Liver Biopsies from Proband 1 Show Non-specific Findings Consistent with Liver Damage
(A) H&E shows moderate macrovesicular steatosis (200× magnification).
(B) Trichrome stain shows bridging fibrosis (100× magnification).
(C) Numerous lipid droplets are present in hepatocytes (HV = 80.0 kV; 2,100× direct magnification).
(D) Glycogen contents are in the normal range and there are no visible abnormalities in the endoplasmic reticulum (HV = 80.0 kV; 11,000× direct magnification).
(E) The bile canaliculus (center) is normal. Electron dense material consistent with biliary material is seen in the hepatocellular cytoplasm surrounding the bile canaliculus (HV = 80.0 kV; 11,000× direct magnification).
(F) There are no abnormalities of mitochondria and peroxisomes are present (HV = 80.0 kV; 21,000× direct magnification).