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隐性遗传性多囊肾:肝纤维化


这不是肾脏,隐性遗传性多囊肾还伴有先天性肝纤维化。图示,门管区扩张,周围的胆管呈放射状排列。肝实质内的许多色暗的成串的细胞是髓质外的岛状肝细胞增生。这是胎儿肝脏的典型表现。
You're right, this isn't kidney, but remember that recessive polycystic kidney disease also manifests with congenital hepatic fibrosis, as seen here in which a portal area is expanded with increased bile ducts radially arranged around the perimeter. The many dark clusters of cells in the hepatic parenchyma are islands of extramedullary hematopoiesis typical for fetal liver.

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