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红细胞生成性原卟啉病患者的腹腔镜胆囊切除术

Laparoscopic cholecystectomy in a patient with erythropoietic protoporphyria.

作者信息

Roe Thomas, Bailey Ian S

机构信息

Southampton General Hospital, Southampton, UK.

出版信息

J Surg Case Rep. 2010 Nov 1;2010(9):3. doi: 10.1093/jscr/2010.9.3.

Abstract

Erythropoietic protoporphyria (EPP) is an inherited defect in haem synthesis causing dangerous phototoxic reactions following exposure to wavelengths of light around 400nm. It can cause catastrophic post-operative complications following open surgery, in which environment various safety measures are now routinely employed. The dangers at laparoscopy have never been discussed in the literature, and nor have any specific precautions been recommended. We describe a 35 year old woman with gallstones undergoing prophylactic laparoscopic cholecystectomy to prevent future cholestasis precipitating porphyric liver failure. A pre-operative trial of the cutaneous effects of the laparoscopic light source was performed to assess the potential risk of use within the peritoneal cavity. The procedure was uneventful and the patient suffered no adverse reaction. We suggest that a trial of the effects of the laparoscopic light source on the skin of EPP patients provides valid reassurance regarding the safety of the laparoscopy for short surgical procedures.

摘要

红细胞生成性原卟啉病(EPP)是一种血红蛋白合成的遗传性缺陷,暴露于400nm左右的波长光线下会引发危险的光毒性反应。开放手术会导致灾难性的术后并发症,目前在这种情况下通常会采取各种安全措施。腹腔镜手术的风险在文献中从未被讨论过,也没有推荐任何具体的预防措施。我们描述了一名35岁患有胆结石的女性,她接受了预防性腹腔镜胆囊切除术以防止未来胆汁淤积引发卟啉性肝衰竭。对腹腔镜光源的皮肤效应进行了术前试验,以评估在腹腔内使用的潜在风险。手术过程顺利,患者未出现不良反应。我们建议,对腹腔镜光源对EPP患者皮肤的影响进行试验,可以为短时间手术的腹腔镜手术安全性提供有效的保证。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a0a2/3649167/3781fdf928ff/jscr-2010-9-3fig1.jpg

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