溶酶体外糖原贮积病的心脏延迟强化分布

Cardiac delayed enhancement distribution in extralysosomial glycogen storage disease.

作者信息

Fiocchi Federica, Ricci Caterina, Ligabue Guido, Reggianini Letizia, Modena Maria G, Cenacchi Giovanna, Torricelli Pietro

机构信息

Dipartimento Integrato dei Servizi Diagnostici e per Immagine, Università degli Studi di Modena e Reggio Emilia-Azienda Policlinico, Modena, Italia.

出版信息

Clin Imaging. 2008 Nov-Dec;32(6):474-6. doi: 10.1016/j.clinimag.2008.04.011.

DOI:10.1016/j.clinimag.2008.04.011

PMID:19006777

Abstract

We describe magnetic resonance (MR) aspect of cardiac glycogenesis in a 49-years old man, presented a progressively declining cardiac function and negative coronary angiography. Delayed enhancement MR confirmed non-ischemic pattern with unusual diffuse distribution of Gadolinium. Cardiac biopsy revealed a Glycogen Storage Disease, extralysosomial type. Cardiac MR with analysis of delayed enhancement distribution is an emerging tool that can discriminate between ischemic and non-ischemic diseases; however to identify the precise aetiology of a non-ischemic distribution, myocardial biopsy is still needed.

摘要

我们描述了一名49岁男性心脏糖原生成的磁共振（MR）表现，该患者出现心脏功能逐渐下降且冠状动脉造影阴性。延迟强化磁共振成像证实为非缺血模式，钆剂分布异常弥散。心脏活检显示为糖原贮积病，非溶酶体型。通过分析延迟强化分布的心脏磁共振成像是一种新兴工具，可区分缺血性和非缺血性疾病；然而，要确定非缺血性分布的确切病因，仍需要进行心肌活检。