Beşbaş Nesrin, Ozaltin Fatih, Emre Sevinç, Anarat Ali, Alpay Harika, Bakkaloğlu Ayşin, Baskin Esra, Buyan Necla, Dönmez Osman, Düşünsel Ruhan, Ekim Mesiha, Göko Faysal, Gür-Güven Ayfer, Kavukçu Salih, Mir Sevgi, Sönmez Ferah
Department of Pediatric Nephrology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Turk J Pediatr. 2010 May-Jun;52(3):255-61.
The clinical course of focal segmental glomerulosclerosis (FSGS) is heterogeneous in children. To evaluate the clinical course and the predictors of outcome in Turkish children with primary FSGS, a retrospective study was conducted by the Turkish Pediatric Nephrology Study Group in 14 pediatric nephrology centers. Two hundred twenty-two patients (92 boys, 130 girls, aged 1-16 years) with biopsy-proven primary FSGS were included. One hundred forty-eight patients were followed-up for a median of 51 months (range: 0.26-270). The clinical course was characterized by complete remission in 50 (33.8%), persistent proteinuria in 50 (33.8%) and progression to renal failure in 48 (32.4%) patients. Progression to end-stage renal disease (ESRD) was significantly higher in patients who did not attain remission. Complete remission, partial remission and progress to renal failure were recorded in 37%, 32% and 28%, respectively, of the patients (n = 73) treated with prednisone combined cyclophosphamide/cyclosporine A. However, in patients (n = 33) treated with pulse methyl prednisolone plus oral prednisone (up to 20 months) combined with cyclophosphamide, complete remission in 51.5% and partial remission in 27.3% of the patients were noted. Progression to renal failure was observed in 9.1% of this group of patients. Multivariate analysis showed that only plasma creatinine at presentation was an independent predictive value for outcome. Patients with serum creatinine level higher than 1.5 mg/dl had 6.6 times increased rate of progression to renal failure. Failure to achieve remission is a predictor of renal failure in children with primary FSGS. The use of immunosuppressive treatment in conjunction with prolonged steroid seems beneficial in primary FSGS in children.
儿童局灶节段性肾小球硬化（FSGS）的临床病程具有异质性。为评估土耳其原发性FSGS儿童的临床病程及预后预测因素，土耳其儿科肾脏病研究组在14个儿科肾脏病中心进行了一项回顾性研究。纳入了222例经活检证实为原发性FSGS的患者（92例男孩，130例女孩，年龄1 - 16岁）。148例患者接受了中位时间为51个月的随访（范围：0.26 - 270个月）。临床病程特点为50例（33.8%）完全缓解，50例（33.8%）持续蛋白尿，48例（32.4%）进展至肾衰竭。未达到缓解的患者进展至终末期肾病（ESRD）的比例显著更高。接受泼尼松联合环磷酰胺/环孢素A治疗的患者（n = 73）中，分别有37%、32%和28%的患者达到完全缓解、部分缓解和进展至肾衰竭。然而，接受脉冲甲基泼尼松龙加口服泼尼松（长达20个月）联合环磷酰胺治疗的患者（n = 33）中，51.5%的患者达到完全缓解，27.3%的患者部分缓解。该组患者中有9.1%进展至肾衰竭。多因素分析显示，仅就诊时的血浆肌酐对预后具有独立预测价值。血清肌酐水平高于1.5 mg/dl的患者进展至肾衰竭的发生率增加6.6倍。未能达到缓解是原发性FSGS儿童肾衰竭的一个预测因素。在儿童原发性FSGS中，联合使用免疫抑制治疗和延长激素治疗似乎有益。
