Benjamin Wilson, BA: Neurocrine Biosciences: Consultant ()
Richard J. Auchus, MD,PhD: Adrenas: Contracted Research (Principal Investigators must provide information, even if received by the institution) (); Aspect Biosystems: Consultant (); Crinetics: Consultant (), Contracted Research (Principal Investigators must provide information, even if received by the institution) (); Lundbeck: Consultant (), Contracted Research (Principal Investigators must provide information, even if received by the institution) (); Merck: Consultant (); Neurocrine: Consultant (), Contracted Research (Principal Investigators must provide information, even if received by the institution) (); OMass: Consultant (); Vertex: Consultant ()
Objective: Previous studies have shown that fecundity rates can be normal in women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency (21OHD). Fertility optimization requires adequate suppression of adrenal-derived follicular-phase progesterone, which is the greatest barrier to achieving pregnancy. Information on maternal and fetal outcomes following conception and throughout pregnancy, however, is limited for mothers with 21OHD. Our goal was to analyze treatments and complications for women with classic and nonclassic 21OHD during pregnancy at one center.
Methods: The medical records of 37 women diagnosed with 21OHD (13 classic and 24 nonclassic) who had at least one previous pregnancy were identified and analyzed using the electronic medical record search engine (EMERSE).
Results: Among the 13 classic and 24 nonclassic 21OHD patients, we identified 34 and 73 pregnancies, respectively. Nearly all pregnancies for women with classic 21OHD delivered via caesarean section (93.3%). Nonclassic 21OHD pregnancies also were more likely to be delivered via caesarean section than the general population (52.8% vs 32.4%). The percentage of classic 21OHD women who experienced at least one complication during pregnancy was comparable to the general population (46.2% vs 46.3%) while women with nonclassic 21OHD more commonly experienced complications (66.7%). The most common complications in women with nonclassic 21OHD were miscarriages and pre-term deliveries, which occurred in 37.0% and 20.9% of pregnancies, respectively, compared to 14.6% and 10.4% for the general population. The miscarriage rate for women with nonclassic 21OHD taking glucocorticoids during the duration of pregnancy was 25.0%, compared to 48.8% in untreated women; however, premature birth rates were the same regardless of glucocorticoid usage. Among nonclassic 21OHD pregnancies, one with donor eggs was terminated for skeletal malformations, and 7 children were confirmed to have 21OHD (3 classic, 4 nonclassic).
Conclusions: Maternal and fetal complication rates are similar between well-managed classic 21OHD women and the general population. Women with nonclassic 21OHD are at a higher risk of miscarriage and preterm births, which glucocorticoid therapy appears to only partially mitigate in our cohort.
*Unless otherwise noted, all abstracts presented at ENDO must not be released to the press or the public until the date and time of presentation. For oral presentations, the abstracts are embargoed until the session begins. The Endocrine Society reserves the right to lift the embargo on specific abstracts that are selected for promotion prior to or during ENDO.*
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