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