Feldt-Rasmussen Ulla
Department of Medical Endocrinology and Metabolism, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark.
Institute of Clinical Medicine, Faculty of Health and Clinical Sciences, Copenhagen University, Copenhagen, Denmark.
Eur J Endocrinol. 2024 Jan 18. doi: 10.1093/ejendo/lvae005.
Appropriate management of adrenal insufficiency in pregnancy is challenging due to the rarity of both primary, secondary and tertiary forms of the disease as well as the lack of evidence-based recommendations to guide clinicians to glucocorticoid and sometimes also mineralocorticoid dosage adjustments. Debut of adrenal insufficiency during pregnancy requires immediate diagnosis as it can lead to adrenal crisis, intrauterine growth restriction and foetal demise. Diagnosis is difficult due to overlap of symptoms of adrenal insufficiency and its crisis with those of pregnancy. Adrenal insufficiency in stable replacement treatment needs careful monitoring during pregnancy to adapt to the physiological changes in the requirement of the adrenal hormones. This is hampered because the diagnostic threshold of most adrenocortical hormones is not applicable during pregnancy. The frequent use of assisted reproduction technology with controlled ovarian hyperstimulation in these patient groups with disease induced low fertility has created an unrecognised risk of adrenal crises due to accelerated oestrogen stimulation with increased risk of even be life-threatening complications for both the woman and foetus. The area needs consensus recommendations between gynaecologists and endocrinologists in tertiary referral centres to alleviate such increased gestational risk. Patient and partner education, use of the EU emergency card for management of adrenal crises can also contribute to better pregnancy outcomes. There is a strong need of more research on e.g. improvement of glucocorticoid replacement as well as crisis management treatment, and biomarkers for treatment optimisation in this field, which suffers from the rare nature of the diseases and poor funding.
孕期肾上腺功能不全的恰当管理颇具挑战性，这是因为原发性、继发性和三发性肾上腺功能不全均较为罕见，且缺乏循证指南来指导临床医生进行糖皮质激素以及有时还包括盐皮质激素的剂量调整。孕期首次出现肾上腺功能不全会导致肾上腺危象、胎儿宫内生长受限和胎儿死亡，因此需要立即诊断。由于肾上腺功能不全及其危象的症状与孕期症状重叠，诊断较为困难。处于稳定替代治疗的肾上腺功能不全患者在孕期需要仔细监测，以适应肾上腺激素需求的生理变化。但这受到阻碍，因为大多数肾上腺皮质激素的诊断阈值在孕期并不适用。在这些因疾病导致生育力低下的患者群体中，频繁使用辅助生殖技术并进行控制性卵巢过度刺激，由于雌激素刺激加速，会带来未被认识到的肾上腺危象风险，甚至会增加对孕妇和胎儿都有生命威胁的并发症风险。该领域需要三级转诊中心的妇科医生和内分泌科医生达成共识性建议，以减轻这种增加的妊娠风险。对患者及其伴侣进行教育，使用欧盟肾上腺危象管理急救卡，也有助于获得更好的妊娠结局。迫切需要开展更多研究，例如改进糖皮质激素替代治疗以及危象管理治疗，以及该领域用于优化治疗的生物标志物，但由于这些疾病的罕见性和资金匮乏，此类研究进展缓慢。
