A Woman with Salt-wasting Congenital Adrenal Hyperplasia Presenting with a Mucinous Ovarian Cystadenoma during Pregnancy

作者：Kamata Yuji^*; Hayashi Akinori; Ogawa Akifumi; Ichikawa Raishi; Moriya Tatsumi; Shichiri Masayoshi

来源：Internal Medicine, 2011, 50(18): 1981-1985.

DOI：10.2169/internalmedicine.50.5401

• congenital adrenal hyperplasia
• steroid 21-hydroxylase deficiency
• plasma renin activity
• 17 alpha-hydroxyprogesterone
• pregnancy

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 17 alpha-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.

• 出版日期2011