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一种铁螯合剂改善了遗传性血色素沉着症所致严重心力衰竭患者的心脏功能。

An Iron-chelating Agent Improved the Cardiac Function in a Patient with Severe Heart Failure Due to Hereditary Hemochromatosis.

机构信息

Department of Cardiovascular Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan.

Department of Pathology, Nagasaki University Hospital, Japan.

出版信息

Intern Med. 2024 Jan 15;63(2):253-258. doi: 10.2169/internalmedicine.1809-23. Epub 2023 May 17.

DOI:10.2169/internalmedicine.1809-23
PMID:37197964
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10864071/
Abstract

A 24-year-old man was admitted to our hospital because of severe heart failure. Although he was treated with diuretics and positive inotropic agents, his heart failure progressed. An endomyocardial biopsy revealed iron deposition in his myocytes. Finally, he was diagnosed with hereditary hemochromatosis. After starting administration of an iron-chelating agent in addition to conventional treatment for heart failure, his condition improved. We should consider hemochromatosis in heart failure patients with severe right ventricular dysfunction in addition to left ventricular dysfunction.

摘要

一位 24 岁男性因严重心力衰竭入院。尽管他接受了利尿剂和正性肌力药物治疗，但心力衰竭仍在进展。心肌内膜活检显示他的心肌细胞中铁沉积。最终，他被诊断为遗传性血色素沉着症。除了心力衰竭的常规治疗外，开始给予铁螯合剂治疗后，他的病情有所改善。我们应该考虑心力衰竭患者除左心室功能障碍外，还存在严重右心室功能障碍的情况下，是否患有血色病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/f0c07e0b68ae/1349-7235-63-0253-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/c4a3cc97937f/1349-7235-63-0253-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/5e1cd0b16ad0/1349-7235-63-0253-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/73eb778a7743/1349-7235-63-0253-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/cce905e4d05b/1349-7235-63-0253-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/0cd7dbc0094e/1349-7235-63-0253-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/f0c07e0b68ae/1349-7235-63-0253-g006.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/c4a3cc97937f/1349-7235-63-0253-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/5e1cd0b16ad0/1349-7235-63-0253-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/73eb778a7743/1349-7235-63-0253-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/cce905e4d05b/1349-7235-63-0253-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/0cd7dbc0094e/1349-7235-63-0253-g005.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5a8c/10864071/f0c07e0b68ae/1349-7235-63-0253-g006.jpg

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Reversal of end-stage heart failure in juvenile hemochromatosis with iron chelation therapy: a case report.铁螯合疗法逆转青少年血色素沉着症终末期心力衰竭：一例报告
J Med Case Rep. 2018 Jan 26;12(1):18. doi: 10.1186/s13256-017-1526-6.
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Severe Myocardial Dysfunction Reversed by Iron-Chelation Therapy in an Asian Patient with Hereditary Hemochromatosis.铁螯合疗法逆转亚洲遗传性血色素沉着症患者的严重心肌功能障碍
Am J Med Sci. 2016 May;351(5):546-8. doi: 10.1016/j.amjms.2016.02.038. Epub 2016 Mar 2.
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Endocrine dysfunction in hereditary hemochromatosis.遗传性血色素沉着症中的内分泌功能障碍。
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