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Pediatric Nephrology 2009-Feb

Management and etiology of the unilateral multicystic dysplastic kidney: a review.

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David S Hains
Carlton M Bates
Susan Ingraham
Andrew L Schwaderer

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In children, unilateral multicystic dysplastic kidney (MCDK) is one of the most frequently identified urinary tract abnormalities. A variety of proposed etiologies has been associated with the underlying pathogenesis of MCDK. These include genetic disturbances, teratogens, in utero infections, and urinary outflow tract obstruction. From 5-43% of the time, MCDK has associated genito-urinary anomalies, both structural and functional in nature. A review of the literature reveals that involution rates are reported to be 19-73%, compensatory hypertrophy of the contralateral kidney occurs from 24-81% of the time, and estimated glomerular filtration rates (GFRs) (by the Schwartz formula) range from 86-122 ml/min per 1.73 m(2) body surface area. Most authors suggest serial ultrasonography to monitor contralateral growth, routine blood pressure monitoring, and a serum creatinine monitoring algorithm. The risk of hypertension in those with MCDKs does not appear to be greater than that of the general population, and the rates of malignant transformation of MCDK are small, if at all increased, in comparison with those in the general population. If the patient develops a urinary tract infection or has abnormalities of the contralateral kidney, shown on ultrasound, a voiding cystourethrogram is recommended. Finally, the body of literature does not support the routine surgical removal of MCDKs.

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