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肾衰竭儿童的获得性囊性肾病

Acquired cystic kidney disease in children with kidney failure.

作者信息

Ma Justin Ming-Yin, Fung Kin-Fen Kevin, Tong Pak-Chiu, Lai Wai-Ming, Ma Alison Lap-Tak, Chan Eugene Yu-Hin

机构信息

Paediatric Nephrology Centre, Hong Kong Children's Hospital, Hong Kong, Hong Kong SAR.

Department of Radiology, Hong Kong Children's Hospital, Hong Kong, Hong Kong SAR.

出版信息

Pediatr Nephrol. 2025 May;40(5):1741-1750. doi: 10.1007/s00467-024-06628-7. Epub 2025 Jan 4.

Abstract

BACKGROUND

This study aimed to evaluate the incidence, contributing factors, and clinical outcomes of acquired cystic kidney disease (ACKD) in children undergoing kidney replacement therapy (KRT).

METHODS

We conducted a cross-sectional, territory-wide study at the designated pediatric nephrology center in Hong Kong. ACKD was defined as the presence of ≥ 3 cysts in the native kidneys, excluding congenital or hereditary cystic diseases. Between June to December 2023, all paediatric patients receiving KRT in Hong Kong underwent ultrasonography, non-contrast magnetic resonance imaging (MRI), or both. Contrast-enhanced computed tomography was performed for patients with complex cysts.

RESULTS

Forty-three children (56% female; median age 14.7 years; IQR, 11.7-18.7) were included in the analysis. ACKD was detected in 18 children (42%). Nine subjects had complex cysts (grade 2, n = 5; grade 2F, n = 2; grade 3, n = 2). Most patients with ACKD (89%) were asymptomatic. One patient (5.5%) developed back pain and gross haematuria 72 months after initiation of KRT. Another patient (5.5%) developed infected cyst with back pain and clinical sepsis 60 months following KRT initiation. A dialysis duration of ≥ 28 months was the only significant factor associated with ACKD development (77.8% vs. 40%; p = 0.028; OR 6.09, 95% CI 1.43-25.82, p = 0.014). The diagnostic yield of paired ultrasound and MRI was superior to ultrasound alone.

CONCLUSIONS

ACKD is prevalent among children and adolescents with kidney failure, with most cases being asymptomatic, however serious complications may arise. Longer duration of dialysis is significantly associated with ACKD development. Therefore, early transplantation and active ACKD surveillance are crucial for children receiving KRT.

摘要

背景

本研究旨在评估接受肾脏替代治疗(KRT)的儿童获得性囊性肾病(ACKD)的发病率、相关因素及临床结局。

方法

我们在香港指定的儿科肾脏病中心进行了一项全地区横断面研究。ACKD定义为在天然肾脏中存在≥3个囊肿,排除先天性或遗传性囊性疾病。2023年6月至12月期间,香港所有接受KRT的儿科患者均接受了超声检查、非增强磁共振成像(MRI)或两者检查。对有复杂囊肿的患者进行了对比增强计算机断层扫描。

结果

43名儿童(56%为女性;中位年龄14.7岁;四分位间距,11.7 - 18.7)纳入分析。18名儿童(42%)检测出ACKD。9名受试者有复杂囊肿(2级,n = 5;2F级,n = 2;3级,n = 2)。大多数ACKD患者(89%)无症状。1名患者(5.5%)在开始KRT 72个月后出现背痛和肉眼血尿。另1名患者(5.5%)在开始KRT 60个月后出现感染性囊肿并伴有背痛和临床脓毒症。透析时间≥28个月是与ACKD发生相关的唯一显著因素(77.8%对40%;p = 0.028;比值比6.09,95%置信区间1.43 - 25.82,p = 0.014)。超声和MRI联合检查的诊断率优于单独超声检查。

结论

ACKD在肾衰竭儿童和青少年中很常见,大多数病例无症状,但可能出现严重并发症。透析时间延长与ACKD发生显著相关。因此,早期移植和积极的ACKD监测对接受KRT的儿童至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8a70/11947055/82998d3ad2e6/467_2024_6628_Figa_HTML.jpg

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