[Perioperative substitution therapy in congenital adrenogenital syndrome with salt loss].

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.