摘要目的:探讨儿童慢性肾脏病(CKD)的病因构成、并发症及治疗情况，为儿童CKD的综合管理提供依据。方法:收集2012年1月至2018年12月在首都医科大学附属北京儿童医院肾脏科住院的CKD患儿临床资料，对其病因构成、并发症、初始治疗情况等信息进行回顾性调查及分析。结果:1．本组371例CKD患儿中，男女比例为1.44∶1.00。年龄分期0～3岁35例，4～6岁54例，7～12岁189例，13～18岁93例。CKD 2期11例，CKD 3期59例，CKD 4期62例，CKD 5期239例。2.先天性肾脏和尿道畸形(CAKUT) 135例，占36.39%；肾小球疾病77例，占20.76%；遗传性肾脏疾病21例，占5.66%；肾小管间质疾病12例，占3.23%；遗传代谢病4例，占1.08%；其他病因5例，占1.35%；病因不详117例，占31.64%。3.其中57例进行了肾活检，肾活检率为15.36%。病理类型以局灶节段性肾小球硬化(18例，31.58%)、硬化性肾小球肾炎(13例，22.81%)和肾小管间质肾病(10例，17.54%)为主。4.贫血和继发性甲状旁腺功能亢进(SHPT)是最常见的并发症，分别为289例(77.90%)和271例(73.05%)，其次为高血压[183例(49.33%)]、心血管疾病[139例(37.47%)]和蛋白质能量消耗[51例(13.75%)]。CKD 5期患儿高血压、贫血、SHPT和心血管疾病均明显高于CKD 2～4期，差异均有统计学意义( χ2＝50.03、122.36、77.07、64.89，均 P<0.01)。肾小球疾病的高血压和心血管疾病发生率高于CAKUT，差异均有统计学意义( χ2＝65.63、40.89，均 P<0.01)。CAKUT的蛋白质能量消耗发生率高于肾小球疾病，差异有统计学意义( χ2＝10.58， P<0.01)。5.共190例患儿需进行初始肾脏替代治疗，血液透析129例(67.89%)，腹膜透析31例(16.32%)，拒绝治疗30例(15.79%)，初始移植0例。 结论:CKD患儿CAKUT为首位病因。本中心CKD 5期CAKUT和肾小球疾病所占比例相近。儿童CKD最常见并发症为贫血。高血压、贫血、SHPT和心血管疾病等随着CKD分期进展呈增多趋势。SHPT多发生于CKD 4期以上患儿。不同病因CKD患儿并发症发生率不同。本中心初始肾脏替代治疗以血液透析为主。

abstractsObjective:To investigate the etiology, complications and treatment of children with chronic kidney disease(CKD), in order to provide evidence for the comprehensive management.Methods:The clinical data of 371 children patients with CKD at stage 2 to 5 admitted to the Department of Nephrology, Beijing Children′s Hospital Affiliated to Capital Medical University from January 2012 to December 2018 were collected.The etiology, complications and treatment and other data were retrospectively investigated and analyzed.Results:(1)A total of 371 children with CKD were enrolled, and the male to female ratio was 1.44∶1.00.Thirty-five cases aged from 0 to 3, 54 cases aged from 4 to 6, 189 cases aged from 7 to 12, 93 cases aged from 13 to 18.Eleven cases were diagnosed at stage 2, 59 cases at stage 3, 62 cases at stage 4, and 239 cases at stage 5.(2) In all patients, 135 cases (36.39%) had congenital anomalies of the kidney and urinary tract(CAKUT), 77 cases (20.76%) had glomerular diseases, 21 cases (5.66%) had hereditary kidney diseases, 12 cases (3.23%) had tubulointerstitial diseases, 4 cases (1.08%) had inherited metabolic diseases, 5 cases (1.35%) had other diseases and in 117 cases (31.64%) the causes of disease were unknown.(3) Renal biopsy was performed in 57 cases with the rate of renal biopsy of 15.36%.The main pathologic types included focal segmental glomerulosclerosis(18 cases, 31.58%), sclerosing glomerulonephritis (13 cases, 22.81%) and tubulointerstitial nephropathy (10 cases, 17.54%). (4)Anemia and secondary hyperparathyroidism(SHPT) were the most common complications, accounting for 77.90% (289 cases) and 73.05% (271 cases), respectively, followed by hypertension (183 cases, 49.33%), cardiovascular disease (CVD) (139 cases, 37.47%) and protein-energy wasting (PEW) (51 cases, 13.75%) successively.The incidence of hypertension, anemia, SHPT and CVD in children with CKD at stage 5 were significantly higher than those in CKD at stage 2-4, and the differences were statistically significant( χ2=50.03, 122.36, 77.07, 64.89, all P<0.01). The incidence of hypertension and CVD in patients with glomerular diseases were higher than those in CAKUT patients, and the differences were statistically significant( χ2=65.63, 40.89, all P<0.01). The incidence of PEW in CAKUT was higher than that in patients with glomerular diseases, and the difference was statistically significant( χ2=10.58, P<0.01). (5)Initial renal replacement therapy was performed in 190 children, hemodialysis in 129 cases (67.89%), peritoneal dialysis in 31 cases (16.32%), and 30 cases (15.79%) refused treatment There was no transplant patient in initial treatment modality. Conclusions:In the center, the major cause of CKD stage 2 to 5 in children was CAKUT, but the proportion of CAKUT and glomerular diseases was similar in CKD stage 5.The most common complication of CKD in children is anemia.Hypertension, anemia, SHPT and CVD increased with the progression of CKD staging.SHPT usually occurs in children with CKD stage 4 and 5.The incidence of complications in children with CKD caused by different factors is different.Hemodialysis is the main method of initial renal replacement therapy in the center.