Renal malformations occur in 33%-70% of cases of Turner syndrome (chromosome 45 and variants). We describe two cases of multicystic dysplastic kidney in Turner syndrome. A literature review of renal abnormalities in Turner syndrome shows the frequency of cystic disease to be 1.76%. In multicystic dysplastic kidney, diagnostic investigation of the contralateral kidney, including voiding cystourethrography, is necessary in view of the high incidence of associated diseases (15%-20% of cases, vesicoureteric reflux) and other anomalies.
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