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Approach to Hematuria and Proteinuria in Children

Approach to Hematuria and Proteinuria in Children. Adi Alherbish. Objectives. To be able to define and recognize hematuria and proteinuria To be able to generate a differential diagnosis of the commonest and most serious causes of hematuria and proteinuria

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Approach to Hematuria and Proteinuria in Children

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  1. Approach to Hematuria and Proteinuria in Children Adi Alherbish

  2. Objectives • To be able to define and recognize hematuria and proteinuria • To be able to generate a differential diagnosis of the commonest and most serious causes of hematuria and proteinuria • To have a clinical approach to both conditions.

  3. Case 1 • 14 year old boy presenting with red urine since last night. Otherwise healthy. Normal BP, no flank pain, no ankle edema. • What’s the next step?

  4. Case 1 • Urine dipstick: negative

  5. Case 2 • 5 year old boy presenting with pallor, and shortness of breath. Urine dip: SG 1.015, Hg 2+, Prot neg, Urinalysis: RBC 0, WBC 0

  6. Case 2 • CBC: Hg 80, WBC 5, Plt 180 • Retics: 3% Hemolytic Anemia • Send blood for: Hg electrophoresis, peripheral smear, Coombs test, G6PD

  7. Case 3 • 14 year old girl, healthy • Regular check up: Urine dip: SG 1.035, Hg 2+, Prot trace Urinalysis: RBC 5- 10 /HPF WBC 0- 5 / HPF

  8. Case 3 • Repeat urinalysis after drinking a bottle of water: Urine SG: 1.015 RBC: 1- 5 /HPF WBC: 0- 5 / HPF

  9. HPF= x 400

  10. Case 5 • 9 year old girl, presenting with fever, rash, coryza, conjuctivitis, and dark urine. • Urine dip: SG 1.015, Hg +3, Prot trace • Urinalysis: RBC > 100/ HPF WBC 10- 25/ HPF

  11. Case 5 • Urine positive for adenovirus

  12. Case 6 • 14 year old girl, presenting with intermittent, sudden onset left flank pain and dark urine. • Urine SG: 1.015, Hg 3+, Prot neg • Urinalysis: RBC 100/ HPF, WBC 0 Crystals present

  13. Case 6 • In clinic: send urine for Ca/ Cr ratio, citrate, oxalate, uric acid, cystine

  14. Case 7 • 14 year old girl, with hypertension, left knee arthritis, dark urine, malar rash • Urine dip: SG 1.010, Hg 2+, Prot 2+ • Urinalysis: RBC 10- 25/ HPF WBC 0 RBC casts

  15. Case 7 • Send blood for: C3, C4, ANA, anti-ds DNA

  16. Hematuria • Presence of > 5 RBC/ HPF, on more than two occasions, in the context of a normal urine specific gravity

  17. The 3 Vital Questions 1 Is it true hematuria? 2 Is it serious (urgent)? 3 What is the cause?

  18. Hematuria Hypertension Oliguria Increased Cr Is it serious? Nephritis Nephrosis • Edema • Nephrotic range proteinuria • Low albumin • Hypercholestrolemia

  19. Rapidly Progressive Glomerulonephritis (RPGN)

  20. Post- strep GN IgA nephropathy Lupus HSP RPGN Immune Complex Pauci- immune Anti- GBM • Wegner’s granulomatosis • Microscopic polyangiitis • Polyartritis nodosa • Goodpasture’s disease

  21. Post- strep GN IgA nephropathy HSP Lupus RPGN Immune Complex Pauci- immune Anti- GBM • Wegner’s granulomatosis • Microscopic polyangiitis • Polyartritis nodosa • Goodpasture’s disease • ASO, anti-DNase • Immunoglobulins • ANA, anti-ds DNA, • C3, C4 • ANCA • Anti- GBM

  22. Post strep Glomerulonephritis • Strep pharyngitis, or strep skin infection, followed 10 to 14 days by microscopic hematuria, nephritis, or nephrosis • Diagnosis: positive ASO low C3 which normalize in 8 weeks • Management: supportive • Prognosis: Excellent (Vog et. Al: 137 cohort- ESRD: none, high Cr 10%)

  23. IgA nephropathy • Typical presentation: intermittent gross hematuria that happen during colds • Other: gross hematuria microscopic hematuria nephritis nephrotic syndrome ESRD

  24. IgA nephropathy • Diagnosis: clinical suspicion IgA level 20% sensitivity! Kidney biopsy- IgA in Immunoflorecence • Treatment: supportive in mild cases ACEI in proteinuria Steroids

  25. Henoch Schonlein Purpura(HSP) • Pathology: IgA nephropathy • Clinical: - purpuric rash - arthritis - intestinal edema (intussusception) - hematuria/ nephritis/ nephrosis

  26. Hemolytic Uremic Syndrome • Pathogenesis: - typical (d+): E. coli O157:H7 shiga toxin 1 induced vascular injury - atypical (d-): alternative complement pathway defect • Clinical: triad of microangiopathic hemolytic anemia, thrombocytopenia, ARF

  27. Alport Syndrome(Hereditary Nephritis) • Homozygous mutation in genes encoding type IV collagen in basement membrane • Genetics: 80% X-linked AR, AD • Clinical: persistent microscopic hematuria, hearing loss, lenticonus

  28. Benign familial hematuria(thin basment membrane nephropathy) • Autosomal dominant • Hetrozygous mutation in type IV collagen • Microscopic hematuria • Screen the parents’ urine • Benign course

  29. Work up for hematuria(History is important!) 1. Gross hematuria: onset, duration, progression, aggravating, relieving factors, associated symptoms 2. UTI symptoms: dysuria, frequency, urgency, urge incontinence 3. Food intake: beet 4. Drugs: rifampin, nitrofurantoin, ibuprofen 5. IgA: gross hematuria onset while having colds 6. post strep: history of sore throat, tonsillitis, skin infection 7. HUS: diarrhea, pallor, fatigue, SOB 8. HSP: pupuric skin rash over legs and buttocks (palpable), join swelling/pain, abdominal pain/bloody stools 9. Goodpasture/Wegners: hemoptysis, cough, SOB 10. SLE/ vasculitis: butterfly rash, discoid rash, mouth ulcers, photosensitivity, CNS seizures/psychosis, join swelling 11. Kidney stones: renal colic, radiation to groins, past history or family history of stones 12. Anatomic: antenatal U/S, single umbilical artery, abdominal swelling 13 Hereditary: family history of deafness, family member with hematuria 14. Bleeding diathesis: hemarthrosis, epistaxis, petechaie, heavy periods in older girls 15. Problems with high blood pressure 16. Family history: nephritis, kidney failure, transplant, deafness, stones, hematuria, consanguinity

  30. Work up for hematuria • Nephritis: ASO, C3, C4, anti-ds DNA, ANA, ANCA, anti- GBM • Kidney and bladder U/S • Stone work up: urine Ca, Cr, oxalate, citrate, cystine, uric acid • Urinalysis in both parents • Bleeding tendency: PT, PTT, INR

  31. Proteinuria (Urine dip) • Negative < 10 mg/dl • Trace 10- 20 mg/dl • 1+ 30 mg/dl • 2+ 100 mg/dl • 3+ 300 mg/dl • 4+ 1000 mg/dl

  32. Urine prot/cr: > 20 mg/mmol 24 h urine collection: > 100 mg/m2/day > 4 mg/m2/hr Proteinuria (Quantitative) Non- nephrotic Nephrotic • Urine prot/cr > 200 mg/mmol • 24 h urine collection: > 1 g/m2/ day > 40 mg/m2/hr

  33. The 3 Vital Questions 1 Is it persistent? 2 Is it nephrotic? 3 What is the cause?

  34. Case 1 • 15 year old, athletic boy • Regular check up: Urine dip: Prot 2+ Urine prot/Cr ratio: 50 mg/mmol • What next?

  35. Case 1 • 8 am: urine prot/Cr ratio- 10 mg/mmol • 4 pm: urine prot/Cr ratio- 50 mg/mmol Orthostatic proteinuria

  36. Non Persistant Proteinuria • Fever • Strenuous exercise • Cold exposure • Epinephrine infusion • Orthostatic

  37. Case 2 • 1 year old infant with failure to thrive. Both height and weight are below the 3rd percentile. He has sings of rickets in exam. • Urine dip: Prot 3+ , Glu 2+

  38. Derakhshan Ali et al. Saudi J Kidney Dis Transpl. 2007 Oct-Dec;18(4):585-9.

  39. Fanconi Synrome • PCT defect • Proximal renal tubular acidosis (type II RTA) • Glucosuria • Aminoaciduria • Phosphaturia • hypokalemia

  40. Congenital: -Finish- type - TORCH infection Nephritis: - postinfectious GN - lupus - Wegner - HUS - Goodpasture Nephrotic: - Minimal change - FSGS - MPGN Drugs: captopril Neoplasia Renal vein throbosis Proteinuria Glomerular Tubualr absorption Protein overload • ATN • Fanconi Syndrome • Cystic/dysplastic • Interstial nephritis • Pyelonephritis • Hemolysis • Rhabdomyolysis • Light chain

  41. Congenital: -Finish- type - TORCH infection Nephritis: - postinfectious GN - lupus - Wegner - HUS - Goodpasture Nephrotic: - Minimal change - FSGS - MPGN Drugs: captopril Neoplasia Renal vein throbosis Proteinuria Glomerular Tubualr absorption Protein overload • ATN • Fanconi Syndrome • Cystic/dysplastic • Interstial nephritis • Pyelonephritis • Hemolysis • Rhabdomyolysis • Light chain • Urine electrophoresis: • Glomerular: albumin • Tubular: other proteins..

  42. Case 3 • 5 year old boy, presenting with puffy eyes, enlarged tummy, and feet swelling. • Exam: normal BP, ascites, pitting edema • Urine dip: Prot 4+ • What’s the next step?

  43. Case 3 • Urine prot/cr 1500 mg/mmol • Serum albumin 15 g/l • High cholesterol

  44. Nephrotic Syndrome • Urine Prot/Cr > 200 mg/mmol • Serum albumin < 25 g/l • Edema • Hyperlipedemia

  45. Nephrotic Syndrome • Minimal change disease • Focal segmental glomerulosclerosis • Membranoproliferative • Membranous GN • Infection: HIV, hepatits, syphilis • Lupus, Ig A, HSP, post strep

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