Autosomal dominant inheritance of multicystic dysplastic kidney.
Unilateral multicystic dysplastic kidney (MCDK) in a normal infant is believed to be a sporadic disorder, with an incidence of about 1 in 4,300 live births. Isolated unilateral MCDK occurring in a family without other genitourinary abnormalities has not been described. We report a family in which isolated unilateral MCDK occurred in a woman and her two children. The mother presented with a palpable abdominal mass during infancy, which on excision was found to be a MCDK. Both the children were found to have MCDK on prenatal ultrasonography, which was later confirmed on postnatal evaluation. The MCDK in the children continues to involute on follow-up urinary tract ultrasonography. The inheritance of MCDK appears to be autosomal dominant in this family.
Srivastava
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Section of Pediatric Nephrology, Children's Mercy Hospital, Kansas City, MO 64108, USA. tsrivastava@cmh.edu
Garola
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Hellerstein
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D002675:Child, Preschool; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D007223:Infant; D007668:Kidney / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D009392:Nephrectomy; D007690:Polycystic Kidney Diseases / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D014463:Ultrasonography