title: A woman with salt-wasting congenital adrenal hyperplasia presenting with a mucinous ovarian cystadenoma during pregnancy.
abstract: Women with congenital adrenal hyperplasia (CAH) caused by steroid 21-hydroxylase deficiency show reduced fertility, especially with the salt-wasting form. A 27-year-old pregnant woman with this disease underwent laparotomy and oophorectomy to remove a multilocular ovarian tumor at 14 weeks of pregnancy. This proved to be a mucinous cystadenoma. Toward the third trimester, she presented with marked elevations of 17α-hydroxyprogesterone and plasma renin activity. Careful management of endocrine and body fluid homeostasis allowed her to give birth to a healthy female infant with normal external genitalia. This case illustrates endocrinological parameters during pregnancy in a woman with classical salt-wasting CAH.
authors.affiliation: Department of Endocrinology, Diabetes and Metabolism, Kitasato University School of Medicine, Japan. ykamata@med.kitasato-u.ac.jp
authors.forename: Yuji
authors.initials: Y
authors.lastname: Kamata
authors.forename: Akinori
authors.initials: A
authors.lastname: Hayashi
authors.forename: Akifumi
authors.initials: A
authors.lastname: Ogawa
authors.forename: Raishi
authors.initials: R
authors.lastname: Ichikawa
authors.forename: Tatsumi
authors.initials: T
authors.lastname: Moriya
authors.forename: Masayoshi
authors.initials: M
authors.lastname: Shichiri
mesh_terms: D019326:17-alpha-Hydroxyprogesterone / Q000097:blood; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood / Q000453:epidemiology*; D000328:Adult; D015897:Comorbidity; D018291:Cystadenoma, Mucinous / Q000000981:diagnostic imaging* / Q000453:epidemiology* / Q000601:surgery; D005260:Female; D006801:Humans; D010535:Laparoscopy; D010051:Ovarian Neoplasms / Q000000981:diagnostic imaging* / Q000453:epidemiology* / Q000601:surgery; D010052:Ovariectomy; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000000981:diagnostic imaging* / Q000453:epidemiology* / Q000601:surgery; D011256:Pregnancy Outcome; D011261:Pregnancy Trimester, First; D012083:Renin / Q000097:blood; D016896:Treatment Outcome; D014463:Ultrasonography
chemical_list: D019326:17-alpha-Hydroxyprogesterone; D012083:Renin