Ozkaya Nuray, Cakar Nilgün, Ekim Mesiha, Kara Nazli, Akkök Nermin, Yalçinkaya Fatos
Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey.
Pediatr Int. 2004 Aug;46(4):436-8. doi: 10.1111/j.1442-200x.2004.01920.x.
Minimal change nephrotic syndrome (MCNS) is the most common cause of primary nephrotic syndrome (NS) during childhood. However, recent studies from different countries have reported an increasing incidence of focal segmental glomerulosclerosis (FSGS) in children.
This is a retrospective study in which 392 Turkish children who were diagnosed with NS during the last 10 years and were followed for at least 2 years, were evaluated. Mean age of the study group was 4.6 +/- 3.4 years (range 0.9-16 years) and 232 were male and 160 were female.
In total, 280 patients were diagnosed as MCNS with their initial presentations, laboratory features, and clinical course. Kidney biopsy was performed in the remaining 112 children according to current recommendations. The results showed that membranoproliferative glomerulonephritis (MPGN) was the most common histopathologic diagnosis, 38 (34%) of the 112 patients were found to have MPGN. The number diagnosed as FSGS was 26 (23%). A significant difference was found between the age groups for both MPGN and FSGS, the former being more common in children >6 years of age and the latter more frequent in children </=6 years. MCNS was found in only 19% of all biopsies performed and the total incidence of MCNS (presumptive + biopsy proven) was 76%. There was no significant difference between the incidence of different histopathological subtypes before and after 1995.
Minimal change nephrotic syndrome is still the most common primary NS in childhood and MPGN is found to be the most common histopathologic subtype in the present patient population who underwent biopsy.
微小病变肾病综合征（MCNS）是儿童原发性肾病综合征（NS）最常见的病因。然而，来自不同国家的近期研究报告称儿童局灶节段性肾小球硬化（FSGS）的发病率在上升。
这是一项回顾性研究，对过去10年中被诊断为NS且随访至少2年的392名土耳其儿童进行了评估。研究组的平均年龄为4.6 +/- 3.4岁（范围0.9 - 16岁），男性232名，女性160名。
共有280例患者根据其初始表现、实验室特征和临床病程被诊断为MCNS。其余112名儿童根据当前建议进行了肾活检。结果显示，膜增生性肾小球肾炎（MPGN）是最常见的组织病理学诊断，112例患者中有38例（34%）被发现患有MPGN。诊断为FSGS的有26例（23%）。MPGN和FSGS在年龄组之间均存在显著差异，前者在>6岁儿童中更常见，后者在≤6岁儿童中更常见。在所有进行的活检中，仅19%发现为MCNS，MCNS的总发病率（推测性 + 活检证实）为76%。1995年前后不同组织病理学亚型的发病率无显著差异。
微小病变肾病综合征仍然是儿童期最常见的原发性NS，在本接受活检的患者群体中，MPGN被发现是最常见的组织病理学亚型。
