[Congenital adrenal hypoplasia of cytomegalic type. Recessive, sex-linked form. Apropos of 3 cases].
Three personal cases, and the cases of literature of congenital adrenal hypoplasia with cytomegaly allow an histological definition. Clinical and biological findings are described. In 2 cases, a diffused hypertrophy of the cells responsible for corticotrope or melanotrope secretion was discovered; it confirmed the peripheral adrenal origin of the condition, as well as high plasmatic level of ACTH, in the third case. Sex-bound cytomegalic adrenal hypoplasia is differenciated from other cortico-adrenal insufficiencies with cytomegaly.
D000302:Adrenal Cortex / Q000473:pathology; D000309:Adrenal Insufficiency / Q000151:congenital* / Q000235:genetics / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005808:Genes, Recessive; D006801:Humans; D007223:Infant; D008297:Male
Hypoplasie surrenalienne congenitale de type cytomegalique. Forme recessive liee au sexe. A propos de trois observations