摘要
目的分析儿童原发性局灶节段肾小球硬化症(FSGS)的临床资料,进一步探讨原发性FSGS的临床特点、病理类型及与预后之间的关系。方法回顾性分析2001年1月1日~2016年12月31日于重庆医科大学附属儿童医院确诊的65例原发性FSGS患儿的临床特点、病理类型及其预后。结果临床分型以肾病综合征最多见49例(75.38%);病理类型以非特殊型最多见(46.15%)。各病理类型之间的病初血甘油三酯水平比较,差异有统计学意义(P<0.05),其中顶端型最高,其后依次为塌陷型、细胞型、非特殊型,门部型最低。各病理类型的临床表现、临床分型、其余实验室检查比较,差异无统计学意义(P>0.05)。随访60例,5例失访,按随访终点的尿蛋白及肾功情况进行预后判断,预后好19例(31.67%),预后中等21例(35.00%),预后差20例(33.33%),其中进入终末期肾病或死亡16例(26.67%)。在随访5年、10年时,分别有40.00%、69.57%的病例进入终末期肾病或死亡。不同病理类型的预后比较,差异有统计学意义(P<0.05),塌陷型预后最差。病初有高血压、血肌酐及血甘油三酯水平与预后呈正相关(P<0.05)。结论33.33%的原发性FSGS患儿预后差,塌陷型较其他病理类型预后差,病初有高血压及血肌酐、甘油三酯水平越高,预后越差。
Objective To investigate the clinical materials of focal segmental glomerulosclerosis(FSGS) in children, and to analyze the relationship between clinical features, pathological variants and prognosis of FSGS.Methods The clinical, pathological features and prognosis of 65 children with FSGS in Children's Hospital Affiliated to Chongqing Medical University from January 1, 2001 to December 31, 2016 were analyzed retrospectively.Results Nephrotic syndrome was the most common type in clinical classification, accounting for 49 cases (75.38%);non-specific types was the most common pathological variant (46.15%). There was significant difference in initial triglyceride levels among five pathological variants (P<0.05), the highest was glomerular tip lesion, followed by collapsing, cellular and not-otherwise specified, the lowest was perihilar variants. There was no significant difference in clinical features, other laboratory tests and clinical types among five pathological variants (P>0.05). 60 cases were followed up and 5 cases were lost. According to the urine protein and renal function at the end of follow-up, the prognosis was good in 19 cases (31.67%), medium in 21 cases (35.00%) and poor in 20 cases (33.33%), among which 16 (26.67%) patients reached end-stage renal disease or died. 40.00% and 69.57% patients reached end-stage renal disease or died after followed up by 5 years and 10 years, respectively. There was significant difference in prognosis of different pathological variants (P<0.05), and the prognosis of collapsing was worst. Initial hypertension, serum creatinine and blood triglyceride levels were positively correlated with prognosis (P<0.05).Conclusion The prognosis of 33.33% children with FSGS was poor. Collapsing variant had a worse prognosis than other pathological variants. The initial hypertension existed, and the higher the level of serum creatinine and triglyceride, the worse the prognosis.
作者
杨琴
李佳承
杨帆
王墨
YANG Qin;LI Jia-cheng;YANG Fan;WANG Mo(Department of Nephrology,Children's Hospital Affiliated to Chongqing Medical University,Chongqing 400014,China)
出处
《医学信息》
2019年第12期89-92,共4页
Journal of Medical Information
