nephrotic syndrome

Nephrotic syndrome in childhoodDr. Nariman Fahmi/ 2014

objectives

Definition Pathogenesis Pathophysiology Clinical features Diagnosis Treatment prognosis

pathogenesis The underlying pathogenesis is unknown But evidence strongly supports the importance of immune mechanism

pathogenesis

Classification



Congenital Idiopathic (90%) secondary

pathology

Idiopathic nephrotic syndrome includes 3 histologic types: minimal change disease, mesangial proliferation, focal segmental glomerulosclerosis.

CLINICAL MANIFESTATION

Age : (2 - 6 )yr. The initial episode and subsequent relapses may follow minor infections

Diagnosis

GUE Appearance frothy Protein urea >++ Normal cast (hyaline cast) Pus cells if (UTI) 24h urine protien >1g/m/d



Hypoalbunemia Total protien (normal 6-8 g/dl) S.Albumin (normal 4-6g/l) Hyperlipidemia s.cholesterol >250 mg/dl All other investigations are normal

Treatment

Family understanding of their child's diseasereduced Sodium intake fluid restriction Treatment of infections-diuretic chlorothiazide (10 mg/kg/dose IV every 12 hr) ormetolazone (0.1 mg/kg/dose PO bid) Furosemide (1–2 mg/kg/dose IV q 12 hr).


IV administration of 25% human albumin (0.5 g/kg/dose . followed by Furosemide Indications Hypovolemic shook Sever anasarca potential complication of parentral albumin therapy Symptomatic volume overload, with hypertension and heart failure, is a, particularly with rapid infusions.

Treatment

Key drug steroid


Indications of renal biopsy before treatment Children with features that make MCNS less likely 1-hematuria 2- hypertension 3-renal insufficiency 4- hypocomplementemia 5- age <1 yr or >8 yr)



prednisone 60 mg/m2/day for at least 4 consecutive weeks. . Eighty to 90% of children will respond to steroid therapy


prednisone dose should be tapered later to 40 mg/m2/day given every other day as a single morning dose. The alternate-day dose is then slowly tapered and discontinued over the next 2–3 mo. steroid resistant, (renal biopsy should be performed.)Many children with nephrotic syndrome will experience at least 1 relapse (3-4+ proteinuria plus edema).

Course of disease

Remission :protein free urine steroid resistant continuous proteinuria (2+ or greater) after 8 wk of steroid therapy Relapse: relapse of (>+++ proteinuria )with oedema for 3 consecutive days.


Steroid-dependent patients may be candidates for alternative agents particularly if the child suffers severe corticosteroid toxicity (cushingoid appearance, hypertension, cataracts, and/or growth failure).


Other drugs 1-Cyclophosphamide 2-high-dose puls methylprednisolone. 2-Cyclosporine 3-Tacrolimus 4-Mycophenolate 5-Angiotensin-converting enzyme (ACE) inhibitors

COMPLICATIONS.

Infection thromboembolic events Hyperlipidemia

the major complication of nephrotic syndrome.

Spontaneous bacterial peritonitis is the most frequent type of infection, sepsis, pneumonia, cellulites', urinary tract infections may also be seen.


PROGNOSIS The majority of children with steroid-responsive nephrotic syndrome have repeated relapses. Children with steroid-resistant generally have a much poorer prognosis. These children develop progressive renal insufficiency.

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