OBJECTIVES: Fertility rates in women with congenital adrenal hyperplasia (CAH) are reported to be poor, but few data are available. We assessed rates and course of pregnancy, mode of delivery and long-term outcome of offspring from women with CAH. DESIGN: A large cohort of women with CAH due to 21-hydroxylase deficiency had initially been diagnosed and followed at one centre. Those women who had given birth were contacted. Information was gathered from hospital records, direct patient contact, structured questionnaire and the Documentation of Pregnancy and Preventive Care Booklets. RESULTS: Between 1978 and 1998, 18 women with CAH (one salt wasting, 12 simple virilizing, five nonclassical) had given birth to 31 children (18 females, 13 males). Delivery was by Caesarean section in 16 out of the 31 children. None of the female newborns was masculinized. Twenty-nine children were born at term, five children were small for gestational age (SGA). Postnatal development was basically normal in all children; 18 are now older than 10 years, seven are between 5 and 10 years old, six are less than 5 years old. CONCLUSIONS: Fertility is reduced in females with CAH, especially those with the severe or salt wasting phenotype. In those women with CAH who do conceive, course and outcome of pregnancy is mostly uneventful, although the rate of SGA offspring may be increased. Psychomotor and somatic long-term development of the children was within normal limits.
OBJECTIVES: Fertility rates in women with congenital adrenal hyperplasia (CAH) are reported to be poor, but few data are available. We assessed rates and course of pregnancy, mode of delivery and long-term outcome of offspring from women with CAH. DESIGN: A large cohort of women with CAH due to 21-hydroxylase deficiency had initially been diagnosed and followed at one centre. Those women who had given birth were contacted. Information was gathered from hospital records, direct patient contact, structured questionnaire and the Documentation of Pregnancy and Preventive Care Booklets. RESULTS: Between 1978 and 1998, 18 women with CAH (one salt wasting, 12 simple virilizing, five nonclassical) had given birth to 31 children (18 females, 13 males). Delivery was by Caesarean section in 16 out of the 31 children. None of the female newborns was masculinized. Twenty-nine children were born at term, five children were small for gestational age (SGA). Postnatal development was basically normal in all children; 18 are now older than 10 years, seven are between 5 and 10 years old, six are less than 5 years old. CONCLUSIONS: Fertility is reduced in females with CAH, especially those with the severe or salt wasting phenotype. In those women with CAH who do conceive, course and outcome of pregnancy is mostly uneventful, although the rate of SGA offspring may be increased. Psychomotor and somatic long-term development of the children was within normal limits.
Authors: Christina Bothou; Gurpreet Anand; Dingfeng Li; Tina Kienitz; Khyatisha Seejore; Chiara Simeoli; Andreas Ebbehoj; Emma G Ward; Rosa Maria Paragliola; Rosario Ferrigno; Klaus Badenhoop; Sophie Bensing; Marianne Oksnes; Daniela Esposito; Ragnhildur Bergthorsdottir; William Drake; Jeanette Wahlberg; Nicole Reisch; Stefanie Hahner; Simon Pearce; Peter Trainer; Gwendolin Etzrodt-Walter; Sébastien P Thalmann; Åse B Sævik; Eystein Husebye; Andrea M Isidori; Henrik Falhammar; Gesine Meyer; Salvatore M Corsello; Rosario Pivonello; Robert Murray; Irina Bancos; Marcus Quinkler; Felix Beuschlein Journal: J Clin Endocrinol Metab Date: 2020-08-01 Impact factor: 5.958