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与镰状细胞性状相关的自体免疫复合物肾炎:肾脏结构和免疫学研究后血红蛋白病的诊断

Autologous immune complex nephritis associated with sickle cell trait: diagnosis of the haemoglobinopathy after renal structural and immunological studies.

作者信息

Ozawa T, Mass M F, Guggenheim S, Strauss J, McIntosh R M

出版信息

Br Med J. 1976 Feb 14;1(6006):369-71. doi: 10.1136/bmj.1.6006.369.

Abstract

A renal tubular epithelial antigen (RTE)--anti-RTE autologous immune complex nephritis associated with sickle cell anaemia (SS) has been reported, but immune complex nephritis has never been described in patients with sickle cell trait (SA). During investigation of a child with "asymptomatic proteinuria" cryoprecipitable complexes of RTE-anti-RTE were detected in the serum and granular deposits of RTE, immunoglobulins, and complement localised on the glomerular basement membranes. Morphological and ultrastructural studies showed increased mesangial matrix, sickled red blood cells in the glomeruli and vessels, and tubular and interstitial abnormalities. These findings prompted haemoglobin electrophoretic studies, which showed previously undiagnosed haemoglobin SA in this patient and her family. These observations suggest that nephritis mediated by similar immunopathogenic mechanisms may be associated with SS and SA haemoglobinopathy. Under some conditions patients with sickle cell trait may experience haemodynamic and oxygenation abnormalities, which may be aetiological factors in the immune complex nephritis associated with SS disease.

摘要

据报道,一种与镰状细胞贫血(SS)相关的肾小管上皮抗原(RTE)-抗RTE自身免疫复合物性肾炎,但镰状细胞性状(SA)患者的免疫复合物性肾炎从未被描述过。在对一名患有“无症状蛋白尿”的儿童进行调查时,在血清中检测到了RTE-抗RTE的冷沉淀复合物,并且在肾小球基底膜上发现了RTE、免疫球蛋白和补体的颗粒状沉积物。形态学和超微结构研究显示系膜基质增加、肾小球和血管中有镰状红细胞以及肾小管和间质异常。这些发现促使进行血红蛋白电泳研究,结果显示该患者及其家族此前未被诊断出患有血红蛋白SA。这些观察结果表明,由类似免疫致病机制介导的肾炎可能与SS和SA血红蛋白病有关。在某些情况下,镰状细胞性状患者可能会出现血流动力学和氧合异常,这可能是与SS病相关的免疫复合物性肾炎的病因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a3c7/1638741/ac29d5fc83ab/brmedj00503-0022-a.jpg

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