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迟发性皮肤卟啉病所致肝脏多结节性局灶性脂肪变性。超声、CT及MRI表现

Hepatic multi-nodular focal fatty metamorphosis in acquired porphyria cutanea tarda. Sonographic, CT, and MRI features.

作者信息

Chevallier P, Bahadoran P, Buckley M J, Hebuterne X, Diaine B, Chevallier A, Coussement A

机构信息

Department of Radiology, Centre Hospitalier Universitaire de Nice, Hôpital de l'Archet II, France.

出版信息

Clin Imaging. 1998 Nov-Dec;22(6):418-21. doi: 10.1016/s0899-7071(98)00069-2.

Abstract

The authors report the sonographic, computed tomography (CT), and magnetic resonance imaging (MRI) findings in a patient with acquired porphyria cutanea tarda and hepatic multi-nodular focal fatty metamorphosis. There has only been one previous description of an association between these two conditions. The diagnosis was confirmed by histological analysis of a liver biopsy specimen. Symptomatology related to the hepatic abnormality may be due to an inflammatory reaction induced by the presence of uroporphyrin crystals in the liver. Each of the radiological techniques demonstrated unusual hepatic abnormalities and, in particular, MRI showed poorly defined areas in the liver which, on T2-weighted sequences, exhibited a hypersignal with fat saturation. Treatment of porphyria cutanea tarda led to clinical remission and resolution of radiological abnormalities.

摘要

作者报告了1例迟发性皮肤卟啉病合并肝脏多结节性局灶性脂肪变性患者的超声、计算机断层扫描(CT)及磁共振成像(MRI)表现。此前仅有1篇关于这两种病症关联的描述。肝活检标本的组织学分析证实了诊断。与肝脏异常相关的症状可能归因于肝脏中尿卟啉结晶的存在所引发的炎症反应。每种放射学技术均显示出异常的肝脏表现,尤其是MRI显示肝脏内边界不清的区域,在T2加权序列上呈脂肪饱和的高信号。迟发性皮肤卟啉病的治疗使临床症状缓解,放射学异常表现消失。

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