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High serum progesterone associated with infertility in a woman with nonclassic congenital adrenal hyperplasia
Author(s) -
Kawarai Yoshimasa,
Ishikawa Hiroshi,
Segawa Tomoya,
Teramoto Shokichi,
Tanaka Tomoaki,
Shozu Makio
Publication year - 2017
Publication title -
journal of obstetrics and gynaecology research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 50
eISSN - 1447-0756
pISSN - 1341-8076
DOI - 10.1111/jog.13288
Subject(s) - medicine , congenital adrenal hyperplasia , endocrinology , hyperandrogenism , infertility , androgen excess , folliculogenesis , follicular phase , polycystic ovary , pregnancy , biology , insulin , insulin resistance , lactation , genetics
Nonclassic congenital adrenal hyperplasia (NCAH) is an autosomal‐recessive disorder caused by 21‐hydroxylase deficiency and manifests as hirsutism and oligomenorrhea due to excess adrenal androgen and progesterone. We report a case of a woman with NCAH who showed continuous high serum progesterone levels in the follicular phase associated with impaired folliculogenesis. NCAH was diagnosed based on high 17‐hydroxyprogesterone levels after rapid adrenocorticotropic hormone loading, and three heterozygous missense mutations in CYP21A2 , encoding 21‐hydroxylase, were identified. Recurrent failure of in vitro fertilization programs occurred because of empty follicles and preterm rupture of leading follicles, and vaginal stenosis with a hypoplastic cervix. Glucocorticoid administration normalized serum progesterone levels, restored folliculogenesis, and stretched the vaginal wall, which contributed to the success of the in vitro fertilization program. The patient delivered at term following blastocyst transfer. Correction of hyperandrogenism and high progesterone levels using glucocorticoids may improve fertility in women with NCAH.