Pregnancy outcomes of eight pregnant women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency

Abstract

To investigate the clinical features and pregnant outcomes of the pregnant women with congenital adrenal hyperplasia (CAH) 21-hydroxylase deficiency (21-OHD). The clinical features, therapies, pregnant outcomes of the pregnant women with 21-OHD were retrospectively reviewed in Peking Union Medical College Hospital, from January 2005 to April 2011. There were 8 pregnant women with 21-OHD including 5 simple virilizing patients and 3 nonclassical 21-OHD women. Eight patients were accepted progestational and prenatal continual lower glucocorticoid treatment. During the gestational period, the dosage of glucocorticoid was adjusted in one pregnancy. The serum level of 17-alpha hydroxyprogesterone (17-OHP) were elevated after pregnancy [(70 ± 38) versus (24 ± 23) nmol/L, P < 0.05]. The fertility and offspring rate of 8 patients was 8/12, the fertility and offspring rate of patients who started treatment at preadolescence was significantly increased (4/5 versus 4/7). Four patients were accepted genital reconstructive surgery (clitorectomy, clitoroplasty, vulvoplasty) before pregnancy. The incidence of GDM was 1/8. All patients selected caesarean at from 37(+6) gestation weeks to 39(+6) gestation weeks. The average newborn birth weight was (3210 ± 447) g, and height was (48 ± 2) cm of 8 neonates, none of them was CAH. Medical and surgical therapy provides satisfactory fertility and pregnancy outcomes for women with 21-OHD. It is safe to pregnant women with 21-OHD and their fetus in continual lower glucocorticoid treatment. The dosage of glucocorticoid should be carefully adjusted during the pregnancy individually according to serum level of 17-OHP.