一名患有先天性肾上腺皮质增生症（CAH）的青少年持续皮下输注氢化可的松（CSHI）。
Continuous subcutaneous hydrocortisone infusion (CSHI) in a young adolescent with congenital adrenal hyperplasia (CAH).
作者信息
Tuli Gerdi, Rabbone Ivana, Einaudi Silvia, di Gianni Valeria, Tessaris Daniele, Gioia Elisa, Lala Roberto, Cerutti Franco
机构信息
Department of Pediatric Endocrinology and Diabetology, Regina Margherita Children's Hospital, University of Torino, Torino, Italy.
出版信息
J Pediatr Endocrinol Metab. 2011;24(7-8):561-3. doi: 10.1515/jpem.2011.226.
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.
先天性肾上腺皮质增生症（CAH）是一种常染色体隐性酶缺陷疾病，由细胞色素P450 21-羟化酶CYP21基因的突变或缺失引起。给予糖皮质激素和盐皮质激素进行口服治疗，以预防肾上腺危象并控制高雄激素血症。在青春期，由于生理和激素变化以及依从性差，这种治疗方式难以管理。我们描述了一例患有CAH的青春期男孩病例，对其进行氢化可的松皮下持续输注后，代谢控制和依从性得到了改善。
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