Continuous subcutaneous hydrocortisone infusion (CSHI) in a young adolescent with congenital adrenal hyperplasia (CAH)

✍ Scribed by Tuli, Gerdi; Rabbone, Ivana; Einaudi, Silvia; di Gianni, Valeria; Tessaris, Daniele; Gioia, Elisa; Lala, Roberto; Cerutti, Franco

• Book ID: 126988654
• Publisher: Walter de Gruyter GmbH & Co. KG
• Year: 2011
• Tongue: English
• Weight: 161 KB
• Volume: 24
• Category: Article
• ISSN: 0334-018X
• DOI: 10.1515/jpem.2011.226

✦ Synopsis

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 diffi cult 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.