Çocukluk Çağı Membranöz Glomerülonefrit Tanılı Hastaların Klinik Değerlendirmesi

Deniz Karakaya, Fatma Yazilitaş, Evrim KARGIN ÇAKICI, Tülin Güngör, Evra Çeli̇kkaya, M. Bülbül
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Abstract

Objective: Membranous nephropathy (MN) is a rare immune complex disease in pediatric population then adults. The prognosis of MN is variable, ranging from spontaneous complete remission to end-stage renal disease (ESRD). The lack of large multicenter studies precludes the possibility of examining in detail the treatment options and clinical outcomes in these patients. The present study aimed to expand the literature on the clinical findings, treatment, and prognosis of MN in pediatric patients. Material and Methods: This single-center retrospective study included 13 patients with a diagnosis of primary and secondary membranous nephropathy. Results: The mean age of the sample was 12.29±3.67 years. Complete remission occurred in 7 (53.8%) patients (of which 1 case was spontaneous remission), and partial remission occurred in 4 (30.8%) patients. In long-term follow-ups; one patient had chronic kidney disease (CKD) and one patient had end-stage renal disease (ESRD). At the last-follow up, proteinuria was noted in 6 (46.2%) patients and microscopic hematuria was noted in 4 (30.8%) and 9 patients were still using low-dose steroids. Conclusion: The current findings have not identified any significant risk factors associated with the prognosis of MN in pediatric patients, but are thought to contribute to the limited data on pediatric MN. Most of the available data on the natural history, treatment options, and long-term outcomes of MN in the pediatric population consists of small, uncontrolled case series. Therefore, we think that larger-scale clinical trials are necessary to clearly elucidate the factors related to the prognosis of pediatric MN.
儿童膜性肾小球肾炎患者的临床评估
目的:膜性肾病(MN)是一种少见的免疫复合物疾病,在儿童中发病率高于成人。MN的预后是可变的,从自发完全缓解到终末期肾脏疾病(ESRD)。缺乏大型多中心研究排除了详细检查这些患者的治疗方案和临床结果的可能性。本研究旨在扩大关于小儿MN的临床表现、治疗和预后的文献。材料和方法:本单中心回顾性研究纳入13例诊断为原发性和继发性膜性肾病的患者。结果:患者平均年龄12.29±3.67岁。完全缓解7例(53.8%),其中自发缓解1例,部分缓解4例(30.8%)。在长期随访中;1例患者患有慢性肾病(CKD), 1例患者患有终末期肾病(ESRD)。最后随访时,蛋白尿6例(46.2%),显微镜下血尿4例(30.8%),9例患者仍在使用小剂量类固醇。结论:目前的研究结果尚未确定与儿科患者MN预后相关的任何重要危险因素,但被认为有助于儿科MN的有限数据。大多数关于小儿MN的自然史、治疗方案和长期结果的现有数据都是由小的、不受控制的病例系列组成的。因此,我们认为有必要进行更大规模的临床试验,以明确小儿MN预后的相关因素。
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