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Acquired Fanconi Syndrome with Proximal Tubular Cytoplasmic Fibrillary Inclusions of lambda Light Chain Restriction
Yao, Ying; Wang, Su-Xia1; Zhang, You-Kang; Wang, Yan; Liu, Li; Liu, Gang
关键词Fanconi Syndrome Immunoglobulin Light Chains Multiple Myeloma
刊名INTERNAL MEDICINE
2014
DOI10.2169/internalmedicine.53.0836
53期:2页:121-124
收录类别SCI
文章类型Article
WOS标题词Science & Technology
类目[WOS]Medicine, General & Internal
研究领域[WOS]General & Internal Medicine
英文摘要

Light chain proximal tubulopathy is a rarely reported entity associated with plasma cell dyscrasia that classically manifests as acquired Fanconi syndrome and is characterized by the presence of kappa-restricted crystals in the proximal tubular cytoplasm. We herein present a case of multiple myeloma with Fanconi syndrome and acute kidney injury due to light chain proximal tubulopathy with light chain cast nephropathy. Prominent phagolysosomes and numerous irregularly shaped inclusions with a fibrillary matrix in the cytoplasm of the proximal tubules were identified on electron microscopy. A monotypic light chain of the lambda type was detected in the distal tubular casts, proximal tubular cytoplasmic lysosomes and fibrillary inclusions on immunofluorescence and immune electron microscopy. This case underscores the importance of conducting careful ultrastructural investigations and immunocytologic examinations of light chains for detecting and diagnosing light chain proximal tubulopathy.

语种英语
WOS记录号WOS:000330216400008
引用统计
被引频次:2[WOS]   [WOS记录]     [WOS相关记录]
文献类型期刊论文
条目标识符http://ir.bjmu.edu.cn/handle/400002259/56681
专题北京大学第一临床医学院_肾脏内科
北京大学第一临床医学院_电镜室
作者单位1.Peking Univ, Dept Med, Div Renal, Hosp 1, Beijing, Peoples R China
2.Peking Univ, Inst Nephrol, Beijing, Peoples R China
3.Minist Hlth China, Key Lab Renal Dis, Beijing, Peoples R China
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GB/T 7714
Yao, Ying,Wang, Su-Xia,Zhang, You-Kang,et al. Acquired Fanconi Syndrome with Proximal Tubular Cytoplasmic Fibrillary Inclusions of lambda Light Chain Restriction[J]. INTERNAL MEDICINE,2014,53(2):121-124.
APA Yao, Ying,Wang, Su-Xia,Zhang, You-Kang,Wang, Yan,Liu, Li,&Liu, Gang.(2014).Acquired Fanconi Syndrome with Proximal Tubular Cytoplasmic Fibrillary Inclusions of lambda Light Chain Restriction.INTERNAL MEDICINE,53(2),121-124.
MLA Yao, Ying,et al."Acquired Fanconi Syndrome with Proximal Tubular Cytoplasmic Fibrillary Inclusions of lambda Light Chain Restriction".INTERNAL MEDICINE 53.2(2014):121-124.
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