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EXTERN CONFERENCE

EXTERN CONFERENCE. History. A 11-year-old Burmese boy CC : Generalized edema 21 days PTA. History. PI : 21 days PTA -He had generalized edema & fever -Dark-colored urine, increased in frequency of urination

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EXTERN CONFERENCE

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  1. EXTERN CONFERENCE

  2. History A 11-year-old Burmese boy CC: Generalized edema 21 days PTA

  3. History PI :21 days PTA -He had generalized edema & fever -Dark-colored urine, increased in frequency of urination (D:N 5:2 ->10:4) but decreased in amount of urine -Increased BW 3 kg Two days later -Admitted at Sangklaburi hospital for 4days, but did not respond to the treatment

  4. History PI (cont.) :15 days PTA, he went to Pahol-ponpayuhasena hospital Physical examination V/S:T 39˚C, BP 130/80 -150/100 mmHg,BW 29 kg, GA:Puffy eyelids Abdomen: ascites Skin: impetigo distributed at trunk and extremities.

  5. History U/A17/6/5029/6/50 PH 5 5.5 Sp.gr 1.020 1.020 Alb 3+ 3+ Sugar -ve -ve Occult blood 3+ 3+ RBC 100-200 >100 WBC 10-20 10-20 Granular cast1-2 Blood chemistry 17/6/503/7/50 Bun 54.6 24.1 Cr 1.7 1.4 Chol 226 Na 130 134 K 5.3 4.7 Cl 111 107 HCO3 12 18 Alb 2.2 Glo 2.6 ALP 127 TB 0.2 DB 0

  6. History PI (cont.) : Treatment at Pahol-ponpayuhasena as shown:- -Fluid and NaRestriction -Input & Output monitoring -Ceftriaxone 50 MKD x 7 days -Antihypertensive drugs : Hydralazine 4.5 mg IV Furosemide 30 mg IV Enalapril 2.5->5->10 mg/d HCTZ(50mg) 0.5 tab PO OD controlled BP between 110-120/70-80 mmHg -Prednisolone 60 mg/day

  7. History • Fever resolved after 2 days of treatment, He had persistent proteinuria so he was referred to Siriraj hospital for renal biopsy • He had no preceding URI symptoms and rash No diarrhea or GI bleeding, no dysuria No Hx of dark-urine, passing stone, No Hx of recurrent edema, no abdominal pain, No Hx of arthralgia, alopecia, photosensitivity or oral ulcer

  8. History Past Hx: Denial of underlying disease History of Malaria at 7 years old. Birth Hx: normal labour,no complication Family Hx: Healthy siblings.No Hx of renal disease Drug Hx: No history of drug allergy No history of drug usage Vaccination Hx: completed EPI program

  9. Physical examination V/S:T 37.2˚C,P 106/min,RR 20/min, BP110/61mmHg(BP<117/79 mmHg) (P95) Height 128 cm(P3), Weight 26.1 kg (P10-25) GA: Good consciousness, mildly pale, no jaundice, pitting edema 1+,puffy eyelids, no dyspnea Skin: no malar rash, no oral ulcer, no palpable purpura HEENT: no injected pharynx,no tonsilar enlargement,no exudate

  10. Physical examination CVS: peripheral pulse all 2+,normal s1 s2, no murmur RS: equal breath sound, no adventitious sound Abd: marked abdominal distension, soft, not tender, fluid thrill +ve, shifting dullness +ve no hepatosplenomegaly Extremities and joints: no sign of inflammation NS: within normal limits

  11. Problem list 1. Gross hematuria for 21 days 2. Generalized edema for 21 days 3. Decreased in amount of urine 4. Increased BW 3 kg 5. Hypertension 6. Proteinuria 7. Acute renal failure( BUN, Cr rising) 8. Hx of fever

  12. Gross hematuria • R/O discoloration of urine • Hemoglobinuria, myoglobinuria • Drug eg. rifampicin… • Glomerular cause • Extra glomerular cause • UTI • Ureteric calculi • Trauma • Bleeding disorder

  13. Investigation • CBC: Hb 10 g/dL Hct 31.8% MCV 79.1fL WBC 12,830/mm3(N 71.3% L 22.2% M 4.2% Eo 2.2%) Platelet 109,000/mm3 • UA: smoky urine, pH 6.0, sp.gr.1.015, protein4+, sugar –ve, occult blood 3+,leukocyte +ve, WBC 30-50, RBC >200,bact 1+, cast 0-1, oval fat body 1+ • Microscopic urine exam: numerous dysmorphic RBC, some oval fat body • Upr-24hr : 1.71 g (58mg/kg/d) • Spot Up/Ucr : 2.94

  14. Glomerular cause Macroscopic hematuria Generalized edema Hypertension Dysmorphic RBC Oval fat body Acute nephritic nephrotic syndrome RPGN Oliguria BUN,Cr rising Acute renal failure

  15. Smoky urine

  16. Rapidly Progressive Glomerulonephritis(RPGN)

  17. Definition • Clinical syndrome of glomerular disease • Rapid loss of renal function > 50% decline in GFR within 3 mo • Nephrotic-nephritic urine sediment • Extensive crescent formation

  18. Signs & Symptoms • Acute nephritic syndrome eg. hematuria, fluid retention, hypertention, edema, oliguria • Azotemia eg.weakness, nausea and vomiting • 50% - asymptomatic.

  19. Pathogenesis Mǿ,T-cell TNF,IL1

  20. Classification • Anti-GBM antibody disease • Immune complex disease • Pauci-immune disease

  21. Anti-GBM antibody disease • Goodpasture syndrome • Anti-GBM disease (only kidney involvement) • Pulmonary hemorrhage and hemoptysis due to Ab against the alveolar BM • Linear deposits of IgG (5-20%) • 10-40% may have positive ANCA findings

  22. 2. Immune complex • Granular deposits of Igin immunofluorescence and electron-dense deposits by electron microscopy • may be perinuclear ANCA (pANCA)–positive without myeloperoxidase (MPO) specificity

  23. 2. Immune complex (cont.) • Primary glomerular disease • IgA nephropathy • Membranoproliferative glomerulonephritis • Multisystemic disease • Lupus nephritis • Collagen-vascular disease • Henoch-Schonlein purpura • Post infection • Poststreptococcal GN • Idiopathic

  24. 3. Pauci-Immune • Little or no deposits observed by immunofluorescence or electron microscopy • Wegener granulomatosis (WG) • Churg-Strauss syndrome • Microscopic polyangiitis (MPA) • Renal-limited necrotizing crescentic glomerulonephritis (NCGN) • 80-90% are ANCA-positive.

  25. Laboratory approach ANCA+ve ANCA-ve Normal C3 Low C3 Pauci immune GN Normal C3 Immune complex GN Anti-GBM disease

  26. Further special investigation • C3 • ANCA • ANA • ASO • Anti DNase

  27. Serum C3 normal Low Serum C4 IgA Hereditary disease Low normal APSGN SLE MPGN Shunt nephritis IE Hepatitis B,C Wegener granulomatosis Good pasture’s syndrome Henoch-Schonlein purpura Normal Serum C3 83-177 mg/dL

  28. Further Investigation result • C391.27mg/dL (83-177 mg/dl) • ANCA negative • ANA negative • ASO 63.7 IU/mL • antiDNase B 95.2 U/mL

  29. Serum C3 normal Low Serum C4 IgA Hereditary disease Low normal APSGN SLE MPGN Shunt nephritis IE Hepatitis B,C Wegener granulomatosis Good pasture’s syndrome Henoch-Schonlein purpura Normal Serum C3 83-170 mg/dL

  30. Light microscope: • diffuse endocapillary proliferation • fibrocellular cresent 6/21 glomeruli, normal capillary wall, no tubulointerstitial fibrosis

  31. RPGN:immune complex type

  32. RPGN:immune complex type

  33. Immunofluorescence : • diffuse granular stainning at capillary wall of IgG, C3

  34. RPGN:immune complex type

  35. RPGN:immune complex type

  36. Diagnosis • Postinfectious glomerulonephritis • non streptococcus spp. • Atypical presentation

  37. Management • Supportive treatment • control of volume status • Antihypertensive drug • Specific therapy • immunosuppressive therapy • Pulses methylprednisolone • plasma exchange (in life-threatening pulmonary hemorrhage)

  38. ProgressionAdmit 5-12/7/50

  39. Investigation • BUN 24 mg/dL Cr 1.0 mg/dL • Na 135 mEq/L K 3.5mEq/L Cl 103 mEq/LHCO321 mEq/L • Alb/Glb 2.3/2.5 TB/DB 0.1/0 AST/ALT 28/26ALP 66 U/L GGT 13 U/L • Total cholesterol 206 mg/dL • Urine Cr 21.1 mg/dL,Urine protein 151 mg/dL • Urine protein/Urine Cr = 7.1 • PT 9.9 sec, aPTT 23.1 sec • Chest x-ray WNL

  40. Management • Record V/S, I/O, BW • Low salt diet • Enalapril(20) ½ tab OD keep BP<117/79 mmHg (P95) • Prednisolone(5) 12tab OD10tab OD(12/7/50) • Renal Biopsy 9/7/50

  41. Progression DateBP max (mmHg) Input Output (ml) 5/7 114 / 70 960 1700 6/7 113 / 61 600 1410 7/7 120 / 67 1200 2300 8/7 126 / 72 950 1580 9/7(Bx) 133 / 87 9001680 10/7 109 / 63 825 1790 11/7 105 / 58 1100 1840 12/7 116 / 54 1200 2400 13/7121/70

  42. Further Investigation result • C391.27mg/dL (83-177 mg/dl) • ANCA negative • ANA negative • ASO 63.7 IU/mL • antiDNase B 95.2 U/mL • Renal biopsycrescent fromation, diffuse stainning of IgG, C3

  43. Summary • Postinfectious GN, non streptococcus spp. • Atypical presentation, normal C3 • HM • prednisolone(5) 5 tab oral OD14-20/7 then4 tab oral OD 21-27/7. • Enalapril(20) ½ tab OD • F/U at Pahol-ponpayuhasena Hospital , F/U U/A monthly

  44. Take home massage 1. RPGN is a clinical syndrome of glomerular disease. Rapid decline of GFR (>50%) within 3 mo. S&Sof glomerulonephritis, oliguria or anuria Nephrotic-nephritic urine sediment.

  45. Take home massage 2. Initial management of RPGN is volume and blood pressure control , Pulse methylpredisolone is the drug of choice 3. Renal biopsy is the investigation of choice to identify the cause of RPGN

  46. Take home massage 4. In Postinfectious glomerulonephritis, C3 level may be normal 5. If RPGN is suspected , the patient should be refer

  47. Thank You

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