Background: Adrenal insufficiency in children is rare and potentially serious because of the risk of acute adrenal insufficiency. This complication is lethal in the absence of prompt and appropriate treatment. Aetiologies are dominated by the genetic causes.
Objective and hypotheses: Report diagnostic circumstances,phenotypic forms and causes of adrenal insufficiency in children and adolescents.
Method: This is a retrospective study of 54 cases of children and adolescents with adrenal insufficiency hospitalised between 1988 2014 we evaluated the clinical, biological, etiological and evolutive features of adrenal insufficiency.
Results: 54 cases were reported (44% boys, 56% girls) The average age was six years ±1.2 (218). The consultation Reason: ADS: 59%; adrenal insufficiency: 20%; Congenital adrenal hyperplasia: 5% melanodermia: 5% salt wasting syndrome: 3.7% early puberty: 1%. A familial form was observed in 14% (10% PEA, 4% HCS). Clinical feature was typical 42% (emaciation, asthenia, anorexia, hypotension melanodermia, digestive disorders). Paucisymptomatic in 58%. Biologically, the Serum potassium was high in 44.44% serum Na was low in 42.59%. glucose was low in 12.94% and symptomatic in 50%. The aetiologies were HCS 73% (21 OH 67% 11 OH 3% B OL 2% 17 βOH 1%) IS autoimmune 22.6% Allgrove sd 1.4%, tuberculosis 1.8%, adrenoleukodystrophy 1.8%. Replacement therapy has helped improve symptoms in all cases. The evolution was complicated iterative decompensation of 45% and difficulties of balancing (overdose 30%).
Conclusion: Adrenal insufficiency is rare in children. Genetic diseases dominated by HCS are the most common causes in infants and very young children. Dehydration with a salt-losing syndrome should suggest this aetiology. In the older child, the autoimmune origin is predominant. It can be isolated or associated with a polyendocrinopathy that it will identify early. Replacement therapy started quickly should be reviewed and monitored to prevent under and overdoses.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology