印度的 IgM 肾病：单中心经验。
IgM nephropathy in India: a single centre experience.
机构信息
Department of Pathology, Laboratory Medicine, Transfusion Services and Immunohematology, G. R. Doshi and K. M. Mehta Institute of Kidney Diseases & Research Centre (IKDRC)- Dr. H.L. Trivedi Institute of Transplantation Sciences (ITS), Civil Hospital Campus, Asarwa, Ahmedabad, 380016 Gujarat, India.
出版信息
Indian J Pediatr. 2012 Aug;79(8):1025-7. doi: 10.1007/s12098-012-0693-0.
OBJECTIVE
To find out the incidence and natural history of IgMN in India.
METHODS
Renal biopsies of children ≤12 y age of last 6 y were retrospectively evaluated. Their clinical and biochemical presentation were correlated. Patients with systemic diseases/disorders were excluded from the study.
RESULTS
Immunoglobulin M nephropathy (IgMN) constituted 11.9% (n = 28) of 236 renal biopsies. Mean age was 10 y, predominant in boys (n = 24), most of the patients presented with proteinuria and edema. The most common associated histopathological finding was mesangial proliferative glomerulonephritis (MePGN) in 60.7% (n = 17) followed by minimal change disease (MCD) in 28.6% (n = 8) and focal segmental glomerulosclerosis (FSGS) in 10.7% (n = 3). In 85.7%, IgM appeared as the sole immunoglobulin deposit mainly in mesangial regions, followed by accompanied C3 in 3.6% (n = 1) and C1q + C3 in 10.7% (n = 3) biopsies.
CONCLUSIONS
IgMN was observed in 11.9% biopsies with commonest morphology of MePGN followed by MCD and FSGS; proteinuria was bad prognosticator in addition to FSGS and co-deposition of other immunoglobulins had no significance.
目的
了解印度 IgMN 的发病率和自然史。
方法
回顾性评估过去 6 年≤12 岁儿童的肾活检。将其临床表现和生化表现进行了相关性分析。将患有系统性疾病/障碍的患者排除在研究之外。
结果
免疫球蛋白 M 肾病（IgMN）构成了 236 例肾活检中的 11.9%（n=28）。平均年龄为 10 岁，主要为男性（n=24），大多数患者表现为蛋白尿和水肿。最常见的相关组织病理学发现是系膜增生性肾小球肾炎（MePGN）占 60.7%（n=17），其次是微小病变性肾病（MCD）占 28.6%（n=8）和局灶节段性肾小球硬化症（FSGS）占 10.7%（n=3）。在 85.7%的患者中，IgM 主要以单纯免疫球蛋白沉积物的形式出现，主要位于系膜区，其次是 3.6%（n=1）的 C3 伴随沉积和 10.7%（n=3）的 C1q+C3 沉积。
结论
IgMN 在 11.9%的活检中观察到，最常见的形态为 MePGN，其次是 MCD 和 FSGS；除 FSGS 外，蛋白尿是不良预后的预测因素，而其他免疫球蛋白的共同沉积则没有意义。
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