with scanty micturition and passage of red colored urine for same duration. O/E, he had puffy face with generalized oedema . He was mildly pale, anicteric , BP: 130/80mm of Hg (> 99 th centile ) & BSUA ++. Genitourinary system examination revealed abdomen was distended; ascites present. Provisional Diagnosis Acute Glomerulonephritis

3. Approach to a child with Hematuria

4. Definition Presence of red blood cell (RBC) in the urine, >10 RBC per cubic mm of freshly voided, uncentrifuged urine or >5 RBC per high power field of 10 ml of freshly voided centrifuged urine.

5. Physiological Pathological Gross Microscopic glomerular non glomerular non glomerular glomerular Heavy proteinuria Fever menstruation Classification painless Painful

6. Classification Painful Hematuria Painless Hematuria UTI Calculi Hypercalciuria IgA nephropathy Renal tumor Renal TB Coagulation disorder Alport syndrome Post traumatic

7. Classification Gross hematuria Microscopic hematuria UTI Infection related GN Renal calculi IgA nephropathy Hypercalciuria alport syndrome TBMD Coagulation disorder Thrombocytopenia malignancy Vasculitis membranous nephropathy IgA nephropathy TBMD alport syndrome idiopathic hypercalciuria renal vesical calculi urinary tract anomalies UTI PKD tuberous sclerosis wilms tumour sickle cell disease Penicilium lithium recurrent

8. Classification Glomerular Nonglomerular PSGN RPGN IgA nephropathy Vasculitis HSP nephritis Lupus nephritis Alport syndrome Membranous nephropathy TBMD HUS Hypercalciuria Renal calculi UTI Hemorrhagic cystitis Coagulation disorder Thrombocytopenia Malignancy Urinary tract anomalies Vascular malformation Trauma Exercise Drugs

9. Conditions mimicking hematuria Drugs: Rifampicin Phenothiazines Nitrofurantoin Desferoxamine Chloroquine Ibuprofen Levodopa

10. Foods: beet root, fruit containing anthocyanins ( eg . black berries, plums, cherries) and food dyes (cake coloring). Pigments: hemoglobinuria (intravascular hemolysis) myglobinuria (rhabdomyolysis) Bilirubin urate crystal (pink).

11. Approach to a child w ith Hematuria

12. History + symptom analysis Physical findings Lab investigations

13. History Age : 5-12 yrs : PSGN 2-8 yrs : HSP <4 yrs : HUS 2-5 yrs : Wilms tumor 2 nd decade: IgA nephropathy Sex : F >>M in > 1-2 yrs : UTI F >>M : SLE nephritis M>F : X-linked form of Alport syndrome Race : whites : Idiopathic hypercalciuria blacks : Sickle cell disease

14. History contd … Amount of urine : Reduced in AGN, AKI Clots in urine : Extraglomerular Frequency , Dysuria, recent enuresis : UTI Frothy urine : Suggests Proteinuria seen in Glomerular diseases Hematuria in relation to time of micturition : Initial stream – from urethra (Urethrorrhagia – spotting In underwear ) Terminal – from bladder (with suprapubic pain, disturbance of micturition) Throughout the urinary stream - glomerular

15. Associated Symptoms Fever Infection SLE AGN Facial puffiness Oedema of legs weight gain Shortness of breath Acute Glomerulonephritis

16. Abdominal pain Urolithiasis (Loin to groin) Cystitis (lower abdominal) Pyelonephritis (loin pain) Abdominal mass Hydronephrosis PKD Wilm’s tumour Joint pain Rashes HSP SLE Neurologic (seizures , irritability) SLE HUS

17. Jaundice Hemolysis, Obstructive jaundice APSGN HUS IgA nephropathy HSP nephritis Recent upper respiratory tract, skin infection, GI infection Bleeding disorders , Hemoptysis in Good Pasture syndrome H/o bleeding from other sites

18. H/o Vision or hearing defects Alport syndrome Family h/o hematuria Hereditary glomerular diseases ( Alport syndrome, Thin glomerular Basement Membrane Disease, IgA Nephropathy), Urolithiasis , Hypercalciuria , Sickle cell disease/trait H/o consanguinity or affected siblings ARPKD, Metabolic disorders

19. Others H/o exercise , trauma , menstruation , recent bladder catheterization or passage of a calculus H/o ingestion of drugs (ATT – Rifampicin, Ibuprofen, Chloroquine, Metronidazole, Iron), i.v. contrast agents (Toxic nephropathy, RVT)

20. Examination HTN AGN PKD Raised temp. Infection SLE AGN Oedema AGN Pallor HUS SLE CKD Bleeding disorders Raised JVP CHF

21. Palpable flank mass Hydronephrosis (Urinary tract obstruction) Wilms tumour ARPKD , hydronephrosis (B/L) Bladder palpable Skin lesions Abnormal external genitalia, hypospadias , cryptorchidism, Genital trauma Distal obstruction Palpable Purpura(HSP ) Butterfly rash (SLE ) Bruises (Trauma, Child abuse) WAGR syndrome Wilms tumour

22. Signs of Congestive cardiac failure, Hypertensive encephalopathy Joint swelling, tenderness Ophthalmologic (Anterior lenticonus, macular flecks, recurrent corneal erosions) AGN HSP, SLE Alport syndrome Hearing assessment (B/L SNHL) Alport syndrome

23. NEXT

24. Glomerular VS Non glomerular Features Glomerular hematuria Non-glomerular hematuria Pattern of hematuria: Hematuria throughout urinary stream. Initial, terminal. Urinary symptoms: Painless. Dysuria, urgency, frequency Associated feature: Sore throat, hypertension, edema. Fever, colicky pain. Family history: Deafness, renal failure. Renal stone, urinary infection. Clinical HTN, edema: Usually present Less common

25. Features Glomerular hematuria Non-glomerular hematuria color Tea colored. Bright red, blood clots. Proteinuria 2+ or more on dipstick; protein creatinine ratio>1. Trace to 1+; protein creatinine ratio<1. Dysmorphic RBC >20%. <15%, eumorphic . Cellular cast RBC cast, leukocyte or tubular cast may be present Absent Crystal absent May be present Glomerular VS Non glomerular Urinalysis

26. No No How to investigate a case of Hematuria P hysiological cause like viral illness, exercise, trauma, menstruation Take History & do physical examination Do Urine R/E including phase contrast microscopy Do a dipstick test & exclude ABCDE ( ABCDE are Dipstick – ve ) A= Alkaptonuria B= billirubinuria C= Carotinemia D=Drugs E= Endocrine/Metabolic- Porphyria +

27. Role of Urine examination A. Urinanalysis 1. Urine dipstick test Based on the peroxidase-like activity of hemoglobin. Positive in presence of- Hb , Myoglobin & RBC False + ve : Urine pH >9, H2O2 False – ve : High ascorbic acid, formalin 2. Urine R/M/E RBC, protein, RBC cast Pus cells, WBC cast Crystalluria Eosinophil Glomerular disease UTI Urolithiasis , Nephrocalcinosis Interstitial nephritis 3. Urine C/S Number & type of bacteria UTI RBC: False negative in case of low specific gravity or alkaline pH as it causes hemolysis . Phase Contrast microscopy >90-95% isomorphic >20% dysmorphic

28. No RBC Hemoglobinuria / Myoglobinuria RBC/+- Hb Hb only, no RBC Search other cause of red urine Nonglomerular Glomerular Check RBC Morphology

29. Urea, Creatinine , Electrolyte, ABG, ASO, C3, Spot urinary PCR, S Albumin, Cholesterol , Glomerular Hematuria No Consistent with PSGN HTN, Azotemia Supportive Treatment USG, ANA, anti ds DNA, Family history of blurring & visual examination, Renal biopsy

30. Positive Renal US +/- VCUG Non glomerular Hematuria Negative Pyelonephritis, Cystitis Hydronephrosis , PUJ, PUV, MCKD, Tumor, PKD,VUR, Lithiasis , RVT Abnormal Urine Culture Urinary Ca /Cr Normal Hypercalciuria Check 24-h Urine Bleeding disorder Meatitis Idiopathic Abnormal Normal

31. Isolated microscopic hematuria Follow up Urine R/E with examination For asymptomatic, isolated microscopic hematuria Repeat urinalysisis weekly × 2 No exercise × 48 hr F/H/O calculi Test parents for hematuria Benign familial hematuria ± hematuria, ± USG, ± X ray KUB region Urinary Ca /Cr ratio Urine R/E, examination, BP

32. Role of Imaging 1. Structure: USG of KUB X-Ray KUB Doppler study of renal vessels and IVC Intravenous urography Spiral CT scan Micturating cystourethrograms Renal cystic disease Hydronephrosis , Hydroureter , nephrocalcinosis , tumor, urolithiasis , Renal parenchymal disease calculi Renal vein thrombosis Nutcracker syndrome Urolithiasis , Wilms tumor and polycystic kidney disease, Renal trauma Urethral and bladder abnormalities ( eg , cystitis), in recurrent UTI to r/o VUR, anomalies 2. Function DTPA DMSA Functional excretory function. To see renal scar.

33. Significant proteinuria (>1g/1.73m2/day) except Post- infectious GN. Recurrent persistent hematuria (Microscopic >2yrs) Recurrent gross hematuria Abnormal renal function, Persistent HTN Hematuria , Proteinuria , diminished renal function, low C3 level persist beyond 2 mo of onset of AGN Serologic abnormalities (abnormal ANA or dsDNA levels) A family history of end stage renal disease. Indication of Renal biopsy

34. Urinanalysis of siblings & parents: Thin glomerular basement membrane disease Urine Ca/ Creatinine ratio: > 0.2 in Idiopathic hypercalciuria . 24hrs urine for Calcium, uric acid, oxalate : Urolithiasis & Nephrocalcinosis Cystogram & renal scan . Slit lamp examination of eye & audiometry .-Alport syndrome) How to screen families in a case of Hematuria Hereditary causes of hematuria : Benign familial Hematuria (Thin basement membrane disease) Alport Syndrome(Hereditary Nephritis) Idiopathic hypercalciuria Polycystic kidney disease Fabry’s disease Nail patella syndrome Sickle cell trait/disease

35. Management

36. Hematuria is a clinical (or laboratory) sign, not a diagnosis. Treatment is according to cause. Reassurance and F/U: isolated microscopic hematuria , familial microscopic haematuria . Antibiotics : To treat cystitis, pyelonephritis Supportive treatment: Proper nutrition Diuretics Fluid and salt restriction Antihypertensives General Management

37. Treatment of complication : Electrolyte abnormality, AKI, HF, acidosis, fluid overload, HTN. Monitoring: Vitals, I/O, weight, urine RME.

38. Management of some specific diseases

39. Adequate fluid intake Appropriate antibiotic Prophylactic antibiotic when indicated . UTI

40. Salt, fruit & fluid restriction Diuretics Antihypertensive Antibiotics Treatment of complication Prognosis 98% complete recovery within 1-2 weeks 1% immediate mortality 1% may proceed to chronic GN/CKD 0.7-7% recurrence PSGN

41. Prednisolone MMF, cyclosporine, azathioprim IV methylprednisolone , IV/oral cyclophosphamide Immunosuppressive in different combination Prognosis Not good 50% turn into CKD within 10 years 90% turn into CKD within 20 years Rx: RRT Membranoproliferative Glomerulonephritis

42. Treatment according to staging Immunosuppressive therapy Prednisolone Cyclophosphamide MMF Prognosis Relapse: 33% Childhood onset: worse prognosis SLE Nephritis

43. Mild disease: no specific therapy Persistent proteinuria : ACEi , corticosteroid Severe nephritis/ Nephrotic syndrome: corticosteroids & cytotoxic drugs. Crescentic glomerulonephritis : IV corticosteroids, cyclophosphamide , plasma exchange in nonresponsive cases. Prognosis 15-40% at least 1 recurrence <1% ESRD HSP Nephritis

44. ACE inhibitor Immune suppressive: prednisolone , azathioprime Omega 3 fatty acid, fish oil Prognosis Good in children RPGN: bad prognosis ESRD: 30-40% cases in 3 rd to 4 th decade Ig A nephropathy

45. ACE inhibitor Renal replacement Prognosis 60% ESRD by 60 years 40% ESRD by 40 years Alport Syndrome

46. Avoid dehydration High liquid intake Moderate salt restriction Avoidance of high protein(animal protein) diet Avoid excess dietary calcium Citrate supplementation Thiazide diuretics Idiopathic hypercalciuria

47. Usually benign. Episodic gross hematuria in association with acute infection. Prognosis: favorable. Follow up: annually for proteinuria & hypertension. Thin Basement Membrane Disease

48. Increased fluid intake Appropriate antibiotics Renal Stone Increased fluid intake Urological consultation Pyelonephritis

49. Every case of hematuria should be properly evaluated. Isolated asymptomatic microscopic hematuria can be an incidental finding. Most of the cases it possess no threat to health, only reassurance & F/U is needed. Gross & microscopic hematuria with family history/additional findings are usually pathological. Proper evaluation & family screening can elicit a diagnosis. With early diagnosis and proper treatment of most of the disease related to hematuria can be well managed. Take home message