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Introduction <p>Multicystic dysplastic kidney disease (MCDK) is a notable congenital anomaly of the kidney and urinary tract, with potential risk for chronic kidney disease, yet data from sub-Saharan Africa remain scarce. This study examined the pattern of MCDK, associated contralateral kidney abnormalities, determined the predictors of MCDK involution and assessed short-term outcomes in children followed beyond one year in South Africa.</p> Method <p>This retrospective study involved children under 13 years of age with suspected unilateral MCDK, confirmed on kidney ultrasound and [<sup>99m</sup>Tc]Tc-MAG3 scans at the Red Cross War Memorial Children’s Hospital between January 1, 2014, and December 31, 2023. Demographic, clinical, and radiologic data were obtained. The Log-rank test and Cox Proportional Hazards regression analyses were used to identify predictors of MCDK involution.</p> Results <p>Among 1,581 new cases, 98 (6.2%) had unilateral MCDK. 50% were male, and 57.1% had left-sided involvement. Median follow-up was 60 months (IQR: 12–72). Contralateral kidney abnormalities occurred in 17 (17.3%), most commonly duplex kidney (35.3%) and ureteropelvic junction obstruction (29.4%), but no vesicoureteric reflux was noted. Of 81 children followed beyond a year, 80.2% demonstrated contralateral hypertrophy, and 69.1% exhibited involution of the affected kidney. Initial kidney size ≤ 5.0&#xa0;cm was the sole predictor of involution (Hazard Ratio: 2.42, 95% CI: 1.31–4.48). Urinary tract infections occurred in 18.5%, proteinuria in 2.5%, hypertension in 1.2%, and 2.5% developed chronic kidney disease related to contralateral dysplasia. One nephrectomy was performed, and no malignancies or deaths. At last follow-up, 28.4% were lost to follow-up, and 12.3% had transitioned to adolescent clinics.</p> Conclusion <p>All MCDK cases were unilateral, with duplex kidney being the most common contralateral abnormality, a distinctive finding previously unreported. Follow-up into adolescence may be beneficial, as progression of chronic kidney disease is rare in those without contralateral anomalies. Multicentre long-term studies are needed to provide standardised follow-up guidelines.</p>

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Paediatric multicystic dysplastic kidney disease in Cape Town, South Africa

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年11月29日

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  • Datonye Christopher Briggs,
  • Khanyisile Hlongwa,
  • Mignon McCulloch,
  • Peter Nourse,
  • Anita Brink,
  • Ashton Coetzee

摘要

Introduction

Multicystic dysplastic kidney disease (MCDK) is a notable congenital anomaly of the kidney and urinary tract, with potential risk for chronic kidney disease, yet data from sub-Saharan Africa remain scarce. This study examined the pattern of MCDK, associated contralateral kidney abnormalities, determined the predictors of MCDK involution and assessed short-term outcomes in children followed beyond one year in South Africa.

Method

This retrospective study involved children under 13 years of age with suspected unilateral MCDK, confirmed on kidney ultrasound and [99mTc]Tc-MAG3 scans at the Red Cross War Memorial Children’s Hospital between January 1, 2014, and December 31, 2023. Demographic, clinical, and radiologic data were obtained. The Log-rank test and Cox Proportional Hazards regression analyses were used to identify predictors of MCDK involution.

Results

Among 1,581 new cases, 98 (6.2%) had unilateral MCDK. 50% were male, and 57.1% had left-sided involvement. Median follow-up was 60 months (IQR: 12–72). Contralateral kidney abnormalities occurred in 17 (17.3%), most commonly duplex kidney (35.3%) and ureteropelvic junction obstruction (29.4%), but no vesicoureteric reflux was noted. Of 81 children followed beyond a year, 80.2% demonstrated contralateral hypertrophy, and 69.1% exhibited involution of the affected kidney. Initial kidney size ≤ 5.0 cm was the sole predictor of involution (Hazard Ratio: 2.42, 95% CI: 1.31–4.48). Urinary tract infections occurred in 18.5%, proteinuria in 2.5%, hypertension in 1.2%, and 2.5% developed chronic kidney disease related to contralateral dysplasia. One nephrectomy was performed, and no malignancies or deaths. At last follow-up, 28.4% were lost to follow-up, and 12.3% had transitioned to adolescent clinics.

Conclusion

All MCDK cases were unilateral, with duplex kidney being the most common contralateral abnormality, a distinctive finding previously unreported. Follow-up into adolescence may be beneficial, as progression of chronic kidney disease is rare in those without contralateral anomalies. Multicentre long-term studies are needed to provide standardised follow-up guidelines.