title: Continuous subcutaneous hydrocortisone infusion (CSHI) in a young adolescent with congenital adrenal hyperplasia (CAH).
abstract: Congenital adrenal hyperplasia (CAH) is an autosomal recessive enzymatic defect caused by mutations or deletions of the cytochrome P450 21-hydroxylase CYP21 gene. Oral therapy with glucocorticoids and mineralcorticoids is administered to prevent adrenal crisis and to control hyperandrogenism. During puberty this type of therapy is difficult to manage owing to physiological and hormonal changes and poor compliance. We describe a case of a pubertal boy affected by CAH, in whom continuous subcutaneous infusion of hydrocortisone led to improved metabolic control and compliance.
authors.affiliation: Department of Pediatric Endocrinology and Diabetology, Regina Margherita Children's Hospital, University of Torino, Torino, Italy. gerdi13tuli@yahoo.it
authors.forename: Gerdi
authors.initials: G
authors.lastname: Tuli
authors.forename: Ivana
authors.initials: I
authors.lastname: Rabbone
authors.forename: Silvia
authors.initials: S
authors.lastname: Einaudi
authors.forename: Valeria
authors.initials: V
authors.lastname: di Gianni
authors.forename: Daniele
authors.initials: D
authors.lastname: Tessaris
authors.forename: Elisa
authors.initials: E
authors.lastname: Gioia
authors.forename: Roberto
authors.initials: R
authors.lastname: Lala
authors.forename: Franco
authors.initials: F
authors.lastname: Cerutti
mesh_terms: D000293:Adolescent; D000294:Adolescent Behavior / Q000523:psychology; D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy* / Q000503:physiopathology / Q000523:psychology; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage* / Q000627:therapeutic use; D016503:Drug Delivery Systems; D006029:Glycosuria / Q000209:etiology / Q000517:prevention & control; D006801:Humans; D006854:Hydrocortisone / Q000008:administration & dosage* / Q000627:therapeutic use; D055104:Infusions, Subcutaneous; D008297:Male; D055118:Medication Adherence / Q000523:psychology; D011629:Puberty, Precocious / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D006854:Hydrocortisone