Cauldwell Matthew, Steer Philip J, Ahsan Masato, Ali Amanda, Ashiq Shabana, Ashworth Rebecca, Basha Deena, Chong Hsu, Corbett Gillian A, Dunne Fidelma, Hill Amanda, Gajewska-Knapik Katarzyna, Jakes Adam, McLaren David, Kinsella Therese, Lee Tara, Levy Miles, MacKiliop Lucy, McAuliffe Fionnuala M, Mohan Aarthi, Mumby Clare, Nana Melanie, Napier Catherine, Neuberger Francesca, Newman Christine, Oosterhouse Tabitha, Shard Amelia, Shehata Hassan, Stocker Linden, Tomlinson Jeremy W, Beck Adele, Vaidya Bijay, Wiles Kate, Williamson Catherine, Zollner Julia, Ward Emma, Turner Helen E
Department of Obstetrics, Maternal Medicine Service, St George's Hospital, London, UK.
Academic Department of Obstetrics and Gynaecology, Chelsea and Westminster Hospital, London, UK.
BJOG. 2025 Jul;132(8):1122-1129. doi: 10.1111/1471-0528.18143. Epub 2025 Mar 30.
To determine characteristics and pregnancy outcomes in women with primary adrenal insufficiency (PAI).
Retrospective multicentre cohort study.
Twenty-three maternity units in the UK and Ireland.
Seventy-nine women with PAI who had 101 pregnancies.
Retrospective chart analysis.
Adrenal crisis, pregnancy outcomes.
We obtained data on 101 pregnancies in 79 women with PAI. Most (51, 64.1%) had autoimmune disease, 8 (10.3%) had prior adrenal infarction/surgery/haemorrhage, 2 (2.6%) had congenital adrenal hyperplasia, and 18 (21.3%) were unclassified. 19 (24%) women experienced a crisis during pregnancy (18.8% of pregnancies). One woman died postpartum. Although all women had recorded endocrinology input during pregnancy, steroid emergency cards were only reportedly carried in 40 (39.6%) pregnancies and 9/19 (47.4%) of those with an adrenal crisis in pregnancy. Compared with the pre-pregnancy dose, only 41% of women received an increased hydrocortisone dose in pregnancy. The caesarean section rate was higher than the UK average: 62/97 (63.9%). The preterm birth rate was 21.2% (21/99) and 12.8% (12/94) of neonates had a birthweight < 10th centile.
Whilst the obstetric outcome of pregnancy with PAI is generally favourable, there are high rates of caesarean birth and prematurity. A high number of women experienced adrenal crisis and further exploration is warranted. Recommendations regarding third trimester increases in hydrocortisone need consideration and potentially strengthening, in light of further evidence. Pregnant women with adrenal insufficiency should carry an NHS steroid warning card; this should be reinforced both by endocrine and obstetric teams because of the increased risk of life-threatening adrenal crisis.
确定原发性肾上腺皮质功能减退症（PAI）女性患者的特征及妊娠结局。
回顾性多中心队列研究。
英国和爱尔兰的23个产科单位。
79例患有PAI的女性，共妊娠101次。
回顾性病历分析。
肾上腺危象、妊娠结局。
我们获取了79例患有PAI的女性101次妊娠的数据。大多数（51例，64.1%）患有自身免疫性疾病，8例（10.3%）曾有肾上腺梗死/手术/出血，2例（2.6%）患有先天性肾上腺皮质增生，18例（21.3%）未分类。19例（24%）女性在孕期发生了危象（占妊娠的18.8%）。1例女性产后死亡。尽管所有女性在孕期都有内分泌科的诊疗记录，但据报道仅40次（39.6%）妊娠以及9/19例（47.4%）孕期发生肾上腺危象的女性携带了类固醇急救卡。与孕前剂量相比，仅41%的女性在孕期增加了氢化可的松剂量。剖宫产率高于英国平均水平：62/97（63.9%）。早产率为21.2%（21/99），12.8%（12/94）的新生儿出生体重低于第10百分位数。
虽然PAI患者妊娠的产科结局总体良好，但剖宫产和早产率较高。大量女性经历了肾上腺危象，有必要进一步探究。鉴于更多证据，关于孕晚期增加氢化可的松剂量的建议需要考虑并可能加以强化。肾上腺功能不全的孕妇应携带英国国家医疗服务体系（NHS）类固醇警示卡；鉴于危及生命的肾上腺危象风险增加，内分泌科和产科团队都应加强这方面的工作。
