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Kidney growth and renal function in unilateral multicystic dysplastic kidney disease
U John1, S Rudnik-Schöneborn, K Zerres
1Division of Paediatric Nephrology, University Children's Hospital, Jena, Germany.
Insights
Multicystic dysplastic kidney (MCDK) often involutes or shrinks over time. Contralateral kidney hypertrophy is common, and renal function generally remains normal, suggesting regular follow-up is preferred over nephrectomy.
Area of Science:
- Pediatric Nephrology
- Urology
- Developmental Biology
Background:
- The natural history and long-term outcomes of multicystic dysplastic kidney (MCDK) require further elucidation.
- Understanding the growth and function of the contralateral kidney in unilateral MCDK is crucial for patient management.
Purpose of the Study:
- To analyze kidney growth and renal function in children with prenatally diagnosed unilateral MCDK.
- To establish the long-term natural history of MCDK and the compensatory changes in the contralateral kidney.
Main Methods:
- Long-term observational study of 33 children with unilateral MCDK diagnosed prenatally.
- Analysis of kidney growth (volume and length), renal function (creatinine, creatinine clearance), and associated abnormalities.
- Mean observation period of 4.9 years (range 1-11.6 years).
Main Results:
- Complete involution of MCDK observed in 48%, with size decrease in 33%.
- Contralateral kidney hypertrophy was prevalent (27/29 children), starting in utero and persisting.
- Renal function remained largely normal, with creatinine clearance within normal limits for most patients.
Conclusions:
- Multicystic dysplastic kidney often undergoes spontaneous regression or size reduction.
- Compensatory contralateral kidney hypertrophy is a common and significant finding.
- Nephrectomy is not recommended for typical MCDK cases; regular monitoring is advised.
Abstract:
The natural history of multicystic dysplastic kidney (MCDK) is not well established. We analyzed kidney growth and renal function in 33 children with prenatally diagnosed unilateral MCDK in a long-term study. The mean observation period was 4.9 years with a range of 1-11.6 years. Abnormalities of the contralateral kidney were found in 10 of 33 patients (30%): ureteropelvic junction obstruction (5), ureterovesical junction obstruction (2), and vesicoureteral reflux (3). In 6 children the dysplastic kidney had been removed. Complete involution was observed in 48% and a decrease of size in 33% of 27 dysplastic kidneys. At the time of last examination, 27 of 29 children showed a volume of the contralateral kidney above the normal range (>145%). Hypertrophy of the contralateral kidney, defined as kidney length above 2 standard deviation scores (SDS), was seen in 24% of 33 children at birth, thus showing that hypertrophy of the contralateral kidney starts in utero and continues throughout childhood. The extent of contralateral hypertrophy was independent of associated abnormalities in this study. Mean creatinine was increased in the whole group (mean +1.13 SDS). Calculated creatinine clearance in 21 patients over 2 years was within normal limits, with a median of 102 ml/min per 1.73 m2 (range 84-143). Based on the results of this and previous studies, nephrectomy cannot be recommended in typical cases, but a regular follow-up of these patients seems necessary.