CUSHINGS-SYNDROME IN CHILDHOOD AND ADOLESCENCE

Objective: To review the diagnosis, management and outcome of Gushing' s syndrome in children and adolescents. Methods: We conducted a retros pective review oi nine cases treated between 1976 and 1996 at the Roya l Children's Hospital, Melbourne, Australia. Results: Six children wit h Gushing's disease and three with primary adrenal disease were identi fied. Mean age at diagnosis in the Gushing's disease patients was 11.3 years and in the children with primary adrenal disease 9.5 years, The most common presenting symptoms were weight gain and delayed growth, Two children had the unusual presenting symptoms of an eating disorder and hemihypertrophy, respectively, Laboratory diagnosis of Gushing's syndrome was established by demonstration of elevated urine free corti sol, loss of normal diurnal variation of serum cortisol, and loss of s uppressibility of cortisol secretion by low dose dexamethasone. Invest igations used to determine the aetiology of hypercortisolism included serum adrenocorticotropic hormone (ACTH) levers, high dose dexamethaso ne suppression tests, imaging studies, and inferior petrosal sinus sam pling. Four patients with Gushing's disease had successful transphenoi dal adenomectomies. Two patients with bilateral primary pigmented nodu lar adrenocortical dysplasia underwent bilateral adrenalectomies. One child with an adrenal adenoma was treated by left adrenalectomy. Concl usions: Gushing's syndrome in children and adolescents remains a diagn ostic challenge, Successful treatment often requires the use of multip le tests to achieve the correct diagnosis, appropriate surgery and a g ood long-term outcome.