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一名患有心肌病且对遗传性血色素沉着症具有遗传易感性的飞行员:病例报告。

An aviator with cardiomyopathy and genetic susceptibility to hereditary hemochromatosis: a case report.

作者信息

Hanson E H, Shue P M, Palm-Leis A, Rowley R K

机构信息

Air Force Residency in Aerospace Medicine, USAFSAM, Brooks AFB, TX, USA.

出版信息

Aviat Space Environ Med. 2001 Oct;72(10):924-7.

Abstract

A 44-yr-old male pilot was diagnosed with non-ischemic cardiomyopathy, possibly as a complication of hereditary hemochromatosis, 8 yr after an acquired left bundle branch block was discovered on a routine ECG. Biochemical testing returned high levels of iron and percentage transferrin saturation, and genetic testing for hemochromatosis was remarkable for a heterozygous H63D mutation in the HFE gene on chromosome 6. Hereditary hemochromatosis should be considered in the differential diagnosis when a patient presents with cardiomyopathy and genetic testing for HFE gene variants influencing iron overload is now available as a clinical adjunct for diagnosis and patient management issues. Cardiomyopathy and symptomatic hemochromatosis are aeromedically disqualifying conditions in the U.S. Air Force; however, early identification of hereditary hemochromatosis susceptibility with biochemical or genetic diagnostic tests, followed by education in primary and secondary prevention, will prevent a significant proportion of the possible sequelae.

摘要

一名44岁的男性飞行员在常规心电图检查中发现获得性左束支传导阻滞8年后,被诊断为非缺血性心肌病,可能是遗传性血色素沉着症的并发症。生化检测显示铁水平和转铁蛋白饱和度百分比升高,血色素沉着症的基因检测显示6号染色体上的HFE基因存在杂合H63D突变。当患者出现心肌病时,鉴别诊断应考虑遗传性血色素沉着症,目前可通过对影响铁过载的HFE基因变异进行基因检测,作为诊断和患者管理问题的临床辅助手段。在美国空军中,心肌病和症状性血色素沉着症属于航空医学不合格情况;然而,通过生化或基因诊断测试早期识别遗传性血色素沉着症易感性,随后进行一级和二级预防教育,将预防很大一部分可能的后遗症。

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