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Clinical features of children with multicystic dysplastic kidney. BACKGROUND: To evaluate the clinical features of patients with multicystic dysplastic kidney (MCDK). METHODS: The medical files of children diagnosed with MCDK between January 2008 and November 2015 were retrospectively reviewed. The demographic, clinical, laboratory and radiological data were evaluated. RESULTS: Of 128 children with MCDK enrolled in the study, 82 (64.1%) were male, and 46 (35.9%) were female (P < 0.05). MCDK were located on left and right sides in 66 (51.6%) and 62 children (48.4%), respectively (P > 0.05). Antenatal diagnosis was present in 64 patients (50%). The mean age at diagnosis was 2.8 ± 2.7 years (range, 0-8 years), and follow-up duration was 4.5 years. Fifteen patients (20.8%) had vesicoureteral reflux. Of these, four underwent endoscopic surgical correction. Other associated urological anomalies were ureteropelvic junction obstruction (n = 6), hypospadias (n = 1), and kidney stones (n = 1). On technetium-99 m dimercaptosuccinic acid scintigraphy, which was performed in all patients, no significant association between grade of reflux and presence of scarring was seen. Hypertension was diagnosed only in one child (0.8%) who required antihypertensive treatment. The prevalence of unilateral undescended testicle in children aged <1 year in the 82 male patients was 4.9%. Seventy-six patients (59.4%) developed compensatory hypertrophy in the contralateral kidney during a 1 year follow-up period. Of the total, only seven children (5.5%) had undergone nephrectomy. CONCLUSIONS: MCDK follows a benign course with relatively few sequelae, and therefore these patients should be closely followed up and conservatively managed.