Erythropoietic Protoporphyria-related Hepatopathy Successfully Treated with Phlebotomy
Erythropoietic Protoporphyria-related Hepatopathy Successfully Treated with Phlebotomy
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DOI：
10.2169/internalmedicine.0673-17
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发表时间：
2018-01-01
影响因子：
1.2
通讯作者：
Kudo, Masatoshi
中科院分区：
文献类型：
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作者：
Yoshida, Akihiro;Hagiwara, Satoru;Kudo, Masatoshi
1例27岁男性，携带红细胞生成素原卟啉(EPP)相关铁络合酶(FECH)突变，因全身不适及血清肝胆酶和胆红素水平显著升高而入院治疗。最初的血浆置换治疗并没有降低血原卟啉或血清肝酶水平，因此开始了静脉切开术。令人惊讶的是，每周一次的静脉采血使血清肝酶水平正常化，同时血液中原卟啉水平显著降低。此病例的临床过程强烈表明，静脉切开术可能是EPP相关性肝病的一种合适的治疗选择。
A 27-year-old man bearing an erythropoietic protoporphyria (EPP)-associated ferrochelatase (FECH) mutation was admitted to our hospital for general malaise and marked elevation of the serum levels of hepatobiliary enzymes and bilirubin. Initial treatment with plasma exchange did not reduce the blood protoporphyrin or serum liver enzyme levels, so phlebotomy was started. Surprisingly, weekly phlebotomy normalized the serum levels of liver enzymes, accompanied by a marked reduction in the blood protoporphyrin levels. The clinical course of this case strongly suggests that phlebotomy may be a suitable treatment option for EPP-related hepatopathy.