Long-term outcome in children and adults with classic focal segmental glomerulosclerosis
Long-term outcome in children and adults with classic focal segmental glomerulosclerosis
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DOI：
10.1053/ajkd.1998.v32.pm9669427
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发表时间：
1998-07-01
影响因子：
13.2
通讯作者：
Rao, P
中科院分区：
文献类型：
--
作者：
Cattran, DC;Rao, P
对来自多伦多肾小球肾炎登记处的93名典型局灶性节段性肾小球硬化患者(55名成人和38名儿童)进行了回顾性研究。平均随访期为11年，累积经验为1,053病人年。除了肾病综合征在儿童中更常见(成人为55%，儿童为76%；P<；0.05)外，成人和儿童在入选时的特征相似。然而，在疾病的演变过程中，这一比例变得非常相似，82%的成年人和89%的儿童出现肾病范围的蛋白尿。在最后一个观察点，患者(成人和儿童)的结局是完全缓解，22%对42%；终末期肾病，42%对34%；慢性肾功能不全，13%对11%；以及持续性异常，24%对13%。尽管完全缓解的儿童比成人多，但与治疗儿童相比，治疗成人的完全缓解率相同(44%比47%)。虽然这篇综述不能确定类固醇治疗的最佳持续时间，但超过6个月的治疗似乎没有好处。预测预后的最佳指南仍然是完全缓解，因为在这两个年龄组中，这一事件的长期肾脏存活率都是100%。那些没有完全缓解的人一般都会进步，尽管即使在10年后，成人的存活率也是62%，儿童的存活率是58%。(C)1998年，由国家肾脏基金会公司提供。
A retrospective study was conducted in 93 patients (55 adults and 38 children) with classical focal segmental glomerulosclerosis drawn from the Toronto Glomerulonephritis Registry. The average follow-up period was 11 years, with a cumulative experience of 1,053 patient-years. Both adults and children were similar in profile at the time of entry, except that the nephrotic syndrome was more common in children (55% of adults v 76% of children; P < 0.05). During evolution of the disease, however, the percentages became very similar with 82% of adults and 89% of children developing nephrotic-range proteinuria. At the last observation point, the outcome of patients (adults v children) was complete remission, 22% versus 42%; end stage renal disease, 42% versus 34%; chronic renal insufficiency, 13% versus 11%; and persisting abnormality, 24% versus 13%. Although there were more children than adults in complete remission, the rate was equal in the treated adults compared with the treated children (44% v 47%). Although optimal duration of steroid therapy cannot be determined by this review, treatment beyond 6 months does not appear to be beneficial. The best guide to prognosis remains complete remission, since long-term renal survival in both age groups with this event was 100%. Those without a complete remission generally progress, although even at 10 years the survival rate is 62% in adults and 58% in children. (C) 1998 by the National Kidney Foundation, Inc.