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婴儿肝肾多囊性疾病(IPCD)属常染色体隐性遗传性疾病。每4千名至1万4千名活产儿中可见一个。一个家庭内多胎患病极为罕见,芝加哥莱茵医院在40年11万名婴儿中只见一例,现将我院尸解一例报告如下。 男婴,9月,因发热、腹泻、粘液便入院。B超提示多囊肾、多囊肝。因中毒性休克死亡。患儿姐姐因多囊肾、多囊肝2岁死亡。尸解:双侧肾体积增大,表面呈多数大小不等的囊状突起。切面布满直径0.1～0.5厘米的园形椭园形囊腔,内充粉红色
Infantile liver and kidney polycystic disease (IPCD) is an autosomal recessive disease. One out of every 4,000 to 14,000 live births is visible. A polygamy in a family is extremely rare, with only one in 110,000 infants at the Rheinland Hospital in Chicago. A case of autopsy in our hospital is reported below. Baby boy, September, due to fever, diarrhea, mucus will be hospitalized. B-Tip polycystic kidney disease, polycystic liver. Died of toxic shock. Children with polycystic kidney disease, polycystic liver 2-year-old died. Autopsy: bilateral renal enlargement, the surface was the size of the majority of cystic processes. Section covered with a diameter of 0.1 to 0.5 cm park-shaped elliptical cysts filled with pink