title: [Lack of ACTH responsiveness in children and paroxysmal central nervous system manifestations. (Study of nine cases of isolated ACTH deficiency) (author's transl)].
abstract: Study of ACTH responsiveness to oral metyrapone and insulin hypoglycemia in children with repetitive nervous system manifestations (convulsions, coma, mental confusion apathy, tremor) has led to diagnosis of isolated ACTH deficiency in nine children within a three year period. Hypoglycemia was ascertained in five children; in four cases no hypoglycemia was proved, possibly because of promptly disappearance or because of other mechanisms accounting for clinical symptoms (occurrence of intracellular overhydratation associated with corticol deficiency is considered). The incidence of isolated ACTH deficiency in children is possibly undervalued. Reappraisal of isolated ACTH deficiency in childhood as to be considered in idiopathic spontaneous hypoglycemia and perhaps in some paroxysmal neurologic and/or digestive manifestations without proved hypoglycemia and so far poorly defined or held for epileptic fits. In order to disclose further additional tropic hormone deficiencies, and to differentiate permanent from transient impairment of ACTH responsiveness which often seems to be related to emotional deprivation syndrome, more protracted follow up studies are needed.
mesh_terms: D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000097:blood / Q000172:deficiency / Q000378:metabolism*; D001786:Blood Glucose / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007030:Hypothalamo-Hypophyseal System / Q000503:physiopathology; D008297:Male; D008797:Metyrapone; D009422:Nervous System Diseases / Q000378:metabolism*; D010913:Pituitary-Adrenal System / Q000503:physiopathology
chemical_list: D001786:Blood Glucose; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone; D008797:Metyrapone
vernacular_title: Troubles de la réactivité de l'ACTH et manifestations neurologiques paroxystiques de l'enfance. A propos de neuf observations de déficits corticotropes isolés.