Suppr超能文献

以胆汁淤积性黄疸为表现的轻链沉积病:一例报告

Light chain deposition disease presenting as cholestatic jaundice: a case report.

作者信息

Kumar Prasanna N

出版信息

Oman Med J. 2012 Jan;27(1):56-9. doi: 10.5001/omj.2012.12.

Abstract

Light-chain deposition disease (LCDD) is characterized by tissue deposition of the immunoglobulin light chains in multiple organs. These deposits appear similar to amyloid on routine sections, but differ in their staining properties and ultrastructural appearance. The deposits of LCCD are non -Congophilic and do not exhibit a fibrillar ultrastructure; while, the proteinaceous substance seen in primary amyloidosis is Congo red positive and fibrillar. One of the most common organs to be involved in LCDD is the kidney. Earlier reports on cases of LCDD have mostly shown simultaneous liver and renal involvement, there are very few cases in the literature describing LCDD of the liver without renal involvement. This report describes a patient who presented with severe cholestatic jaundice and liver cell failure with normal renal function.

摘要

轻链沉积病(LCDD)的特征是免疫球蛋白轻链在多个器官中沉积。这些沉积物在常规切片上看起来与淀粉样蛋白相似,但在染色特性和超微结构外观上有所不同。LCCD的沉积物不具有嗜刚果红性,也不呈现纤维状超微结构;而原发性淀粉样变性中所见的蛋白质物质刚果红呈阳性且为纤维状。LCDD最常累及的器官之一是肾脏。早期关于LCDD病例的报道大多显示肝脏和肾脏同时受累,文献中很少有描述无肾脏受累的肝脏LCDD病例。本报告描述了一名表现为严重胆汁淤积性黄疸和肝细胞功能衰竭但肾功能正常的患者。

相似文献

9
Coexistence of amyloidosis and light chain deposition disease in the heart.心脏淀粉样变性和轻链沉积病共存。
Cardiovasc Pathol. 2021 Mar-Apr;51:107315. doi: 10.1016/j.carpath.2020.107315. Epub 2020 Nov 29.

本文引用的文献

1
Light chain deposition disease presenting as massive hepatomegaly.
Pathology. 2010 Apr;42(3):307-10. doi: 10.3109/00313021003631171.
7
Kappa light chain deposition disease of the liver.肝脏κ轻链沉积病
Eur J Gastroenterol Hepatol. 1998 May;10(5):429-30. doi: 10.1097/00042737-199805000-00013.

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验