[The cortico-resistent idiopathic nephrotic syndrome of child. Study of 14 observations].
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 alkylant agents in 11 cases (cyclophasplamide in 6 cases and chlorambucil in 5 cases) and ciclosparine 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.
Hachicha
Mongia
M
Service de Pédiatrie-CHU Hedi Chaker, Sfax, Tunisie.
Kammoun
Thouraya
T
Mahfoudh
Abdelmajid
A
Aloulou
Hajeur
H
Sfaihi
Lamia
L
Hammami
Yahia
Y
Zaghden
Nooman
N
Hachicha
Jamil
J
Triki
Ali
A
D000305:Adrenal Cortex Hormones / Q000494:pharmacology*; D000477:Alkylating Agents / Q000627:therapeutic use; D002648:Child; D003241:Consanguinity; D004351:Drug Resistance; D005260:Female; D006801:Humans; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology*; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D000477:Alkylating Agents