迟发性丙酸血症患者的心肝联合移植手术

IF 0.6 Q4 GASTROENTEROLOGY & HEPATOLOGY

ACG Case Reports Journal Pub Date : 2024-10-10 eCollection Date: 2024-10-01 DOI:10.14309/crj.0000000000001526

Mattie K White, Omar T Sims, Jason Eng, Jamak Modaresi Esfeh

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引用次数: 0

摘要

患有丙酸血症的阿米什病人可能会延迟发病，尽管代谢得到控制，但仍会迅速出现严重的心脏衰竭。医学治疗仅限于严格的低蛋白饮食和纠正潜在的分解代谢诱因。肝移植适用于反复出现代谢失代偿的小儿患者。关于小儿肝脏和心脏联合移植的报道很有限。我们介绍了首例肝脏和心脏联合移植的病例，患者是一名阿米什成人，新诊断为丙酸血症和严重的非缺血性扩张型心肌病，需要高级心脏生命支持。需要特别注意密切监测代谢状态和预防分解代谢状态。

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Combined Heart and Liver Transplantation in Delayed-Onset Propionic Acidemia.

Amish patients with propionic acidemia can have delayed presentations with rapid onset, severe cardiac decompensation despite metabolic control. Medical management is limited to strict low protein diet and correction of underlying catabolic triggers. Liver transplantation is indicated in pediatric patients with recurrent metabolic decompensation. There are limited reports of pediatric combined liver and heart transplantation. We present the first case of combined liver and heart transplantation in an Amish adult with newly diagnosed propionic acidemia and severe nonischemic dilated cardiomyopathy on advanced cardiac life support. Special considerations were necessary for close metabolic status monitoring and prevention of catabolic states.

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来源期刊

ACG Case Reports Journal GASTROENTEROLOGY & HEPATOLOGY-

自引率

14.30%

发文量

170

审稿时长

12 weeks

期刊介绍： ACG Case Reports Journal is a peer-reviewed, open-access publication that provides GI and hepatology fellows, private practice clinicians, and other healthcare providers an opportunity to share interesting case reports with their peers and with leaders in the field. ACG Case Reports Journal publishes case reports, images, videos and letters to the editor in all topics of gastroenterology and hepatology, including: Biliary Colon Endoscopy Esophagus Functional Bowel Disorders Inflammatory Bowel Disease Liver Nutrition and Obesity Pancreas Pathology Pediatric Small Bowel Stomach.