title: [Perioperative substitution therapy in congenital adrenogenital syndrome with salt loss].
abstract: Patients with an inborn error of adrenal steroid synthesis suffer from primary adrenocortical insufficiency requiring corticosteroid replacement therapy and appropriate stress coverage. The case report of a two year old infant with congenital adrenal hyperplasia of the salt-losing type demonstrates the consequences of an accidental steroid underdosage. The necessity of increased cortisol replacement therapy in case of anesthetic procedures--even of short duration and without surgical measures--is underlined. The details of adequate perioperative steroid and i.v. fluid therapy in pediatric patients with congenital adrenal hyperplasia of the salt-losing type are discussed.
mesh_terms: D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy*; D000758:Anesthesia; D002675:Child, Preschool; D004573:Electrolytes / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism; D007432:Intraoperative Period; D008901:Mineralocorticoids / Q000627:therapeutic use
chemical_list: D004573:Electrolytes; D005938:Glucocorticoids; D008901:Mineralocorticoids; D006854:Hydrocortisone
vernacular_title: Perioperative Substitutionstherapie bei kindlichem adrenogenitalen Syndrom mit Salzverlust.