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Clinical Characteristics and Outcomes of Children with Unilateral Multicystic Dysplastic Kidney: A Cohort Study
Gurram Vandana1, Pediredla Karunakar1, Sudarsan Krishnasamy1
1Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, 605006, India.
Insights
Most unilateral multicystic dysplastic kidney (MCDK) cases are detected before birth. While some children experience involution or compensatory hypertrophy, progression to chronic kidney disease (CKD) is rare.
Area of Science:
- Pediatric Nephrology
- Urology
- Medical Genetics
Background:
- Unilateral multicystic dysplastic kidney (MCDK) is a congenital anomaly.
- Early detection and monitoring are crucial for pediatric kidney health.
Purpose of the Study:
- To investigate the clinical characteristics and outcomes of unilateral MCDK in children.
- To assess the rate of MCDK involution, compensatory hypertrophy, and chronic kidney disease (CKD) progression.
Main Methods:
- Retrospective assessment of clinical features and extrarenal manifestations in 106 children with unilateral MCDK.
- Follow-up evaluation for MCDK involution, contralateral kidney hypertrophy, and CKD staging.
Main Results:
- Antenatal detection in 92.4% of cases.
- 32.1% showed complete MCDK involution by a median follow-up of 60 months.
- Contralateral kidney abnormalities were present in 28.3%; CKD progression occurred in only 2.8%.
Conclusions:
- Unilateral MCDK is predominantly diagnosed prenatally.
- While involution and hypertrophy are common, significant CKD progression is infrequent.
- Malignant transformation was not observed in this cohort.
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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