Nephrotic syndrome pdf - د. بسمة عادل

Nephrotic syndrome

Dr.Basma Adel
FIFTH GRADE 2017-2018

At the end of this lecture you
should know:

 Types
 Pathophysiology
 Clinical manifestations
  D.Dx.
 Investigations.
  Treatment .
 Complications.

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heavy proteinuria

edema

hypoalbuminemia

hyperlipidemia

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( However, this theory does not apply to all pts with NS).

Proteinuria & Hypoalbuminemia is due to
increase permeability of the glomerular capillary
wall

Edema and decreases plasma oncotic
pressure

transudation

fluid

from

the

intravascular

compartment

the

interstitial space

The reduction in intravascular volume



decreases renal perfusion pressure

Activation of  renin-angiotensin --
aldosterone  system     increase
reabsorption of  Na,H2O

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• Hyperlipidemia is due to hepatic lipoprotein

synthesis (stimulated by hypoalbuminemia)
combined with decrease lipid catabolism due
to urinary loss of lipoprotein lipase

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1- Idiopathic (most common 90%).
2- Secondary to other diseases or
syndromes.
3- Congenital.

NS can be divided into:

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It is divided into the following pathological types:

1.Minimal Change NS(85%) :characterized by effacement "fusion"
of the epithelial cell foot processes

2. Focal Segmental Glomerulosclerosis :characterized by
mesangialproliferation and segmental scarring

3.Membranous Nephropathy: characterized by thickening of
basement membrane with subepithelial deposits.

4. Other types include: Mesangial Proliferation &
Membranoproliferative Glomerulonephritis (type 1 & 2).

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Clinical manifestations

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usually occur between

2-6

yr.(MCNS), whereas FSGS is tend to
occur in older children.

: and subsequent

relapses may follow minor infections
or sometimes insect bite.

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Children usually present
With mild

periorbital

 lower extremities edema.

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Then edema becomes generalized with

development of ascites,

   pleural effusions, and

 genital edema

Other symptoms include:
irritability, anorexia,
abdominal pain & diarrhea

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D.Dx

Glomerulonephritidis,

II.

Protein-losing enteropathy.

III.

Protein malnutrition (kwashiorker)

IV.

Hepatic failure

Congestive HF.

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INVx:

• GUE for Proteinuria (3+ or 4+) or(> 40 mg/m2/hr) or

(>150 mg/kg/hr).

• Urinary protein/creatinine ratio (1st voiding at morning)

>2.

• Serum Albumin <2.5 g/ dL.

• Serum Cholesterol & Triglyceride (cholesterol >200

mg/dL).

• Serum Urea & Creatinin, C3 & C4 are typically normal.

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• Age <1 yr or >8 yr
• Family hx of renal disease,
• Extrarenal findings "

(arthritis, rash, anemia),

• hypertension,Hematuria,

Pulmonary edema,

• Renal insufficiency,

Hypocomplementemia

• Resistance to steroid Rx.

Renal

Biopsy is

not

routinely

indicated

unless :

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Treatment:

Corticosteroids are the cornerstone in Rx of NS

Prednisone orally, 60 mg/m2/day
(max. 80) as single dose in morning
For 4-6 wk ).

Then the dose should be tapered to
40 mg/m2 EOD for at least 4 wk,
then tappered & stopped over the
next 1-2 mo ( total 3-4 mo ).

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The response( -ve or trace proteinuria for 3 consecutive
days). to steroid usually occur within 2-4 wk of daily steroid
Rx

• Children with 1st episode with mild to moderate edema
can be managed as outpatient by the following (in addition
to prednisone):-

1. Salt intake "No added salt".

2. Diuretics orally ” with caution due to the risk of
thromboembolism.”

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• Children

with severe
edema,
should be
hospitalized
& managed
by the
following (in
addition to
prednisone)

1.Fluid restriction (in

addition to sodium

restriction) may be

necessary if the child is

hyponatremic

2.Swollen scrotum may be

elevated.

3. Diuretics e.g.

furosemide orally or IV,

1-2 mg/kg/dose.

4. If the above measures

are not effective, give

25% albumin IV (0.5-1

g/kg/dose) slowly

followed by furosemide

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• Relapse in NS: Many children with NS will

experience at least 1 relapse

• patients who are frequent relapsers, steroid

dependent or resistant or children suffer from
severe SE of corticosteroids are candidate for
alternative agents such as Cyclophosphamide,
Cyclosporine..ets.

• ACE inhibitors & Angiotensin II blockers may be
helpful as adjunct Rx to decrease proteinuria in
steroid-resistant patients.

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Complications:

1• Infection; it is the major Cx of NS, Spontaneous bacterial
peritonitis is the most frequent infection, although sepsis, pneumonia,
cellulitis, and UTI may also occur. S. pneumoniae is the most common
organism as well as other Gram -ve bacteria e.g. E. coli.

Vaccinations, especially "polyvalent" pneumococcal, varicella

& influenza vaccines can be given during remission or low

dose alternate day steroids

Note: Corticosteroid Rx usually mask fever and other signs

of inflammation,

thus it need high index of suspicion for infection combined

with aggressive Rx after Dx

2 • Thromboembolism; it is uncommon Cx due to high prothrombotic factors
(fibrinogen level, thrombocytosis, hemoconcentration, relative
immobilization) and low fibrinolytic factors (urinary losses of antithrombin III,
proteins C and S), thus overaggressive diuresis should be avoided .Anti-
coagulation Px is not recommended unless there is previous thromboembolic
event

3• Hyperlipidemia; CVS events e.g. MI is rare in children.

4• Psychological effects; patient with NS should not be considered as an "ill

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Secondary Nephrotic Syndrome

1. Glomerular diseases may have a nephrotic component e.g. Membranous
nephropathy; Membranoproliferative GN, PostinfectiousGN , SLE, and HSP

2. Infections & infestation e.g. HBV, HCV, HIV, Malaria and Schistosomiasis.

3. Drugs e.g. NSAIs, Penicillamine, Captopril, Phenytoin, Gold & Lithium.

4. Malignancy e.g. Lymphoma & Leukemia (but mainly in adults).

5. Syndromes that may associated with NS include: Alport syndrome, Hurler
syndrome, Alagille syndrome, Glycogen storage disease .... etc

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Congenital Nephrotic Syndrome

Appears at birth or within the 1st 3 mo pf life.
Causes of congenital NS include:-

1. Finnish-Type NS: It is the most common cause of

congenital NS, inherited as AR. It is manifested in utero

as fetal hydrops, large placenta high a fetoprotein;

prematurity, respiratory distress, hypothyroidism and

separation of cranial sutures.

The disease is persistent edema and progressive renal failure

with death by age of 5 yrs
Rx. Aggressive nutrition , ACE inhibitor, IV albumin, IVIG,

unilateral (or bilateral) nephrectomy, chronic dialysis, and

kidney transplantation; whereas corticosteroids &

immunosuppressives are of no value in Finnish-type NS.

2. Congenital Infections e.g. TORCHS can cause congenital NS

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