Lüsebrink Enzo, Krieg Kathrin, Massberg Steffen, Orban Martin
Intensive Care Unit, Medizinische Klinik und Poliklinik I, Klinikum der Universität München, Marchioninistraße 15, 81377 Munich, Germany.
DZHK (German Center for Cardiovascular Research), partner site Munich Heart Alliance, Medizinische Klinik und Poliklinik I, Klinikum der Universität München, Marchioninistraße 15, 81377 Munich, Germany.
Eur Heart J Case Rep. 2021 Feb 20;5(2):ytab031. doi: 10.1093/ehjcr/ytab031. eCollection 2021 Feb.
Adrenal crisis is an acute life-threatening exacerbation of the Addison's disease or primary adrenal insufficiency (PAI) and is associated with a high mortality rate. It can be the first manifestation of adrenal insufficiency and is caused by a critical lack of glucocorticoids.
Here, we report the case of a 19-year-old woman presenting to the emergency room with unspecific symptoms, i.e. lethargy, fatigue, arthralgia, dyspnoea, and hypotension. The patient's examination showed major pericardial effusion resulting in cardiac tamponade requiring immediate pericardiocentesis. In the further course, acute right heart failure and progressive cardiogenic shock occurred. Due to recurrent bradycardia and finally asystole, the patient had to be resuscitated for 15 min in total until return of spontaneous circulation. However, non-invasive haemodynamic stabilization of the patient was not successful. Hence, venoarterial extracorporeal membrane oxygenation (VA-ECMO) was used as salvage intervention to provide temporary circulatory support. We diagnosed an Addison crisis as first manifestation of her previously unknown Addison's disease. An appropriate substitution therapy with hydrocortisone and fludrocortisone was immediately initiated and the patient's condition rapidly improved. After a total in-hospital stay of 4 weeks, she recovered completely and could be discharged from hospital.
An Addison crisis requires rapid diagnosis and immediate treatment to end a life-threatening condition caused by critical glucocorticoid deficiency. In patients with non-specific symptoms, such as fatigue, hypotension, weight loss, and hyponatraemia, adrenocortical insufficiency should be considered as differential diagnosis. If patients suffer from an Addison crisis, clinical suspicion requires immediate substitution of hydrocortisone as this is essential for patient's survival. Venoarterial extracorporeal membrane oxygenation therapy can serve as a bridge to diagnosis and effective treatment in patients requiring temporary cardiopulmonary support, especially as salvage intervention for patients in cardiogenic shock. To our knowledge, this is the first case of a young patient with acute Addison crisis and cardiogenic shock, who was successfully salvaged by VA-ECMO support.
肾上腺危象是艾迪生病或原发性肾上腺皮质功能减退症（PAI）的一种急性危及生命的加重情况，死亡率很高。它可能是肾上腺皮质功能减退的首发表现，由糖皮质激素严重缺乏引起。
在此，我们报告一例19岁女性因非特异性症状，即嗜睡、疲劳、关节痛、呼吸困难和低血压而就诊于急诊室的病例。患者检查显示大量心包积液导致心脏压塞，需要立即进行心包穿刺术。在后续病程中，出现急性右心衰竭和进行性心源性休克。由于反复出现心动过缓并最终发展为心搏停止，患者总共接受了15分钟的复苏，直至恢复自主循环。然而，患者的非侵入性血流动力学稳定未成功。因此，采用静脉-动脉体外膜肺氧合（VA-ECMO）作为挽救性干预措施以提供临时循环支持。我们诊断该患者的艾迪生病危象为其先前未知的艾迪生病的首发表现。立即开始使用氢化可的松和氟氢可的松进行适当的替代治疗，患者的病情迅速改善。住院4周后，她完全康复并出院。
肾上腺危象需要快速诊断和立即治疗，以终止由严重糖皮质激素缺乏引起的危及生命的状况。对于出现疲劳、低血压、体重减轻和低钠血症等非特异性症状的患者，应考虑肾上腺皮质功能减退作为鉴别诊断。如果患者患有肾上腺危象，临床怀疑时应立即给予氢化可的松替代治疗，因为这对患者的生存至关重要。静脉-动脉体外膜肺氧合治疗可作为需要临时心肺支持患者诊断和有效治疗的桥梁，尤其是作为心源性休克患者的挽救性干预措施。据我们所知，这是首例通过VA-ECMO支持成功挽救的急性艾迪生病危象合并心源性休克的年轻患者。
