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一例伴有远端肾小管酸中毒和溶血性贫血的罕见家族性系统性红斑狼疮病例。

AN UNUSUAL CASE OF FAMILIAL SYSTEMIC LUPUS ERYTHEMATOSUS WITH DISTAL RENAL TUBULAR ACIDOSIS AND HEMOLYTIC ANEMIA.

作者信息

Agrwal Shipra, Mantan Mukta, Dabas Aashima

机构信息

MD pediatrics.

出版信息

Iran J Kidney Dis. 2019 Sep;13(5):337-339.

Abstract

Systemic lupus erythematosus (SLE) in children is associated with renal involvement in a majority. While glomerular involvement is a common manifestation, tubular involvement is rare. Tubular dysfunctions previously described with SLE are renal tubular acidosis (Type 1 and less commonly type 4). Isolated renal tubular acidosis without glomerular involvement has not been reported in childhood SLE. We report an adolescent girl with SLE, autoimmune thyroiditis and distal RTA who subsequently developed autoimmune hemolytic anemia. During a follow up of almost 7 years the girl never developed any proteinuria while the RTA persisted. Also during this period her mother was also diagnosed with SLE manifesting as thrombocytopenia.

摘要

儿童系统性红斑狼疮(SLE)多数伴有肾脏受累。虽然肾小球受累是常见表现,但肾小管受累罕见。先前描述的与SLE相关的肾小管功能障碍是肾小管酸中毒(1型,4型较少见)。儿童SLE中尚未报道过无肾小球受累的孤立性肾小管酸中毒。我们报告一名患有SLE、自身免疫性甲状腺炎和远端肾小管酸中毒的青春期女孩,随后发展为自身免疫性溶血性贫血。在近7年的随访中,该女孩从未出现蛋白尿,而肾小管酸中毒持续存在。在此期间,她的母亲也被诊断为表现为血小板减少症的SLE。

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