[Congenital tumor-like histiocytosis of the liver with cholestasis in a newborn infant].
A female newborn weighing 2650 g had hepatomegaly and deepening obstructive jaundice. She died of hepatic failure three weeks later. There were grayish white periportal histiocytic nodules of 1-2 mm in diameter. Histiocytes contained a small amount of neutral lipids and PAS-positive material. Single elements had multiple nuclei arranged in circles like in Touton cells. Bile duct and ductules were among them. The picture resembled the C variant of Niemann-Pick's disease but basic histochemical tests on fixed tissue excluded this possibility. The pathogenesis of unique disease remained obscure.
D002779:Cholestasis / Q000150:complications*; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000150:complications*; D008113:Liver Neoplasms / Q000150:complications / Q000151:congenital*; D008206:Lymphatic Diseases / Q000150:complications / Q000151:congenital*
Vrozená tumoriformní histiocytôza jater s cholestázou u novorozence.