Oliveira Diana, Lages Adriana, Paiva Sandra, Carrilho Francisco
Endocrinology, Diabetes and Metabolism Department, Coimbra Hospital and University Center, Coimbra, Portugal.
Endocrinol Diabetes Metab Case Rep. 2018 Apr 12;2018. doi: 10.1530/EDM-17-0179. eCollection 2018.
Addison's disease, or primary adrenocortical insufficiency, is a long-term, potentially severe, rare endocrine disorder. In pregnancy, it is even rarer. We report the case of a 30-year-old pregnant patient with Addison's disease, referred to Obstetrics-Endocrinology specialty consult at 14 weeks gestation. She had been to the emergency department of her local hospital various times during the first trimester presenting with a clinical scenario suggestive of glucocorticoid under-replacement (nausea, persistent vomiting and hypotension), but this was interpreted as normal pregnancy symptoms. Hydrocortisone dose was adjusted, and the patient maintained regular follow-up. No complications were reported for the remainder of gestation and delivery. Pregnant patients with Addison's disease should be monitored during gestation and in the peripartum period by multidisciplinary teams. Adjustments in glucocorticoid and mineralocorticoid replacement therapy are often necessary, and monitoring should be based mainly on clinical findings, which becomes increasingly difficult during pregnancy. Patient education and specialized monitoring are key to avoiding complications from under- or over-replacement therapy in this period.
An increase in glucocorticoid replacement dose is expected to be necessary during pregnancy in a woman with Addison's disease.Patient education regarding steroid cover and symptoms of acute adrenal crisis are fundamental.Monitoring in this period is challenging and remains mainly clinical.The increase in hydrocortisone dose often obviates the need to increase fludrocortisone dose.
艾迪生病，即原发性肾上腺皮质功能不全，是一种长期的、潜在严重的罕见内分泌疾病。在妊娠期间，其更为罕见。我们报告一例30岁患有艾迪生病的孕妇病例，该患者在妊娠14周时转诊至妇产科 - 内分泌专科会诊。在孕早期，她多次前往当地医院急诊科，临床表现提示糖皮质激素替代不足（恶心、持续呕吐和低血压），但这些症状被解释为正常妊娠症状。氢化可的松剂量进行了调整，患者维持定期随访。妊娠和分娩的其余过程未报告并发症。患有艾迪生病的孕妇在妊娠期和围产期应由多学科团队进行监测。糖皮质激素和盐皮质激素替代治疗通常需要调整，监测应主要基于临床症状，而在妊娠期间这变得越来越困难。患者教育和专业监测是避免在此期间替代治疗不足或过量引发并发症的关键。
患有艾迪生病的女性在妊娠期间预计需要增加糖皮质激素替代剂量。关于类固醇覆盖和急性肾上腺危象症状的患者教育至关重要。在此期间的监测具有挑战性，且主要仍基于临床症状。氢化可的松剂量的增加通常无需增加氟氢可的松剂量。
