Adrenocorticotropic Hormone and 17-Hydroxyprogesterone Levels during High-dose Glucocorticoid Supplement for the Management of Clitoroplasty of CYP21A2 Deficiency

Endocrine Journal

Yukihiro HASEGAWA²  Yasuhiro NAIKI¹  Kenji NANAO³ 

[1] Division of Endocrinology and Metabolism, National Center of Child Health and Development;Endocrinology, Metabolism and Genetics Unit, Tokyo Metropolitan Kiyose Children's Hospital;Division of Pediatrics, National Kasumigaura Hospital

关键词: Clitoroplasty;    CYP21A2 deficiency;    ACTH;    17OHP;   

DOI  :  10.1507/endocrj.51.367

学科分类：内分泌与代谢学

来源: Japan Endocrine Society

【 摘 要 】

References(12)ACTH and 17-hydroxyprogesterone (17OHP) levels during clitoro- and labinoplasty for CYP21A2 deficiency have not been reported. In this study, we measured these levels during surgery. Intensive glucocorticoid supplement (IGS Tx; intravenous bolus injection of daily dose (14.6 to 22.2 mg/m2) of hydrocortisone followed by continuous infusion of three times the daily dose for 24 hours) was done to eight patients for surgery. ACTH and 17OHP levels were generally suppressed during operation by this protocol, but mid-surgical elevations of ACTH and 17OHP levels were observed in four out of the eight. In another three patients than the eight as described, oral dexamethasone pretreatment (1 mg/m2) was administered for two days before surgery in addition to IGS Tx. ACTH and 17OHP levels were completely suppressed throughout operation, indicating that this kind of additional pretreatment is more effective approach.

【 授权许可】

Unknown