syndrome neprotique idiopathique corticoresistant de l'enfant. Etude de 14 observations

ABSTRACT

Cortico-resistent nephrosis represent 10% of idiopathic nephrosis in nephrosis in children and progresses in almost half of the cases to end-stage renal failure. 14 cases of cortico-resistant idiopathic nephrotic syndrome, collected over a period of 13 years [1990 - 2002], are reported. This study involved 10 boys and 4 girls, aged 8 years on average. A history of allergy was present in two cases. Two other children had a family history of cortico-resistant nephrosis [CRN]. Consanguinity was found in 6 patients. The diagnosis of cortico-resistant idiopathic nephrotic syndrome [CRINS] was suggested by oedema in all cases. Two patients had a transitory high blood pressure and 5 others had a macroscopic hematuria associated with oedema. Laboratory studies confirmed the diagnosis of nephrotic syndrome, which was severe in 10 cases, and revealed a transitory renal insufficiency in 5 cases, a hyperlipidemia in 3 cases and signs of hyperaldosteronism in 12 cases. Renal biopsy practised in all patients shaved small glomerular lesions in 6 cases, a focal and segmental hyalinosis in 8 cases, one of which was associated with chronic tubulo-interstitial lesions. Treatment was based on alkylanting agents in 11 cases [cyclophosphamide in 6 cases and chlorambucil in 5 cases] and cyclosparine in 6 cases. 4 years later on average, the course was marked by complete remission in one case, relapse of the nephrotic syndrome in 3 cases, persistant CRINS without end-stage renal failure in 4 cases and progression to end-stage renal failure in 4 cases