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Segmental multicystic dysplastic kidney in children
Evelyne Kalyoussef1, Jonathan Hwang, Vinay Prasad
1Division of Urology, Robert Wood Johnson Medical School, New Brunswick, New Jersey 08903, USA.
Insights
Segmental multicystic dysplastic kidney, a rare condition, typically affects infants. This report details the first adolescent case, expanding understanding of pediatric kidney disease.
Area of Science:
- Pediatric Nephrology
- Urology
- Medical Genetics
Background:
- Multicystic dysplastic kidney (MCDK) disease is a congenital anomaly of the kidney and urinary tract.
- Segmental MCDK is an exceptionally rare subtype, with limited documented cases.
- Previous literature exclusively reported infantile presentations of segmental MCDK.
Observation:
- This study presents the first documented case of segmental MCDK in an adolescent female.
- The patient's clinical presentation and diagnostic findings are detailed.
- A comprehensive review of the 18 previously reported cases is included.
Findings:
- The adolescent presentation challenges the typical infantile diagnosis paradigm for segmental MCDK.
- This case expands the known age range for segmental MCDK.
- Analysis of published reports provides insights into the natural history and potential variations of the condition.
Implications:
- Highlights the importance of considering segmental MCDK in adolescents presenting with relevant symptoms.
- Contributes to a better understanding of the spectrum of pediatric kidney malformations.
- Informs diagnostic and management strategies for rare congenital kidney anomalies.
Abstract:
Pediatric segmental multicystic dysplastic kidney is a rare subtype of multicystic dysplastic kidney disease, with only 18 case reports published. All children previously reported with this condition presented during infancy. This is the first case of segmental multicystic dysplastic kidney in an adolescent girl. The case is discussed, along with a review of published reports.
