Distinguishing Features of Childhood Renal Dysplasia.

IF 1.2 4区 医学 Q3 PEDIATRICS Klinische Padiatrie Pub Date : 2024-09-20 DOI:10.1055/a-2373-0785
Ece Mekik, Zeynep Birsin Özçakar, Nilgun Cakar, Burcu Biral Coşkun, Beyza Doğanay, Fatos Yalcinkaya
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Abstract

Background: Renal dysplasia (RD) is a major cause of chronic kidney disease (CKD) in childhood. Herein, we present a single-center experience about distinctive features of different subtypes of dysplasia.

Methods: All children with RD admitted between January 2018 and June 2019 were included in this cross-sectional study. Patients were classified as having unilateral RD, bilateral RD, posterior urethral valves (PUV), and multicystic-dysplastic kidneys (MCDK).

Results: A total of 198 patients (108 unilateral RD, 31 bilateral RD, 16 PUV, and 43 MCDK) with a follow-up period of 80 (1.5-240) months were included. The overall consanguinity rate was 22%, whereas it was 56% in bilateral RD patients. Two-thirds had additional urogenital anomalies. Hypertension was detected in 16% of the patients, and 20% had median proteinuria of 16 (5.2-173) mg/m2/h, which was significantly higher in the PUV and bilateral RD groups. The overall rate of CKD was 23%, which was more frequent in bilateral RD and PUV groups. The median age at end-stage renal disease was 91.5 (0.5-208) months, including 12 renal transplant patients.

Conclusions: As different subtypes of RD have distinct clinical and prognostic features, the follow-up of RD patients should be individualized, and modifiable complications such as proteinuria and HT should be monitored closely.

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儿童肾发育不良的鉴别特征。
背景:肾发育不良(RD)是儿童慢性肾病(CKD)的主要病因。在此,我们就不同亚型发育不良的显著特征介绍了单中心的经验:这项横断面研究纳入了2018年1月至2019年6月期间收治的所有RD患儿。患者被分为单侧RD、双侧RD、后尿道瓣膜(PUV)和多囊性肾脏发育不良(MCDK):共纳入 198 例患者(108 例单侧 RD、31 例双侧 RD、16 例 PUV 和 43 例 MCDK),随访时间为 80(1.5-240)个月。总体近亲率为 22%,而双侧 RD 患者的近亲率为 56%。三分之二的患者有额外的泌尿生殖系统异常。16%的患者患有高血压,20%的患者蛋白尿中位数为16(5.2-173)毫克/平方米/小时,PUV组和双侧RD组的蛋白尿中位数明显更高。慢性肾功能衰竭的总发生率为 23%,在双侧 RD 组和 PUV 组中更为常见。终末期肾病的中位年龄为 91.5(0.5-208)个月,其中包括 12 名肾移植患者:结论:由于不同亚型的RD具有不同的临床和预后特征,因此对RD患者的随访应个体化,并应密切监测可改变的并发症,如蛋白尿和高密度脂蛋白血症。
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来源期刊
Klinische Padiatrie
Klinische Padiatrie 医学-小儿科
CiteScore
1.10
自引率
0.00%
发文量
135
审稿时长
6-12 weeks
期刊介绍: Das Forum für wissenschaftliche Information in der Kinderheilkunde ausgewählte Originalarbeiten aus allen Bereichen der Pädiatrie Visite: Ihr Forum für interessante Krankengeschichten und außergewöhnliche Kasuistiken aktuelle Fortschritte in Diagnostik und Therapie jährliche Schwerpunkthefte: Ergebnisse der pädiatrischen Onkologie plus Medizin und Markt topaktuelle Informationen aus der Industrie
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