Approach to Nephrotic Syndrome

Dr Abhay Mange

15 yrs old Male, Surendra Upwanshi

r/o Bitoli , Balaghat (mp) admitted on 7/2/14 with c/o

Swelling over face and lower limbs since 15 days

Decreased urine out put since 10 days

No h/o fever, sore throat, skin infection, rash, joint pain

No h/o Jaundice / hemoptysis

No h/o DM

On examination Conscious / oriented

Afebrile

Pulse -90 /min , all pulsation +

Resp- 18/min

Bp -180/100 mmhg

Edema feet +

Periorbital edema +

Jvp –nr

No pallor, icterus

P/A – ff +

RS –wnl

CVS –wnl

CNS –wnl

Investigations Hb -10.2 gm %

TLC – 8400/cumm

T- 71, L-27, E-2 , M-2 %

PS

RBCS-Normocytic,normochromic

WBCS- WNL,PLATELETS –adequate

No prasite seen

URINE EXAM

Protein = ++++

Sugar = negative

RBCS = 25-30/hpf

pus cells = 4-5/hpf

No cast seen

24 hr urine protein = 7930 gm

KFT- BUL=130 mg/dl ,

SC = 1.6 mg/dl

Na + = 136 meq/l

K + =5.6 meq/l

LFT TP = 5 gm

Total Cholesterol = 264 mg/dl

ECG- WNL

X-RAY CHEST –WNL

USG KUB

Rt. = 9.4 X 4.3 cm, Lt.=9.9 x 4.4 cm

Diffuse increase in echo texture ,maintained CMD

Provisional Diagnosis

Nephrotic syndrome

Renal biopsy planned

Investigations

ASO = 58 IU/L (Negative)

C3 = 0.3 gm/l (0.9 -1.8)

ANA = Negative

HBsAg = Negative

HCV = Negative

HIV = Negative

INR =1.2

Treatment Inj Cefotaxime 0.5 gm tds

Inj lasix 80 mg bd

Tab cilnidepine 20 mg tds

Tab prazocin 5 mg od

Tab calcium lactate 1 tds

Tab atorvaststin 10 mg

Human albumin

BIOPSY TAKEN

Inj Methylprednisolone 750 mg od 3 day

Tab prednisolone 50 mg od ct…

Renal biopsyMicroscopy : -

Glomeruli are enlarged in size and shows diffuse segmental areas of increase in mesangial matrix and hypercellularity and occasional infiltrating polymorph.

Focal areas of endocapillary hypercellularity are noted.

Focal areas of glomerular basement membrane thickening are noted.

Lobular accentuation is noted in 05 – 06 glomeruli.

Silver stain shows tram tracking.

The interstitium show mild mononuclear cell inflammatory infiltrate.

The blood vessels are unremarkable.

Immunofluorescence Study

IgG : Positive (++) coarsely granular diffuse

mesangial deposits are seen.

IgM : Negative

IgA : Negative

C3 : Positive (++) coarsely granular diffuse

mesangial deposits are seen.

Impression :- Membranoproliferative

Glomerulonephritis with IgG and C3 positivity.

Final diagnosis

Idiopathtic Membranoproliferative

glomerulonephritis

Nephrotic syndrome

Approach to acute Glomerulonephritis

DISCUSSION

Definition

Nephrotic syndrome is a clinical complex characterized by a number

of renal and extrarenal features, most prominent of which are

Proteinuria

(in practice > 3.0 to 3.5gm/24hrs),

Hypoalbuminemia,

Edema,

Hypertension,

Hyperlipidemia,

Lipiduria and

Hypercoagulabilty.

Classification

Nephrotic syndrome can be

Primary, being a disease specific to the kidneys,

Secondary, being a renal manifestation of a systemic

general illness

Primary causes

Primary causes include-

Minimal-change nephropathy(70-90% in children and 10-

15% in adult)

Focal glomerulosclerosis (15% in adult)

Membranous nephropathy (30% in adult)

Membranoproliferative glomerulonephritis .

Secondary causes

Secondary causes include-

Diabetes mellitus

Lupus erythematosus

Amyloidosis and paraproteinemias

Viral infections (eg, hepatitis B, hepatitis C, HIV )

Preeclampsia

Workup

Diagnostic studies for nephrotic syndrome may include the

following:

Urinalysis

Urine sediment examination

Urinary protein measurement (24-hr)

Serum albumin

Serologic studies for infection and immune abnormalities

Renal ultrasonography

Renal biopsy

Renal biopsy

Indications Unexplained renal failure

Acute nephritic syndrome

Nephrotic syndrome

Isolated nonnephrotic proteinuria

Isolated glomerular hematuria

Renal masses (primary or secondary)

Renal transplant rejection

Connective-tissue diseases ( SLE)

Renal biopsy

2 biopsy cylinders

• minimal length 1 cm

• diameter 1.2 mm

# isotonic saline – fast local transport

• cryopreservation of one piece for immunefluorescence

• fixation with paraformaldehyde or buffered (4%)

formaldehyde for paraffin embedding

• fixation with 3% glutaraldehyde for electron microscopy

or

# direct fixation with paraformaldehyde or formaldehyde and

shipping (indirect immunehistology by APAAP (alkaline

phosphatase) or others

Renal biopsy

Absolute Contraindications

Uncorrectable bleeding diathesis

Uncontrollable severe hypertension

Active renal or perirenal infection

Skin infection at biopsy site

Relative contraindications

Uncooperative patient

Anatomic abnormalities of the kidney which may increase risk

Small kidneys

Solitary kidney

Renal biopsy

Complications

Bleeding- may occur in 3 distinct locations

Collecting system -blood is seen in the urine,Obstuction

Under the renal capsule-cause increase in the release of renin-hypertension

Into the perinephric space-Hematoma

The injured kidney can also undergo fibrosis-chronic hypertension and perhaps even renal failure can result if the contralateral kidney is compromised – “page kidney effect”

AV fistules

THANK YOU