Nephrotic Syndrome Johnny

Nephrotic Syndrome
Ho Yan Sheng 1301-1208-3006

THE NEPHROTIC SYNDROME
Definition:
Abuminuria > 3- 3.5 grams/day

Hypoalbuminemia
Edema
Hyperlipidemia
(Not all need to be present)

Hypoalbuminemia:
Multifactorial, with urinary protein loss

Increased albumin catabolism in PT.
Redistriubtion.
Edema:
1) Protein oncotic pressure loss of plasma
water into interstitial space
2) direct effects of proteinuria on the distal tubules to

stimulate salt/water retention.
Not from intravascular depletion and RAS activation.

Brief Overview of Proteinuria
Normal proteinuria: < 150mg / day.
Mainly Tamm-Horsfall protein( tubular cell protein)
Proteins are restricted based on size & charge
< 20 kDa can pass and are reabsorbed/catabolized

(b2-microglublin, light chains etc.)
Proteins b/w 40-120 kDa (intermediate size) can be

found only if the barrier to filtration is lost.
Abnormal Proteinuria
1) TUBULAR:
Inability to reabsorb proteins normally found in Prox tubules
secondary to renal dz.
(eg: Fanconi Syndrom )
2) OVERFLOW:
Overproduction of plasma proteins and therefore exceed renal
handling capacity
(MGUS, Myeloma, trauma, sepsis, myoglobin)
3) GLOMERULAR:
a defect in glomerular barrier that allows passage of proteins.
Proteinuria in Nephrotic Syndrome
Glomerular Proteinuria:
Disruption of the Glomerular Basment membrane
leads to protein leakage
Nephrin: Transmembrane protein respsonble for cell-

cell/matrix adhesion and integrity of the slit diaphragm.
Gene expression is reduced in NS.
Neurohormonal mechanisms: Thromboxane etc

ETIOLOGY & CLASSIFICATION
Primary / Idiopathic
Secondary -
1) Systemic Dz (DM, SLE, Amyloid, Vasculitis)
2) Infections (Bacterial, Viral, Parasitic)
3) Meds (NSAIDS, Captopril, Lithium, Heroin)
4) Malignancy (Solid tumours, Hodgkins, NHL)
5) Others(Hereditary, sickly, pregnancy etc.)
PRIMARY NEPHROTIC SYNDROME
Histologic/Pathologic classification
THE APPROACH
Step 1: Primary VS Secondary
Anam & Physical and basic labs (cbc, creat)

Glucose/A1C, ANA, Complement, ANCAs
Hep B/C, VDRL
Immunoglobulins
24hr protein collection.
Renal Ultrasound
1) MEMBRANOUS Glomerulopathy
# 1 cause of idiopathic NS in adults

M:F 2:1
Present with NS and nonselective proteinuria.
Micr. Hematuria 50%.
HTN 25%
Etiology:
Idiopathic(majority).
Infections:
Hep B/C , Syphilis, malaria, endocarditis and others.
Autoimmune
SLE, RA and other CVD.
PBC (6 cases), ank spondylitis, graves
Malignancy
Breast, lung, colon & others.
Drugs
LM: Thickening of GBM with spikes representing new
basement membrane & subepithelial deposits.
Th/: Steroids.
Prognosis:
40% Spontaneous remission.
40% Repeated relapses
20% slow decline in GFR over 10 years.
2) MINIMAL CHANGE DISEASE (Lipoid Nephrosis)
80% of NS in ages < 16

20% of NS in adults
NS and bland urinary sediment (i.e no casts)
Microscopic hematuria in 25%
HTN, renal failure is rare.
Etiology:
Idiopathic (majority)
Secondary: Drugs (NSAIDS, rifampin),
Malignancy (Hodgkin/NHL>>>solid), viral
infections.
EM shows effacement of foot processes on the

visceral epithelial cells (foot process fusion).
Tx: Steroids(3 mo)if fail response/freq relapse +/-

cyclophosphamide (induce long lasting remission)
Prognosis: 50% remission in adults after 8weeks of

pred
TX
single morning dose of 1 mg/kg/day, maximum of 80 mg, is continued for a
minimum of 8 weeks.
For those patients not in remission at 8 weeks, daily prednisone may be
continued for another 2 months
until remission is attained
Complete Remission
Use dipstick >>>a complete remission is defined as a reduction of
proteinuria to #300 mg of urinary protein in a 24-hour period
Supportive
A no-added-salt diet is always recommended for patients with MCDit will
reduce edemaformation and reduce the tendency to develop hypertension.
In addition, angiotensinconverting enzyme (ACE) inhibitors and/or
angiotensin receptor blockers (ARBs) are useful in ameliorating proteinuria
and are the first choice for the treatment of hypertension if it appears in
these patients
3)Focal Segmental Glomerular Scelerosis
FSGS
Increasing incidence ~ 20-25% in adults

Blacks >> whites : 50% of NS in blacks.
Usually presents as nephrotic syndrome with
hypertension (40%)
Mild renal failure (30%)
Microscopic hematuria (50%)
Etiology:
Idiopathic(majority)
Systemic Dz.
Seconary to sustained glom.capillary htn.( congenital, reflux,
chronic nephritis, sickle cell)
Prognosis:
Spontaneous remission: rare
70% are steroid responsive but relapse common.
Treatment:
Steroids
Complications of Nephrotic Syndrome.
HyperLipidemia
Profile: High LDL, VLDL and low/norm HDL.
Suggestions: Treat if duration of NS is anticipated

to be prolonged. ACE inhibitors work +/- statins
Thrombosis
Multifactorial pathogenesis.
35% have Renal vein thrombosis in membranous.
RVT is usually asymptomatic.
DVT / PE range from 10-30% .
Dx: Gold standard is renal venography, but ct/mri
are useful ( moreso for chronic RVT).
Prophylaxis: no rctsbut suggested in
membranous nephropathy
Nephrotic Syndrome: An Acquired Hypercoagulable
State
1. Low ATIII: increased clearance

2. Low functional protein S: decreased clearance
of C4BP; increased clearance of free protein S
3. Increased levels of factors V, VII, VIII, vWf, and
fibrinogen
4. Increased lipoprotein (a)
ANEMIA:
Uncommon
microcytic
Unclear mechanism : ? Epo dysfuntion ?
Transferrin loss in urine?
Hypocalcemia from low Vit D.
Hypoglobulinemia
TREATMENT
1) Treat primary process (i.e DM, SLE, Cancer )
2) Control Proteinuria
ACE/ARBs: upregulate Nephrin and reduce proteinuria
Proteinuria is toxic to kidneys and can lead to RF.
3) Control Complications of NS
Edema: Salt restricition and loop diuretics
Control lipids ? Poor evidence but often used.
Anti-coag if present with thromboembolic dz.
Nephrotic Syndrome Diet
General Guidelines: Foods that can be taken:
Cow's milk, skimmed milk
The main aim of nutritional
management of Nephrotic Yogurt
syndrome is to replace the protein Wheat, cereals, sprouts,

loss by having an adequate intake pulses and legumes.
of proteins. However high intake of
Eggs, fish, dry fish,
protein must be avoided to prevent chicken, lean meat, etc.
any tubular damage to the kidneys Vegetables and fruits
caused by filtering of the excess Soups, sauces, chocolate
proteins. drinks, juices, etc (but with
low sodium content)
Sodium intake in diet should be
Wafers, popcorns,
low. chutneys which are
prepared in less salt.
Fat intake should also be low.
Moderate to low intake of
Fluid intake should be restricted. vegetable oils, butter and
mayonnaise.
Noodles, spaghetti,
pancakes, etc (low in salt)

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Nephrotic Syndrome

Ho Yan Sheng 1301-1208-3006

Abuminuria > 3- 3.5 grams/day

(Not all need to be present)

Multifactorial, with urinary protein loss

2) direct effects of proteinuria on the distal tubules to

Not from intravascular depletion and RAS activation.

Proteins are restricted based on size & charge

< 20 kDa can pass and are reabsorbed/catabolized

Proteins b/w 40-120 kDa (intermediate size) can be

Nephrin: Transmembrane protein respsonble for cell-

Neurohormonal mechanisms: Thromboxane etc

Step 1: Primary VS Secondary

Anam & Physical and basic labs (cbc, creat)

# 1 cause of idiopathic NS in adults

80% of NS in ages < 16

EM shows effacement of foot processes on the

Tx: Steroids(3 mo)if fail response/freq relapse +/-

Prognosis: 50% remission in adults after 8weeks of

Increasing incidence ~ 20-25% in adults

Profile: High LDL, VLDL and low/norm HDL.

Suggestions: Treat if duration of NS is anticipated

1. Low ATIII: increased clearance

syndrome is to replace the protein Wheat, cereals, sprouts,

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