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与肠道淋巴管扩张症相关的复发性溶血尿毒综合征

Recurrent hemolytic uremic syndrome associated with intestinal lymphangiectasia.

作者信息

Kalman Süleyman, Bakkaloğlu Sevcan, Dalgiç Buket, Ozkaya Ozan, Söylemezoğlu Oğuz, Buyan Necla

机构信息

Department of Pediatric Nephrology, Gazi University, Besevler, Ankara, Turkey.

出版信息

J Nephrol. 2007 Mar-Apr;20(2):246-9.

PMID:17514630
Abstract

A 17-year-old boy was admitted to the hospital twice in a year for 2 episodes of hemolytic uremic syndrome (HUS). During these 2 HUS episodes he had diarrhea, decreased serum complement, decreased total protein and decreased serum albumin concentrations. We suggest that protein-losing enteropathy and hypocomplementemia due to intestinal lymphangiectasia is may be a rare cause of atypical HUS.

摘要

一名17岁男孩一年内因两次溶血性尿毒症综合征(HUS)发作而两次入院。在这两次HUS发作期间,他出现腹泻、血清补体降低、总蛋白降低和血清白蛋白浓度降低。我们认为,由于肠道淋巴管扩张引起的蛋白丢失性肠病和低补体血症可能是不典型HUS的罕见病因。

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1
Recurrent hemolytic uremic syndrome associated with intestinal lymphangiectasia.与肠道淋巴管扩张症相关的复发性溶血尿毒综合征
J Nephrol. 2007 Mar-Apr;20(2):246-9.
2
Recurrent hemolytic uremic syndrome with hypocomplementemia and intestinal lymphangiectasia.复发性溶血尿毒症综合征伴低补体血症和肠淋巴管扩张症。
Nephron. 1997;76(4):481-4. doi: 10.1159/000190232.
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[Primary intestinal lymphangiectasis (Waldmann's disease) and recurrent hemolytic uremic syndrome--a pathogenetic connection?].
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Familial pachydermoperiostosis in association with protein-losing enteropathy.家族性厚皮性骨膜病伴蛋白丢失性肠病。
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Hemolytic uremic syndrome with hypocomplementemia, serum C3NeF, and glomerular deposits of C3.伴有低补体血症、血清C3NeF及C3肾小球沉积物的溶血性尿毒症综合征
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Primary intestinal lymphangiectasia: French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins).原发性肠淋巴管扩张症:法国国家诊断与治疗方案(PNDS;Protocole National de Diagnostic et de Soins)
Orphanet J Rare Dis. 2025 Jul 10;20(1):356. doi: 10.1186/s13023-025-03657-9.
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Protein-losing enteropathy as a new phenotype in atypical hemolytic uremic syndrome caused by CD46 gene mutation.CD46 基因突变导致非典型溶血尿毒症综合征中肠病相关性蛋白丢失:一种新表型。
Pediatr Nephrol. 2024 Dec;39(12):3513-3520. doi: 10.1007/s00467-024-06451-0. Epub 2024 Aug 3.
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Primary intestinal lymphangiectasia in an elderly female patient: A case report on a rare cause of secondary immunodeficiency.
老年女性患者的原发性肠淋巴管扩张症:一例罕见的继发性免疫缺陷病因病例报告
Medicine (Baltimore). 2017 Aug;96(31):e7729. doi: 10.1097/MD.0000000000007729.
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Eculizumab in secondary atypical haemolytic uraemic syndrome.依库珠单抗治疗继发性非典型溶血性尿毒症综合征
Nephrol Dial Transplant. 2017 Mar 1;32(3):466-474. doi: 10.1093/ndt/gfw453.
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Anaemia in Waldmann's disease: A rare presentation of a rare disease.瓦尔登斯特伦巨球蛋白血症中的贫血:一种罕见疾病的罕见表现。
World J Gastrointest Endosc. 2015 May 16;7(5):567-72. doi: 10.4253/wjge.v7.i5.567.
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Primary intestinal lymphangiectasia: Minireview.原发性肠淋巴管扩张症:综述
World J Clin Cases. 2014 Oct 16;2(10):528-33. doi: 10.12998/wjcc.v2.i10.528.
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Intestinal lymphangiectasia in adults.成人肠淋巴管扩张症。
World J Gastrointest Oncol. 2011 Feb 15;3(2):19-23. doi: 10.4251/wjgo.v3.i2.19.
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Primary intestinal lymphangiectasia: four case reports and a review of the literature.原发性肠道淋巴管扩张症:4 例病例报告及文献复习。
Dig Dis Sci. 2010 Dec;55(12):3466-72. doi: 10.1007/s10620-010-1161-1. Epub 2010 Mar 3.
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Primary intestinal lymphangiectasia (Waldmann's disease).原发性肠淋巴管扩张症(瓦尔德曼病)。
Orphanet J Rare Dis. 2008 Feb 22;3:5. doi: 10.1186/1750-1172-3-5.