目的 总结近10年西部地区某三甲医院慢性肾脏病(CKD)5期患儿的病因及临床特征。方法 回顾性收集2010年1月1日至2021年6月30日就诊于宁夏医科大学总医院的50例0~18岁CKD5期患儿的临床资料，对其病因构成及临床特征进行分析。结果 50例CKD5期患儿中男31例(62.0 %)，女19例(38.0 %)；发病年龄12.7(0.5~18.0)岁，其中0~6岁8例(16.0 %)，7~12岁17例(34.0 %)，13~18岁25例(50.0 %)；首诊无明显症状9例(18.0 %)，以肾脏症状为主15例(30.0 %)，以肾外症状为主26例(52.0 %)；原发病因为肾小球疾病26例(52%)、先天性肾脏及尿路畸形12例(24.0 %)；并发症中贫血49例(98.0 %)，慢性肾脏病-矿物质和骨异常(CKD-MBD)包括高磷血症49例(98.0 %)、继发性甲状旁腺功能亢进38例(76.0 %)、低钙血症33例(66.0 %)、维生素D缺乏27例(54.0 %)，水电解质酸碱紊乱包括高尿酸血症40例(80.0 %)、高钾血症15例(30.0 %)，高血压28例(56.0 %)，生长障碍18例(36.0 %)；完善肾脏穿刺活检术7例(14.0 %)，全外显子基因测序5例(10.0 %)；失访2例(4.0 %)，死亡11例(22.0 %)，长期进行血液透析15例(30.0 %)，长期进行腹膜透析13例(26.0 %)，肾脏移植11例(22.0 %)。结论 本地区CKD5期患儿主要病因为肾小球疾病；发生率男性高于女性，年长儿高于低年龄儿童；首诊症状以肾外表现多见，最常见的并发症为贫血及CKD-MBD；完成肾脏移植的患儿预后较好，但绝大部分患儿选择透析治疗，以血液透析为主；主要死亡原因为重症感染和心血管系统疾病。

Objective To summarize the etiology and clinical features of 50 children with stage 5 chronic kidney disease (CKD) treated at a tertiary hospital in western China in the past 10 years and to establish a theoretical basis for the investigation of chronic kidney disease in children. Methods The clinical data of 50 children aged 0-18 years with CKD5 who visited the General Hospital of Ningxia Medical University from January 1, 2010 to June 30, 2021 were retrospectively collected, and their etiological composition and clinical features were analyzed. Results Among the 50 children with CKD stage 5, 31 (62.0 %) were male and 19 (38.0 %) were female. The age of onset was 0.5-18 years old, including 8 cases (16.0 %) aged 0-6 years, 17 cases (34.0 %) 7-12 years old, and 25 cases (50.0 %) aged 13-18 years. At the initial diagnosis, there were 9 cases (18.0 %) with no obvious symptoms, 15 cases (30.0 %) with primarily renal symptoms, and 26 cases (52.0 %) with mainly extrarenal symptoms. The primary cause of the disease was glomerular disease in 26 cases (52%) and congenital renal and urinary tract malformations in 12 cases (24.0 %). Complications observed were anemia in 49 cases (98.0 %), chronic kidney disease-mineral and bone abnormalities (CKD-MBD) included hyperphosphatemia in 49 cases (98.0 %), secondary hyperparathyroidism in 38 cases (76.0 %), hypocalcemia in 33 cases (66.0 %), vitamin D deficiency in 27 cases (54.0 %), water electrolyte acid-base disorders including hyperuricemia in 40 cases (80.0 %), hyperkalemia in 15 cases (30.0 %), hypertension in 28 cases (56.0 %), growth disorder in 18 cases (36.0 %), and refinement of nephropuncture biopsy in 7 cases (14.0 %), exoms sequencing in 5 cases (10.0 %). loss of follow-up in 2 cases (4.0 %), death in 11 cases (22.0 %), long-term hemodialysis in 15 cases (30.0 %), long-term peritoneal dialysis in 13 cases (26.0 %), and kidney transplantation in 11 cases (22.0 %). Conclusions The main cause of children with CKD stage 5 in this region is glomerular disease; the incidence rate is higher in males than females, and higher in older than younger children; the first symptom is more common with extra-renal manifestations, and the most common complications are anemia and CKD-MBD; the prognosis of children who have completed renal transplantation is better, but the vast majority of them opt for dialysis treatment, which is predominantly hemodialysis; and the main causes of death are severe infections and diseases of cardiovascular system.