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自身免疫性肾上腺功能不全患儿发生循环衰竭并需要机械循环支持:病例报告。

Circulatory collapse requiring mechanical circulatory support in a child with autoimmune adrenal insufficiency: a case report.

机构信息

Cardiac Critical Care, The Heart Institute, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, MLC 2003, Cincinnati, OH, 45229, USA.

Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA.

出版信息

BMC Pediatr. 2024 Oct 14;24(1):656. doi: 10.1186/s12887-024-05130-1.

Abstract

BACKGROUND

We report a rare case of new onset autoimmune adrenal insufficiency in childhood, presenting with severe shock requiring mechanical circulatory support. In the current era of readily available imaging, laboratory and other diagnostic investigations, medical history and careful physical exam can often provide valuable diagnostic information for timely therapy.

CASE PRESENTATION

A 7-year-old boy with a history of mild intermittent asthma, presented with severe cardiogenic shock requiring extracorporeal membrane oxygenation (ECMO). Bronzing of his entire body was noted on physical exam. Stress dose hydrocortisone was given for suspected adrenal insufficiency. After weaning from ECMO and extensive rehabilitation, the patient recovered and was discharged home.

CONCLUSION

Primary adrenal insufficiency (PAI) should be considered in the context of physical exam and laboratory findings, even in the presence of circulatory shock.

摘要

背景

我们报告了一例罕见的儿童新发自身免疫性肾上腺功能不全病例,表现为严重休克,需要机械循环支持。在当前影像学、实验室和其他诊断检查易于获得的时代,详细的病史和仔细的体格检查通常可以为及时治疗提供有价值的诊断信息。

病例介绍

一名 7 岁男孩,有轻度间歇性哮喘病史,因心源性休克需体外膜氧合(ECMO)治疗。体格检查发现全身青铜色。考虑到肾上腺功能不全,给予应激剂量的氢化可的松。在从 ECMO 脱机并进行广泛康复后,患者恢复并出院回家。

结论

即使存在循环休克,也应根据体格检查和实验室发现考虑原发性肾上腺功能不全(PAI)。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/92f9/11479536/9c1ca50a9c96/12887_2024_5130_Fig1_HTML.jpg

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