title: Isolated ACTH deficiency. Metabolic and endocrine studies in a 7-year-old boy.
abstract: Metabolic and endocrine studies on a 7-year-old boy who presented with hypoglycaemic convulsions are reported in detail, proving the diagnosis of isolated ACTH deficiency--a rare cause of hypoglycaemia in childhood. Adrenaline secretion during insulin-induced hypoglycaemia was reduced. Low blood alanine levels occurred during starvation-induced hypoglycaemia, together with raised total blood ketone bodies; blood glucose did not increase adequately after oral alanine at this time. Hypoglycaemia in isolated ACTH deficiency appears to be due to a combination of impaired alanine mobilisation and a decreased rate of gluconeogenesis.
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000172:deficiency*; D000409:Alanine / Q000378:metabolism; D002648:Child; D006801:Humans; D007003:Hypoglycemia / Q000209:etiology*; D007030:Hypothalamo-Hypophyseal System / Q000503:physiopathology; D008297:Male; D010913:Pituitary-Adrenal System / Q000503:physiopathology; D012640:Seizures / Q000209:etiology
chemical_list: D000324:Adrenocorticotropic Hormone; D000409:Alanine