Carter C O
Birth Defects Orig Artic Ser. 1974;10(4):16-21.
Both the adult and the childhood type of polycystic disease of liver and kidney may present in childhood, but they may be clearly distinguished histologically. The childhood type appears to involve a similar disorder of kidney and liver and is always recessive. It may be subdivided into four subtypes of increasing severity-perinatal, neonatal, infantile and juvenile-which breed true within families. The first causes death in the neonatal period, while the last may permit survival into adult life.
成人型和儿童型肝肾多囊病均可在儿童期出现,但在组织学上可明确区分。儿童型似乎涉及肝肾类似的病症,且总是隐性遗传。它可细分为严重程度递增的四种亚型——围产期、新生儿期、婴儿期和青少年期——在家族中呈纯合子遗传。第一种导致新生儿期死亡,而最后一种可能使人存活至成年。