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通过用聚酰亚胺薄膜覆盖手术室灯光成功治疗红细胞生成性原卟啉症所致肝衰竭的肝移植病例报告

Successful Liver Transplantation for Liver Failure With Erythropoietic Protoporphyria by Covering the Operating Theater Lights With Polyimide Film: A Case Report.

作者信息

Hanaki Takehiko, Noda Takehiro, Eguchi Hidetoshi, Iwagami Yoshifumi, Akita Hirofumi, Asaoka Tadafumi, Gotoh Kunihito, Kobayashi Shogo, Umeshita Koji, Mori Masaki, Doki Yuichiro

机构信息

Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University, Osaka, Japan; Division of Surgical Oncology, Department of Surgery, School of Medicine, Tottori University Faculty of Medicine, Yonago, Japan.

Department of Gastroenterological Surgery, Graduate School of Medicine, Osaka University, Osaka, Japan.

出版信息

Transplant Proc. 2020 Mar;52(2):625-629. doi: 10.1016/j.transproceed.2019.12.004. Epub 2020 Feb 4.

Abstract

BACKGROUND

Erythropoietic protoporphyria is a rare disease of heme biosynthesis resulting in excessive accumulation of protoporphyrin in various organs. The most typical symptom is photosensitivity caused by activated protoporphyrins (wavelength ~400 nm). Accumulated protoporphyrin in the liver also causes liver failure, and liver transplantation is the only life-saving treatment. Phototoxic injury to abdominal organs has been reported during liver transplantation. Thus, to avoid phototoxic injury during liver transplantation, it has previously been conducted with only shadowless lights and ceiling lights off in the operating theater. Here, we report a case of a safe and successful liver transplantation in a patient with erythropoietic protoporphyria where the operating theater lights were covered with polyimide film.

CASE PRESENTATION

A 50-year-old man presented with hepatic failure owing to erythropoietic protoporphyria. Before liver transplantation, the shadowless lights and ceiling lights in the operating theater were covered entirely with polyimide film. This filter completely blocked the harmful wavelength of light (400-470 nm). Orthotopic liver transplantation was safely and successfully performed with adequate illumination and patient monitoring. The patient followed a normal postoperative course without phototoxic injuries or protoporphyrin re-accumulation.

CONCLUSION

Covering not only shadowless lights but also all ceiling lights in the operating theater with the polyimide film allowed safe surgery, safe anesthesia, and safe monitoring of the patient who underwent liver transplantation for severe liver failure owing to erythropoietic protoporphyria.

摘要

背景

红细胞生成性原卟啉病是一种血红素生物合成的罕见疾病,导致原卟啉在各个器官中过度积累。最典型的症状是由活化的原卟啉(波长约400nm)引起的光敏感性。肝脏中积累的原卟啉也会导致肝衰竭,肝移植是唯一的挽救生命的治疗方法。肝移植期间曾有腹部器官光毒性损伤的报道。因此,为避免肝移植期间的光毒性损伤,此前在手术室仅使用无影灯且关闭顶灯进行手术。在此,我们报告一例红细胞生成性原卟啉病患者安全成功进行肝移植的病例,该病例中手术室的灯用聚酰亚胺薄膜覆盖。

病例介绍

一名50岁男性因红细胞生成性原卟啉病出现肝衰竭。肝移植前,手术室的无影灯和顶灯全部用聚酰亚胺薄膜覆盖。该滤光片完全阻挡了有害波长的光(400 - 470nm)。在充足照明和患者监测下安全成功地进行了原位肝移植。患者术后恢复正常,未出现光毒性损伤或原卟啉重新积累。

结论

用聚酰亚胺薄膜覆盖手术室的不仅无影灯而且所有顶灯,使得因红细胞生成性原卟啉病导致严重肝衰竭而接受肝移植的患者能够进行安全的手术、安全的麻醉和安全的监测。

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