Extern conference - Mahidol University
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Transcript Extern conference - Mahidol University
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.
History
U/A
17/6/50
PH
5
Sp.gr
1.020
Alb
3+
Sugar
-ve
Occult blood 3+
RBC
100-200
WBC
10-20
Granular cast 1-2
29/6/50
5.5
1.020
3+
-ve
3+
>100
10-20
Blood chemistry
Bun
Cr
Chol
Na
K
Cl
HCO3
Alb
Glo
ALP
TB
DB
17/6/50
54.6
1.7
226
130
5.3
111
12
2.2
2.6
127
0.2
0
3/7/50
24.1
1.4
134
4.7
107
18
History
PI (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 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
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
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
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 cause
Macroscopic hematuria
Generalized edema
Hypertension
Dysmorphic RBC
Oval fat body
Acute nephritic nephrotic syndrome
RPGN
Oliguria
BUN,Cr rising
Acute renal failure
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
Mǿ,T-cell
TNF,IL1
Classification
1.
2.
3.
Anti-GBM antibody disease
Immune complex disease
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
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
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.
Laboratory
approach
ANCA+ve
Pauci immune GN
Normal C3
ANCA-ve
Normal C3
Anti-GBM disease
Low C3
Immune complex
GN
Further special investigation
C3
ANCA
ANA
ASO
Anti DNase
Serum C3
normal
IgA
Hereditary disease
Low
Serum C4
Low
normal
APSGN
Wegener granulomatosis
Good pasture’s syndrome
Henoch-Schonlein purpura
Normal Serum C3 83-177 mg/dL
MPGN
Shunt
nephritis
IE
Hepatitis
B,C
SLE
Further Investigation result
C3 91.27 mg/dL (83-177 mg/dl)
ANCA negative
ANA negative
ASO 63.7 IU/mL
antiDNase B 95.2 U/mL
Serum C3
normal
IgA
Hereditary disease
Low
Serum C4
Low
normal
APSGN
Wegener granulomatosis
Good pasture’s syndrome
Henoch-Schonlein purpura
Normal Serum C3 83-170 mg/dL
MPGN
Shunt
nephritis
IE
Hepatitis
B,C
SLE
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
hemorrhage)
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
(ml)
5/7
6/7
7/7
8/7
9/7(Bx)
10/7
11/7
12/7
13/7
BP max (mmHg)
Input Output
114 / 70
113 / 61
120 / 67
126 / 72
133 / 87
109 / 63
105 / 58
116 / 54
121 / 70
960 1700
600 1410
1200 2300
950 1580
900 1680
825 1790
1100 1840
1200 2400
Further Investigation result
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
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
Take home massage
4. In Postinfectious glomerulonephritis, C3 level
may be normal
5. If RPGN is suspected , the patient should be
refer
Thank You