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继发性血色素沉着症中的难治性双心室心力衰竭。

Refractory biventricular heart failure in secondary hemochromatosis.

作者信息

Furth P A, Futterweit W, Gorlin R

出版信息

Am J Med Sci. 1985 Nov;290(5):209-13. doi: 10.1097/00000441-198511000-00006.

Abstract

Cardiac hemochromatosis usually presents with clinical congestive heart failure and an enlarged heart. A woman with secondary hemochromatosis and features of both restrictive and congestive cardiomyopathy with a normal sized heart is described. Despite all interventions she had progressive cardiac impairment and expired eight days following presentation. The clinical and laboratory findings of 49 cases of cardiac hemochromatosis during the last 50 years are reviewed. While the disease usually progresses slowly, a significant minority die within fourteen days of recognition. Not infrequently pericardial tamponade is entertained as a cause for this rapid deterioration; however, both a restrictive and congestive cardiomyopathy appear to be responsible for this catastrophic presentation.

摘要

心脏血色素沉着症通常表现为临床充血性心力衰竭和心脏扩大。本文描述了一名患有继发性血色素沉着症且兼具限制性和充血性心肌病特征但心脏大小正常的女性。尽管采取了所有干预措施,她仍出现进行性心脏损害,并在就诊八天后死亡。本文回顾了过去50年中49例心脏血色素沉着症的临床和实验室检查结果。虽然该病通常进展缓慢,但仍有相当一部分患者在确诊后十四天内死亡。心包填塞常被认为是这种快速恶化的原因;然而,限制性和充血性心肌病似乎都与这种灾难性表现有关。

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