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一例以肾病综合征为表现的原发性免疫缺陷病。

A case of primary immune deficiency presenting with nephrotic syndrome.

作者信息

Aydin Zeki, Gursu Meltem, Ozturk Savas, Kilicaslan Isın, Kazancioglu Rumeyza

机构信息

Department of Nephrology , Haseki Training and Research Hospital , Istanbul , Turkey.

Department of Pathology, Istanbul Medical Faculty , Istanbul University , Istanbul , Turkey.

出版信息

NDT Plus. 2010 Oct;3(5):456-8. doi: 10.1093/ndtplus/sfq083. Epub 2010 May 11.

DOI:10.1093/ndtplus/sfq083
PMID:25984052
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4421686/
Abstract

Common variable immunodeficiency (CVID) is the most common form of severe antibody deficiency. The disorder is associated with a broad spectrum of clinical manifestations, including infections and chronic lung, gastrointestinal and autoimmune diseases. A 29-year-old female patient has had frequent sinopulmonary infections and gastroenteritis for the last 20 years and had been given broad-spectrum antibiotics for treatment. Immunoglobulin (Ig) levels were at undetectable levels. Renal biopsy was consistent with AA amyloidosis. She is now under follow-up with periodic intravenous Ig treatment without any infection during the last 10 months. CVID must be kept in mind in patients with recurrent sinopulmonary infections.

摘要

普通可变型免疫缺陷(CVID)是严重抗体缺陷最常见的形式。该病症与广泛的临床表现相关,包括感染以及慢性肺部、胃肠道和自身免疫性疾病。一名29岁女性患者在过去20年中频繁发生鼻窦肺部感染和肠胃炎,并接受了广谱抗生素治疗。免疫球蛋白(Ig)水平检测不到。肾活检符合AA型淀粉样变性。她目前正在接受定期静脉注射Ig治疗的随访,在过去10个月中未发生任何感染。对于反复发生鼻窦肺部感染的患者,必须考虑到CVID。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/397e/4421686/8d179c1ea69f/sfq083fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/397e/4421686/add2c4ca8dc4/sfq083fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/397e/4421686/8d179c1ea69f/sfq083fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/397e/4421686/add2c4ca8dc4/sfq083fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/397e/4421686/8d179c1ea69f/sfq083fig2.jpg

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本文引用的文献

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Am J Surg Pathol. 2007 Dec;31(12):1800-12. doi: 10.1097/PAS.0b013e3180cab60c.
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Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency.一大群常见可变免疫缺陷患者的长期随访及结果
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