The liver is bile-stained and shows numerous nodules measuring up to 2 cm. across.
This child died at the age of 15 months; a previous liver biopsy had shown a cirrhosis.
At autopsy 1.5 l of bile-stained ascitic fluid was present in the abdomen; the liver weighed 415
grams (at this age the liver usually weighs between 300 and 350 grams). The biliary system was
patent. The histology of the lesion is that of a macronodular cirrhosis with broad collagenous bands
separating the nodules; there was giant cell transformation in some of the cells and there was
marked intra-canalicular bile plugging. Neither in this specimen nor in the biopsy was there any
evidence of any inborn error of metabolism; no a-1 anti-trypsin deficiency was detected and there
was no evidence of hepatitis B or cytomegalo virus infection. The appearance were those of a
macronodular cirrhosis of post-necrotic scarring type for which there was no definite aetiology.