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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 - PowerPoint PPT Presentation
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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
(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
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.
U/A 17/6/50 29/6/50
PH 5 5.5Sp.gr 1.020 1.020Alb 3+ 3+Sugar -ve -veOccult blood 3+ 3+RBC 100-200 >100WBC 10-20 10-20Granular cast 1-2
Blood chemistry17/6/50 3/7/50
Bun 54.6 24.1Cr 1.7 1.4Chol 226Na 130 134K 5.3 4.7Cl 111 107HCO3 12 18Alb 2.2Glo 2.6ALP 127TB 0.2DB 0
History
HistoryPI (cont.): Treatment at Pahol-ponpayuhasena as shown :-
-Fluid and Na Restriction-Input & Output monitoring-Ceftriaxone 50 MKD x 7 days-Antihypertensive drugs :
Hydralazine 4.5 mg IV Furosemide 30 mg IVEnalapril 2.5->5->10 mg/dHCTZ(50mg) 0.5 tab PO ODcontrolled BP between 110-120/70-80 mmHg
-Prednisolone 60 mg/day
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
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
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
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
Physical examination
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
Gross hematuria
R/O discoloration of urine Hemoglobinuria, myoglobinuria Drug eg. rifampicin…
Glomerular cause Extra glomerular cause
UTI Ureteric calculi Trauma Bleeding disorder
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
Glomerular causeMacroscopic hematuriaGeneralized edemaHypertensionDysmorphic RBCOval fat body
Oliguria BUN,Cr rising
RPGN
Acute renal failure
Acute nephritic nephrotic syndrome
Smoky urine
Rapidly Progressive Glomerulonephritis
(RPGN)
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
Signs & Symptoms
Acute nephritic syndrome eg. hematuria, fluid retention, hypertention, edema, oliguria
Azotemia eg.weakness, nausea and vomiting 50% - asymptomatic.
Pathogenesis
TNF,IL1
Mǿ,T-cell
Classification
1. Anti-GBM antibody disease
2. Immune complex disease
3. Pauci-immune disease
1. 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
2. Immune complex
Granular deposits of Ig in immunofluorescence and electron-dense deposits by electron microscopy
may be perinuclear ANCA (pANCA)–positive without myeloperoxidase (MPO) specificity
Primary glomerular disease IgA nephropathy Membranoproliferative glomerulonephritis
Multisystemic disease Lupus nephritis Collagen-vascular disease Henoch-Schonlein purpura
Post infection Poststreptococcal GN
2. Immune complex (cont.)
Idiopathic
Little or no deposits observed by immunofluorescence or electron microscopy
Wegener granulomatosis (WG) Churg-Strauss syndrome Microscopic polyangiitis (MPA) Renal-limited necrotizing crescentic glomerulonephri
tis (NCGN) 80-90% are ANCA-positive.
3. Pauci-Immune
Laboratory approach
ANCA-veANCA+ve
Low C3Pauci immune GN
Immune complex GN
Normal C3
Normal C3
Anti-GBM disease
Further special investigation
C3
ANCA ANA ASO Anti DNase
Serum C3
normal
Low
IgAHereditary disease
Wegener granulomatosisGood pasture’s syndromeHenoch-Schonlein purpura
MPGNShunt nephritisIEHepatitis B,C
Low
normal
Serum C4
SLEAPSGN
Normal Serum C3 83-177 mg/dL
C3 91.27 mg/dL (83-177 mg/dl) ANCA negative ANA negative ASO 63.7 IU/mL antiDNase B 95.2 U/mL
Further Investigation result
Serum C3
normal
Low
IgAHereditary disease
Wegener granulomatosisGood pasture’s syndromeHenoch-Schonlein purpura
MPGNShunt nephritisIEHepatitis B,C
Low
normal
Serum C4
SLEAPSGN
Normal Serum C3 83-170 mg/dL
Light microscope: diffuse endocapillary
proliferation fibrocellular cresent 6/21
glomeruli, normal capillary wall, no tubulointerstitial fibrosis
RPGN:immune complex type
RPGN:immune complex type
Immunofluorescence : diffuse granular stainning at
capillary wall of IgG, C3
RPGN:immune complex type
RPGN:immune complex type
Diagnosis
Postinfectious glomerulonephritis non streptococcus spp. Atypical presentation
Management
Supportive treatment control of volume status Antihypertensive drug
Specific therapy immunosuppressive therapy Pulses methylprednisolone plasma exchange (in life-threatening pulmonary h
emorrhage)
Progression Admit 5-12/7/50
Investigation
BUN 24 mg/dL Cr 1.0 mg/dL Na 135 mEq/L K 3.5 mEq/L Cl 103 mEq/L HCO321 mEq/L Alb/Glb 2.3/2.5 TB/DB 0.1/0 AST/ALT 28/26 ALP 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
Management
Record V/S, I/O, BW Low salt diet Enalapril(20) ½ tab OD keep BP<117/79
mmHg (P95) Prednisolone(5) 12tab OD10tab
OD(12/7/50)
Renal Biopsy 9/7/50
Progression
Date BP 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 / 87 900 1680 10/7 109 / 63 825 1790 11/7 105 / 58 1100 1840 12/7 116 / 54 1200 2400 13/7 121 / 70
C3 91.27 mg/dL (83-177 mg/dl) ANCA negative ANA negative ASO 63.7 IU/mL antiDNase B 95.2 U/mL Renal biopsycrescent fromation,
diffuse stainning of IgG, C3
Further Investigation result
Summary
Postinfectious GN, non streptococcus spp. Atypical presentation, normal C3 HM
prednisolone(5) 5 tab oral OD 14-20/7 then 4 tab oral OD 21-27/7.
Enalapril(20) ½ tab OD F/U at Pahol-ponpayuhasena Hospital , F/U U/A monthly
Take home massage
1. RPGN is a clinical syndrome of glomerular
disease.
Rapid decline of GFR (>50%) within 3 mo.
S&S of glomerulonephritis, oliguria or anuria
Nephrotic-nephritic urine sediment.
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
4. In Postinfectious glomerulonephritis, C3 level may be normal
5. If RPGN is suspected , the patient should be refer
Take home massage
Thank You