先天性偏側性および両側性多嚢腎の同胞例

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  • センテンセイ ヘンソクセイ オヨビ リョウソクセイ タ ノウ ジン ノ ドウホ

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The cases of siblings with congenital multicystic kidney were reported. The elder is a 25 days old male infant and the younger is a 9 days of female infant. Affected side was left in the former and bilateral in the latter. Affected kidneys were removed in both cases. Female patient with bilateral involvement is doing well five years after the operation as the functioning area were remained at the upper portion of left kidney. Though congenital unilateral multicystic kedney is well defined group of unilateral, not hereditary, relatively rare disease, bilaterality or other pathology of the contralateral side were reported in the recent years. 86 cases of unilateral and a case of bilateral type were collected from Japanese literature during 1960 through 1982. Our cases are the first reported cases of occurrence in siblings and the female case is the second reported case of bilateral involvement in Japan. The nomenclature, congenital unilateral multicystic kidney became inadequate since clinical and pathological evidence are accurnlated, and this condition are now named multicystic dysplasia of the kidney or multicystic dysplastic kidney. Therefore, further investigation of this disease will be required under the new concept that this disease is dysplasia of the kidney.

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