What is the best diagnostic and therapeutic management strategy for an Addison patient during pregnancy?
What is the best diagnostic and therapeutic management strategy for an Addison patient during pregnancy?
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DOI：
10.1111/cen.12097
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发表时间：
2013-04-01
影响因子：
3.2
通讯作者：
Arlt, Wiebke
中科院分区：
文献类型：
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作者：
Lebbe, Marie;Arlt, Wiebke
妊娠期间原发性肾上腺功能不全（PAI）的新诊断非常罕见，难以识别，因为恶心，疲劳和低血压等体征和症状可能类似于正常妊娠的特征。然而，如果诊断被忽视，类固醇替代治疗延迟，随后由妊娠剧吐、发热或分娩引发的肾上腺危象可导致严重的孕产妇和胎儿发病率，甚至死亡。如果临床怀疑患有PAI，我们建议测量皮质醇和促肾上腺皮质激素的配对样本，如果临床可行，还建议进行简短的突触反应测试。我们提出了特定的三个月通过截断短synacthen测试，考虑到总的上升，也游离皮质醇在怀孕期间。如果临床高度怀疑氢化可的松，则不应延迟经验性氢化可的松治疗。所有患有PAI的孕妇都应接受内分泌和产科专家小组的监测。孕晚期不仅在生理上与总皮质醇的升高有关，而且与游离皮质醇的升高有关，因此需要定期调整糖皮质激素的剂量。由于黄体酮的抗盐皮质激素特性，盐皮质激素的需求在怀孕期间可能会发生变化。由于妊娠期血浆肾素生理性升高，监测仅限于临床评估，包括血压和血清电解质。重要的是，患有PAI的孕妇和她的伴侣接受过关于并发疾病中糖皮质激素剂量调整的良好教育，并且都接受过氢化可的松紧急注射技术的培训。应向产科工作人员提供关于分娩和分娩期间氢化可的松覆盖的明确书面指导。通过适当的替代治疗，PAI患者可以期待顺利怀孕并分娩健康婴儿。
A new diagnosis of primary adrenal insufficiency (PAI) during pregnancy is extremely rare and difficult to recognize as signs and symptoms such as nausea, fatigue and hypotension may resemble features of normal pregnancy. However, if the diagnosis is overlooked and steroid replacement delayed, subsequent adrenal crisis triggered by hyperemesis gravidarum, fever or delivery can cause severe maternal and foetal morbidity and even mortality. In case of clinical suspicion of PAI, we recommend to measure paired samples of cortisol and ACTH and, if clinically feasible, a short synacthen test. We propose trimester-specific pass cut-offs for the short synacthen test that take into account the rise of total and also free cortisol during pregnancy. Empirical hydrocortisone treatment should never be delayed if the clinical suspicion is high. All pregnant women with PAI should be monitored by a team of endocrine and obstetric specialists. The third trimester is physiologically associated with a rise not only in total but also free cortisol and thus requires regular adjustment of the glucocorticoid dose. Mineralocorticoid requirements may change during pregnancy due to the anti-mineralocorticoid properties of progesterone. As plasma renin physiologically increases in pregnancy, monitoring is limited to clinical assessment including blood pressure and serum electrolytes. It is crucial that a pregnant woman with PAI and her partner are well educated regarding the adjustment of glucocorticoid dose in intercurrent illness and that both are trained in hydrocortisone emergency injection techniques. The obstetric staff should be provided with clear and written guidance for hydrocortisone cover during labour and delivery. With the appropriate replacement therapy, PAI patients can expect to have an uneventful pregnancy and deliver a healthy infant.