Acquired cystic kidney disease in children with kidney failure.

IF 2.6 3区 医学 Q1 PEDIATRICS
Pediatric Nephrology Pub Date : 2025-05-01 Epub Date: 2025-01-04 DOI:10.1007/s00467-024-06628-7
Justin Ming-Yin Ma, Kin-Fen Kevin Fung, Pak-Chiu Tong, Wai-Ming Lai, Alison Lap-Tak Ma, Eugene Yu-Hin Chan
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引用次数: 0

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; ORadj 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.

肾衰竭儿童的获得性囊性肾病
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、影响因素和临床结局。方法:我们在香港指定的儿童肾脏学中心进行了一项横断面的区域性研究。ACKD被定义为原生肾脏存在≥3个囊肿,不包括先天性或遗传性囊性疾病。在2023年6月至12月期间,所有在香港接受KRT治疗的儿科患者都接受了超声检查、非对比磁共振成像(MRI)检查,或两者兼而有之。对复杂囊肿的患者进行对比增强计算机断层扫描。结果:43例儿童(56%为女性;中位年龄14.7岁;IQR, 11.7-18.7)纳入分析。18例患儿(42%)发现ACKD。9例患者有复杂囊肿(2级,n = 5;2F级,n = 2;3年级,n = 2)。大多数ACKD患者(89%)无症状。1例患者(5.5%)在KRT开始72个月后出现背部疼痛和肉眼血尿。另一名患者(5.5%)在KRT开始后60个月出现了感染囊肿并伴有背部疼痛和临床败血症。透析持续时间≥28个月是与ACKD发生相关的唯一显著因素(77.8% vs. 40%;p = 0.028;ORadj 6.09, 95% CI 1.43 ~ 25.82, p = 0.014)。超声联合MRI的诊断率优于单纯超声。结论:ACKD在肾衰竭的儿童和青少年中普遍存在,大多数病例无症状,但可能出现严重的并发症。较长的透析时间与ACKD的发生显著相关。因此,早期移植和积极的ACKD监测对于接受KRT的儿童至关重要。
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来源期刊
Pediatric Nephrology
Pediatric Nephrology 医学-泌尿学与肾脏学
CiteScore
4.70
自引率
20.00%
发文量
465
审稿时长
1 months
期刊介绍: International Pediatric Nephrology Association Pediatric Nephrology publishes original clinical research related to acute and chronic diseases that affect renal function, blood pressure, and fluid and electrolyte disorders in children. Studies may involve medical, surgical, nutritional, physiologic, biochemical, genetic, pathologic or immunologic aspects of disease, imaging techniques or consequences of acute or chronic kidney disease. There are 12 issues per year that contain Editorial Commentaries, Reviews, Educational Reviews, Original Articles, Brief Reports, Rapid Communications, Clinical Quizzes, and Letters to the Editors.
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