Liver transplantation for acute-on-chronic liver failure from erythropoietic protoporphyria

被引：12

作者：

Park, Pyoung-Jae ^{[1
]}

Hwang, Shin ^{[1
]}

Choi, Young-Il ^{[1
]}

Yu, Young-Dong ^{[1
]}

Park, Gil-Chun ^{[1
]}

Jung, Sung-Won ^{[1
]}

Yoon, Sam-Youl ^{[1
]}

Song, Gi-Won ^{[1
]}

Ha, Tae-Yong ^{[1
]}

Lee, Sung-Gyu ^{[1
]}

机构：

[1] Univ Ulsan, Coll Med, Asan Med Ctr, Dept Surg,Div Hepatobiliary Surg & Liver Transpla, Seoul 138736, South Korea

来源：

CLINICAL AND MOLECULAR HEPATOLOGY | 2012年 / 18卷 / 04期

关键词：

Liver transplantation; Acute-on-chronic liver failure; Erythropoietic protoporphyria;

D O I：

10.3350/cmh.2012.18.4.411

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

Erythropoietic protoporphyria (EPP) is an inherited disorder of the heme metabolic pathway that is characterized by accumulation of protoporphyrin in the blood, erythrocytes, and tissues, and cutaneous manifestations of photosensitivity, all resulting from abnormalities in ferrochelatase (FECH) activity due to mutations in the FECH gene. Protoporphyrin is excreted by the liver, and excess protoporphyrin leads to cholelithiasis with obstructive episodes and chronic liver disease, finally progressing to liver cirrhosis. Patients with end-stage EPP-associated liver disease require liver transplantation. We describe here a 31-year-old male patient with EPP who experienced acute-on-chronic liver failure and underwent deceased-donor liver transplantation. Surgical and postoperative care included specific shielding from exposure to ultraviolet radiation to prevent photosensitivity-associated adverse effects. The patient recovered uneventfully and was doing well 24 months after transplantation. Future prevention and treatment of liver disease are discussed in detail.

引用

页码：411 / 415

页数：5

共 20 条

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