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NAZURAH

Nephrotic Syndrome..…(NS)Nephrotic Syndrome..…(NS)

Nephrotic syndromeNephrotic syndrome

Proteinuria

Proteinuria >40mg/m2/hour or an early morning ur pr creatinine index >200mg/mmol

EdemaHypoalbuminemia Hypercholesterolemia

Classification:

A-Primary Idiopathic NS (INS): majority

Accounting for 90% of NS in child. mainly discussed.

Unknown cause

B-Secondary NS:

Include post streptococcal glomerulonephritis and SLE

1.The construction of the

glomerular basement memb-

rane has changed.

2.The loss of the negative

charges on the GBM.

Pathophysiology:

The Main Trigger Of primary Nephrotic Syndrome and Fundamental and highly important change of

pathophysiology :-

Proteinuria

Pathogenesis of Proteinuria:-

Increase glomerular permeability for proteins due to loss of negative charged glycoprotein

Degree of protineuria:- Mild less than 0.5g/m2/day Moderate 0.5 – 2g/m2/day Sever more than 2g/m2/day

Type of proteinuria:- A-Selective proteinuria: where proteins of low molecular

weight .such as albumin, are excreted more readily than protein of HMW

B-Non selective : LMW+HMW are lost in urine

How many pathological types causes nephrotic syndrome?

Investigations:-

1-Urine analysis:-

A-Proteinuria : 3-4 + SELECTIVE.

b-24 urine collection for protein>40mg/m2/hr for children

c- volume: oliguria (during stage of edema formation)

d-Microscopically:-

microscopic hematuria 20%, large number of hyaline cast

Investigations:-

2-Blood: A-serum protein: decrease >5.5gm/dL , Albumin levels are

low (＜ 2.5gm/dL).

B-Serum cholesterol and triglycerides: Cholesterol ＞ 5.7mmol/L (220mg/dl).

C-- ESR↑ ＞ 100mm/hr during activity phase

. 3.Serum complemen: Vary with clinical type.

4.Renal function

.

Kidney Biopsy:-

Considered in: 1-Secondary N.S

2-Steroid resistant N.S

3- Gross Hematuria

4-Hypertension

5- Renal Impairment

Complications of NS:-

1-Infections:Infections is a major complication in children with NS. It frequently trigger relapses.

Nephrotic pt are liable to infection because :A-loss of immunoglobins in urine.B-the edema fluid act as a culture medium.C-use immunosuppressive agents.

D- malnutrition

The common infection : URI, peritonitis, cellulitis and UTI may be seen.

Organisms: encapsulated (Pneumococci, H.influenzae), Gram negative (e.g E.coli

Complication…..

2-Hypercoagulability (Thrombosis). Hypercoagulability of the blood leading to venous or arterial

thrombosis: Hypercoagulability in Nephrotic syndrome caused by:

1-Higher concentration of I,II, V,VII,VIII,X and fibrinogen

2- Lower level of anticoagulant substance: antithrombin III

3-decrease fibrinolysis.

4-Higher blood viscosity

5- Increased platelet aggregation

6- Overaggressive diuresis

3-ARF: pre-renal and renal

4- cardiovascular disease :-Hyperlipidemia, may be a risk factor for cardiovascular disease.

5-Hypovolemic shock

6-Others: growth retardation, malnutrition, adrenal cortical insufficiency

Management of NS:

General (non-specific )

Corticosteroid therapy

General therapy:-

Normal diet with adequate caloriesNo added salt to the diet whn child has

edemaAvoiding infection: very important.Penicillin V is recommended at diagnosis

and during relapses Severe edema: Restricting fluid intake

Human albumin (20-25%)- symptomatic

grossly edematous together with IV frusemide(diurresis)

GENERAL ADVICE

Home urine albumin monitoring (1st urine specimen)

Consult doctors if 1)albuminuria >= 2+ for consecutives day or out 7 days.

2)edematous

Immunisation

on corticosteroid treatment and within 6 weeks (killed vacines)

after 6 weeks cessation (live vaccine)

pneumococcal vaccine

Corticosteroid—prednisone therapy:-

REMISSION : Urine dipstick trace or nil for 3 consecutives days within 28 days.

RELAPSE: Urine albumin excretion > 40mg /m2/hour or urine dipstick >= 2+ for 3 consecutives days

FREQUENT RELAPSES : >= 2 Relapses within 6 month of initial diagnosis or >= 4 relapses within 12 month periods

STEROID DEPENDENT NEPHROTIC SYNDROME : >= 2 Consecutives relapses occuring during steroid taper or within 14days of cessation of steroid

Side Effects With Long Term Use of Steroids “Steroid toxicity

hyperglycemiamyopathypeptic ulcerpoor healing of wound.HirsutismThromboembolism

-Stunted growth

Cataracts

- Pseudotumor cerebri

-Psycosis

-Osteoporosis

- Cushingoid features

-Adrenal gland suppression

Alternative agent:-

When can be used:

Steroid-dependent patients, frequent relapsers, and steroid-resistant patients.

Cyclophosphamide Pulse steroids

Cyclosporin A

Tacrolimus

Microphenolate

Treatment

Cytotoxic drugs with corticosteroid: (for steroid dependent or steroid resistant)Cyclophosphamide (CTX): p.o. or intravenously Side effects: liver injury, inhibition of bone marrow, etc.

Cyclosporine (for those failed responsing to combination of steroid and cytotoxic

drugs)Dose: 5mg/kg/d, bid, p.o.Side effects: renal and liver toxic injury, expensive, etc.

Treatment

Mycophenolate mofetil, MMF (for steroid dependent or steroid resistant)

Dose:1.5-2g/d, bid, p.o. for 3-6 months, maintaining 0.5 year

THE END….

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