Nephrotic Syndrome

Ramadhina Anggita Suci Aulia

1310211059
FKUPN VETERAN JAKARTA

Nephrotic Syndrome
A disease results

from increased
permeability of
Glomerular
Basement
Membrane (GBM)
to plasma protein.

Glomerulonephritis

Glomerulus capillary loop with basement

membrane which allows passage of specific
molecules into the nephron
Glomerulonephritis inflammation/damage
of the glomerular basement membrane
resulting in altered function. Relatively
uncommon cause of kidney injury.
Can present as nephrotic and/or nephritic
syndrome.

Causes of Nephrotic Syndrome

Primary glomerulonephritis

Minimal change disease (80% paeds cases).

Focal segmental glomerulosclerosis (most
common cause in adults).
Membranous glomerulonephritis.

Systemic Causes

Secondary glomerulonephritis

Diabetic nephropathy.
Sarcoidosis.
Autoimmune: SLE, Sjogrens.
Infection: Syphilis, hepatitis B, HIV.
Amyloidosis.
Multiple myeloma.
Vasculitis.
Cancer.
Drugs: gold, penicillamine, captopril, NSAIDs.

Essentials of Diagnosis
1. Massive edema.
2. Proteinuria > 3.5 g/dL.
3. Hypoalbuminemia < 3 g/dL.
4. Hyperlipidemia: Cholesterol > 300 mg/dL.
5. Lipidiuria: Free fat, oval fat bodies, fatty

casts.

Classification of Nephrotic
Syndrome

A. Minimal Glomerular Lesion (>80%)

Light micro: normal.

Immunofluorescence: normal.
Electron micro: fusion of the foot
processes of the podocyte.
Renal function Remains Good.
B. Membranous Glomerulonephritis (2527%)
Light micro: glomerular capillary walls
thickening and swelling mesangial cell.
Proliferation (-).
Electron micro: irregular lumpy deposits
between glom. Basement membrane and
epithelial cell.

Classification of Nephrotic
Syndrome

C. Membranoproliferative

Glomerulonephritis /
Hypocomplementemic (5%)
Light micro: thickening of gomerular
capillaries, mesangial proliferation, and
obliteration of glomeruli.
Electron micro: subendothelial deposit,
growth of mesangium into capillary walls.
Immunofluorescence: presence of C3
complement and rarely immunoglobulin.
D. Proliferative Glomerulonephritis (5%)
stage in the course of poststreptococcal

Difference Between Nephritic and

Nephrotic Syndrome
Nephrotic
Syndrome

Nephritic
Syndrome

Definition

Increased permeability
of the glomerulus leading
to loss of proteins into
the tubules.

Thin glomerular
basement membrane
with pores that allow
protein and blood into
the tubule.

Criteria

*Massive proteinuria:
qualitative proteinuria:
3+ or 4+,
quantitative
proteinuria : more than
40 mg/m2/hr in children
(selective).

-Hematuria: RBC in
urine (gross hematuria)

*Hypo-proteinemia :
total plasma proteins <
5.5g/dl and serum
albumin : < 2.5g/dl.
*Hyperlipidemia:
serum cholesterol : >
5.7mmol/L

-Hypertension:
130/90
mmHg in
school-age
children
120/80
mmHg in
preschool-age
children
110/70
mmHg in
infant and
toddlers
children
-Azotemia renal

Symptoms and Signs

1. Edema appear and increase slowly
2.
3.
4.
5.
6.

(hydrothorax and ascites).

Fluid collects in serous cavities
abdoment becomes protuberant.
Anorexia.
Short of breath.
Hypertension.
Changes in retina and retinal vessel.

Pathogenesis and
Pathophysiology

Pathogenesis of Proteinuria
Increase glomerular permeability for proteins due to loss of

negative charged glycoprotein

Degree of protineuria:-

1. Mild less than 0.5g/m2/day

2. Moderate 0.5 2g/m2/day
3. Severe more than 2g/m2/day

Pathogenesis of Hypoalbuminemia
*Due to hyperproteinuria----- Loss of
plasma protein in urine mainly the
albumin.
*Increased catabolism of protein
during acute
phase.

Pathogenesis of Hyperlipidemia
*Response

to Hypoalbuminemia reflex to liver --

synthesis of generalize protein ( including
lipoprotein ) and lipid in the liver ,the lipoprotein
high molecular weight no loss in urine
hyperlipidemia

*Diminished catabolism of lipoprotein

Pathogenesis of Edema
*Reduction plasma colloid osmotic pressure
secondary to hypoalbuminemia Edema and
hypovolemia
*Intravascular volume antidiuretic hormone
(ADH ) and aldosterone(ALD) water and
sodium retention Edema
*Intravascular volume glomerular filtration
rate (GFR)
water and sodium retention Edema

How many pathological types

causes nephrotic syndrome?

Laboratory Findings
Urinalysis:
- proteinuria: 4-10 g or more/24 h.
- contain fatty droplets (oval fat bodies).
- Hematuria.
Blood Test:
- plasmanya lipemic.
- blood cholesterol greatly elevated.
- plasma protein is greatly reduced.
- reduction of gamma globulin in pure
nephrosis, but greatly elevated in SLE.

Differential Diagnosis for

Oedema

Congestive Cardiac Failure

Liver disease

Raised JVP, pulmonary oedema, mild

proteinuria
Hypoalbuminaemia, ascites/oedema

What investigations can you do?

You decide to send your patient to the renal
clinic...

Management

Conservative

Medical

Monitor U&E, BP, fluid balance, weight

Salt and fluid restriction
Treat underlying cause
Diuretics
ACE-inhibitors/ARBs
Corticosteroids/immunosuppression
Dialysis
Anticoagulation

Surgical

Renal transplant

Complications

Increased susceptibility to infection

Thromboembolism

20% adult cases

Due to reduced serum IgG, reduced
complement activity, reduced T cell function
40% adult cases
Partly due to increased clotting factors and
platelet abnormalities

Hyperlipidaemia

due to hepatic lipoprotein synthesis to restore

osmotic pressure

Prognosis

Varies
With treatment, generally good prognosis

Especially minimal change disease (1%

progress to ESRF)

Without treatment, very poor prognosis

Children under 5 or adults older than 30 =

worse prognosis

References;
1.
2.

3.
4.
5.

Oxford Handbook of Clinical Medicine

Oxford Handbook for the Foundation
Programme
Essential Revision Notes for the MRCP
Www.almostadoctor.com
Www.pathologystudent.com