NEPHROTIC SYNDROME

Question 1

What is nephrotic syndrome

Question 2

Types of nephrotic syndrome

Answer 2

1) Primary
2) Secondary

Question 3

Types of Primary Nephrotic syndrome

Answer 3

MINIMAL CHANGE DISEASE
MEMBRANOUS NEPHROPATHY
FOCAL SEGMENTAL GLOMERULOSCLEROSIS

Question 4

Types of secondary nephrotic syndrome

Answer 4

MINIMAL CHANGE DISEASE
MEMBRANOUS NEPHROPATHY
FOCAL SEGMENTAL GLOMERULOSCLEROSIS
DIABETIC NEPHROPATHY
AMYLOID NEPHROPATHY

Question 5

Causes of Nephrotic syndrome

Answer 5

MINIMAL CHANGE DISEASE
MEMBRANOUS NEPHROPATHY
FOCAL SEGMENTAL GLOMERULOSCLEROSIS
DIABETIC NEPHROPATHY
AMYLOID NEPHROPATHY

Question 6

Clinical manifestations of Nephrotic syndrome

Answer 6

HYPOALBUMINEMIA
EDEMA
HIGH LIPIDS
HYPERLIPIDEMIA
PROTEINURIA >3.5g/f
LIPIDURIA

Question 7

Complications of Nephrotic syndrome

Answer 7

ATHEROSCLEROTIC CARDIOVASCULAR DISEASES
XANTHOMA
XANTHELASMA
SECONDARY HYPERTENSION
DVT
RENAL VEIN THROMBOSIS
PULMONARY EMBOLISM
STREPTOCOCCUS PNEUMONIAE

Question 8

What is xanthoma

Answer 8

Fat deposition under the skin

Question 9

What is xanthelasma

Answer 9

Fat deposition around the eyes

Question 10

Diagnosis of Nephrotic syndrome

Answer 10

1) URINALYSIS WITH MICROSCOPY
Heavy proteinuria & lipiduria (fat oval bodies)
No hematuria, sterile pyuria

2) 24hr URINE PROTEIN / UACR (SPOT URINALYSIS)
Proteinuria>/=3.5g/d

3) SERUM ALBUMIN & LIPID PANEL
Hypoalbuminemia
Hyperlipidemia

4) CLINICAL MANIFESTATIONS (HINTS)
a) MCD
children
Infection
Hodgkin’s lymphoma
NSAIDS
b) MN
Caucasian adult
HIV
HBV, HCV
Syphilis
Gold/ Penicillamine meds
c) FSGS
African American, Hispanic adult male
HIV
Heroin use
Sickle cell disease
Obesity
d) DIABETIC MELLITUS
HbA1c>7%
e) AMYLOIDOSIS
SPEP or UPEP
Fat pad biopsy

5) RENAL BIOPSY
a) LIGHT MICROSCOPY
• MCD
Normal
• MN
Thick GBM
• FSGS
Focal hyalinosis & sclerosis if glomerulus
• DIABETES MELLITUS
Nodular sclerosis in glomerular space (KIMMELSTIEL-WILSON NODULES)
GBM thickening
Mesangial expansion
• AMYLOIDOSIS
Nodular sclerosis in glomerular space

b) ELECTRONE MICROSCOPY
• MCD
Podocytes effacement
• MN
podocytes effacement
Immune complex subendothelial deposition
Spike & dome GBM
• FSGS
Podocytes effacement
• AMYLOIDOSIS
Amyloid fibrils with apple green birefringence on congo red stain

c) IMMUNOFLUORESCENCE MICROSCOPY
• MCD
Normal
• MN
Granular green appearance of subendothelial immune complex deposits

Question 11

Treatment of Nephrotic syndrome

Answer 11

Treat complications
1) PROTEINURIA
Mechanism of proteinuria:
Hypoalbuminemia - edema - low circulating volume - RAAS activation - High AT II - high IGBP - high GFR - proteinuria
Treatment:
Limit protein intake (diet)
ACE Inhibitors
ARBs

2) HYPERLIPIDEMIA
Diet changes
STATINS

3) EDEMA
Fluid & Na+ restrictions
Diuretics

4) HYPERCOAGULABLE STATE
Anticoagulations

5) INCREASED INFECTION RISK
Pneumococcal vaccination
Reduce edema

6) TREAT UNDERLYING CAUSE
For primary MCD, MN, FSGS
STEROIDS
LONG TERM IMMUNOSUPPRESSANTS