IRON THERAPY FOR HEPATIC-DYSFUNCTION IN ERYTHROPOIETIC PROTOPORPHYRIA
IRON THERAPY FOR HEPATIC-DYSFUNCTION IN ERYTHROPOIETIC PROTOPORPHYRIA
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DOI：
10.7326/0003-4819-105-1-27
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发表时间：
1986-07-01
影响因子：
39.2
通讯作者：
BICKERS, DR
中科院分区：
文献类型：
--
作者：
GORDEUK, VR;BRITTENHAM, GM;BICKERS, DR
红细胞生成性原卟啉是由于遗传性铁络合酶缺陷引起的，导致红细胞和血浆中游离原卟啉浓度升高。皮肤光敏是主要的临床表现，但在一些患者中，原卟啉在肝脏中的积聚可能会导致致命的肝功能衰竭。目前还没有针对这种致命并发症的治疗方法。我们报告一位患有红细胞生成性原卟啉且肝功能测试结果异常的患者，口服铁剂治疗后导致红细胞和粪便中游离原卟啉水平显著降低，肝功能恢复正常。应考虑铁剂治疗以预防红细胞生成性原卟啉增多症患者的潜在致死性肝功能衰竭。
Erythropoietic protoporphyria, caused by an inherited deficiency of ferrochelatase, results in increased free protoporphyrin concentrations in erythrocytes and plasma. Cutaneous photosensitivity is the primary clinical manifestation, but in some patients, protoporphyrin accumulation in the liver may lead to fatal hepatic failure. No treatment exists for this lethal complication. We report the case of a patient with erythropoietic protoporphyria and abnormal results of liver function tests in whom oral iron therapy led to a substantial decrease in free erythrocyte and stool protoporphyrin levels with return of liver function to normal. Iron therapy should be considered for preventing potentiallly fatal hepatic failure in patients with erythropoietic protoporphyria.