膜样肾小球病伴隐蔽性IgG-k沉积1例小儿特发性关节炎。

IF 2.6 3区 医学 Q1 PEDIATRICS
Priyanka Chati, Jill Krissberg, Kammi Henriksen, Brian Nolan, Meredith Harris
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引用次数: 0

摘要

膜样肾小球病伴隐匿性IgG-kappa沉积(mgid)是一种罕见的疾病,主要发生在既往诊断为自身免疫性疾病的年轻女性中。我们报告了一位12岁的女性,患有系统性青少年特发性关节炎(sJIA),尽管肾功能正常,但伴有持续性非肾病范围蛋白尿。她接受了两次肾脏活检,第二次最终证实了她的诊断。初步活检提示轻度C3肾小球肾炎(C3GN)。她开始使用血管紧张素转换酶抑制剂(ACE-I),但没有好转。蛋白尿进展到肾病范围,促使开始使用大剂量类固醇,随后类固醇逐渐减少。由于持续的蛋白尿,在类固醇断奶期间添加麦考酚酸酯。尽管进行了全剂量霉酚酸盐和ACE-I治疗,但由于缺乏反应,再次进行了活检,发现了mgid。她继续服用全剂量的霉酚酸酯和赖诺普利,她的蛋白尿有明显改善。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Membranous-like glomerulopathy with masked IgG-k deposits in a pediatric patient with juvenile idiopathic arthritis.

Membranous-like glomerulopathy with masked IgG-kappa deposits (MGMID) is a rare entity described primarily among young females with previously diagnosed autoimmune diseases. We present a 12-year-old female with systemic juvenile idiopathic arthritis (sJIA) with persistent non-nephrotic range proteinuria despite normal kidney function. She underwent two kidney biopsies with the second ultimately confirming her diagnosis. The initial biopsy was suggestive of mild C3 glomerulonephritis (C3GN). She was started on an angiotensin-converting enzyme inhibitor (ACE-I) without improvement. Proteinuria progressed to the nephrotic range, prompting initiation of high-dose steroids followed by a steroid taper. Mycophenolate was added during steroid weaning due to ongoing proteinuria. Despite full-dose mycophenolate and ACE-I therapy, a repeat biopsy was performed due to lack of response and revealed MGMID. She remains on full-dose mycophenolate and lisinopril with significant improvement in her proteinuria.

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来源期刊
Pediatric Nephrology
Pediatric Nephrology 医学-泌尿学与肾脏学
CiteScore
4.70
自引率
20.00%
发文量
465
审稿时长
1 months
期刊介绍: International Pediatric Nephrology Association Pediatric Nephrology publishes original clinical research related to acute and chronic diseases that affect renal function, blood pressure, and fluid and electrolyte disorders in children. Studies may involve medical, surgical, nutritional, physiologic, biochemical, genetic, pathologic or immunologic aspects of disease, imaging techniques or consequences of acute or chronic kidney disease. There are 12 issues per year that contain Editorial Commentaries, Reviews, Educational Reviews, Original Articles, Brief Reports, Rapid Communications, Clinical Quizzes, and Letters to the Editors.
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