Clinical Characteristics and Outcomes of Children with Unilateral Multicystic Dysplastic Kidney: A Cohort Study.

IF 1.7 4区医学 Q2 PEDIATRICS

Indian pediatrics Pub Date : 2025-03-01 Epub Date: 2025-03-03 DOI:10.1007/s13312-025-00006-8

Gurram Vandana, Pediredla Karunakar, Sudarsan Krishnasamy, Ramachandran Ramge Sivakumar, Bobbity Deepthi, Bondada Hemanth Kumar, Sivamurukan Palanisamy, Ramesh Ananthakrishnan, Sriram Krishnamurthy

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引用次数: 0

Abstract

Objectives: To study the clinical profile and outcomes of children with unilateral multicystic dysplastic kidney (MCDK).

Methods: We assessed the clinical features and extrarenal manifestations in children with unilateral MCDK. These children were followed up to ascertain involution, compensatory hypertrophy and progression of chronic kidney disease (CKD) stage.

Results: We enrolled 106 children with unilateral MCDK which was detected antenatally in 98 (92.4%), while evaluating for urinary tract infection in three (2.8%), and incidentally in five (4.7%) children. Abnormalities in the contralateral kidney and extrarenal manifestations at initial presentation were detected in 30 (28.3%) and 15 (14.2%), respectively. At a median (IQR) follow-up of 60 (32, 87) months, 34 (32.1%) children demonstrated complete involution of the MCDK, while 72 (67.9%) showed compensatory hypertrophy in the contralateral kidney. The median age at involution of MCDK was 48.5 (33, 86.5) months. Twenty-two (20.7%) children had non-regression of MCDK, and two (1.9%) underwent nephrectomy. Eight (7.5%) children developed hypertension and two children were detected to have proteinuria. One child, each, progressed to CKD stage 2 and stage 3a; and another child (0.9%) progressed to end stage kidney disease. None of the patients developed malignant transformation.

Conclusions: Majority of cases (92.4%) of MCDK had been detected antenatally. The rate of involution was 32.1% at a median follow-up of 60 months. Although, 28.3% of cases of MCDK had abnormalities in the contralateral kidney, progression of CKD to a higher stage occurred only in three (2.8%) cases.

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单侧多囊性肾发育不良儿童的临床特点和预后：一项队列研究。

目的：探讨儿童单侧多囊性发育不良肾（MCDK）的临床特点及预后。方法：对单侧MCDK患儿的临床特点及外表现进行评价。对这些儿童进行随访，以确定慢性肾脏疾病（CKD）的复发、代偿性肥厚和进展。结果：我们纳入106例单侧MCDK患儿，其中98例（92.4%）在产前检测到，同时评估3例（2.8%）患儿的尿路感染，5例（4.7%）患儿的尿路感染。30例（28.3%）和15例（14.2%）患者在首发时发现对侧肾脏异常和肾外表现。在60（32,87）个月的中位（IQR）随访中，34（32.1%）名儿童表现为MCDK完全消退，而72（67.9%）名儿童表现为对侧肾脏代偿性肥大。MCDK复发时的中位年龄为48.5（33,86.5）个月。22例（20.7%）患儿MCDK未消退，2例（1.9%）行肾切除术。8名（7.5%）儿童出现高血压，2名儿童检出蛋白尿。各有一名儿童进展为CKD 2期和3a期；另一名儿童（0.9%）进展为终末期肾病。所有患者均未发生恶性转化。结论：绝大多数MCDK（92.4%）是产前检出的。中位随访60个月，复发率为32.1%。尽管28.3%的MCDK患者在对侧肾脏出现异常，但CKD进展到更高阶段的病例仅发生在3例（2.8%）。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Indian pediatrics 医学-小儿科

CiteScore

3.30

自引率

8.70%

发文量

344

审稿时长

3-8 weeks

期刊介绍： The general objective of Indian Pediatrics is "To promote the science and practice of Pediatrics." An important guiding principle has been the simultaneous need to inform, educate and entertain the target audience. The specific key objectives are: -To publish original, relevant, well researched peer reviewed articles on issues related to child health. -To provide continuing education to support informed clinical decisions and research. -To foster responsible and balanced debate on controversial issues that affect child health, including non-clinical areas such as medical education, ethics, law, environment and economics. -To achieve the highest level of ethical medical journalism and to produce a publication that is timely, credible and enjoyable to read.