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Two cases of atypical membranoproliferative glomerulonephritis showing opposite clinical course

机译:两例非典型性膜增生性肾小球肾炎临床表现相反

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Atypical membranoproliferative glomerulonephritis (MPGN) is considered to progress to typical MPGN, and it is believed that it can be treated with corticosteroids. However, consensus that atypical MPGN is a continuum of morphologic manifestations of typical MPGN cannot be reached. Herein, we report two cases of atypical MPGN with opposite clinical course. Case 1 was a 4-year-old boy with macrohematuria and proteinuria with no prodromal symptoms. His serum C3 level had abruptly dropped, and renal biopsy confirmed a diagnosis of atypical MPGN. After performing kidney biopsy, his urinary abnormality improved and his C3 level had normalized 1 year after onset without medication. At the most recent follow-up, neither proteinuria nor hematuria was detected. Case 2 was a 7-year-old girl with microhematuria and proteinuria at her school urinary screening. Her first biopsy finding was similar to dense deposit disease, and the second biopsy showed atypical MPGN. Oral corticosteroids were started from this point, but heavy proteinuria and hypocomplementemia could not be improved sufficiently. We immediately performed third kidney biopsy and diagnosed typical MPGN. These findings suggest that the indication of therapy for atypical MPGN should be re-examined. Aggressive therapy such as steroid administration is not necessarily essential and effective for therapeutic intervention of all atypical MPGN. Moreover, atypical MPGN may involve different etiologic and pathogenetic factors, rather than a continuum of morphologic manifestations of MPGN.
机译:非典型性膜增生性肾小球肾炎(MPGN)被认为发展为典型的MPGN,据信可以用糖皮质激素治疗。但是,无法达成共识,即非典型MPGN是典型MPGN形态学表现的连续体。在此,我们报告了两例临床过程相反的非典型MPGN病例。病例1是一个4岁男孩,患有大血尿和蛋白尿,无前驱症状。他的血清C3水平突然下降,并且肾脏活检证实了非典型MPGN的诊断。进行肾脏活检后,他的尿液异常状况得到改善,发病后一年(未用药)C3水平恢复正常。在最近的随访中,未检测到蛋白尿和血尿。案例2是一名7岁的女孩,在她的学校尿液筛查中有微血尿和蛋白尿。她的第一次活检发现类似于致密沉积病,第二次活检显示为非典型MPGN。从这一点开始口服皮质类固醇激素,但是不能充分改善重蛋白尿和低补体血症。我们立即进行了第三次肾脏活检,并诊断出典型的MPGN。这些发现表明,应重新检查非典型MPGN的治疗指征。对于所有非典型MPGN的治疗干预,积极的治疗(例如类固醇给药)不一定是必不可少的和有效的。此外,非典型MPGN可能涉及不同的病因和致病因素,而不是MPGN形态学表现的连续体。

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