Hepatic Ultrastructural Findings of Familial Hyperbilirubinemia Syndrome

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Park, Sung Hye; Chi, Je G.
Issue Date
Seoul National University College of Medicine
Seoul J Med 1994;35(4):239-253
Congenital/familial hyperbilirubinemiaGilbert syndromeCrigler-Najjar syndromeDubin-Johnson syndromeRotor syndromeUltrastructural study
Seven liver biopsies of congenital/familial hyperbilirubinemia were
studied ultrastructurally including 3 cases of Dubin-Johnson syndrome, 2 cases of
Rotor syndrome, one case of Gilbert syndrome and one case of type 2 Crigler-Najjar
syndrome. All five cases of Dubin-Johnson syndrome and Rotor's syndrome had
conjugated hyperbilirubinemia and both cases of Gilbert's syndrome and a CriglerNajjar
syndrome had unconjugated hyperbilirubinemia. In Gilbert's syndrome, the
microvilli of the sinusoidal membrane of hepatocytes showed decreased height and
number with collagen lay down in the sinusoidal spaces, Megamitochondria, mild
proliferation of smooth endoplasmic reticulum, and dilated rough endoplasmic
reticulum were also noted. Lipofuscin bodies were seen, but they were less numerous
than characteristic Dubin-Johnson bodies. In Crigler-Najjar syndrome, bile canalicular
and ductular cholestasis were noted both light microscopically and ultrastructurally.
Most bile canaliculi are filled with ovoid homogeneous electron dense
material (bile pigments). Widening of the intercellular spaces with increased number
of microvilli on the lateral surface of hepatocytes were present. All three cases of
Dubin-Johnson syndrome revealed characteristic abundant lysosomal bodies and
dilatation of bile canaliculi. These bodies were numerous and membrane bound in
round, oval or pleomorphic shapes with variable degrees of electron densities.
Dilated bile canaliculi showed expanded lumen with decreased number of microvilli.
In Gilbert and Rotor syndromes, the hepatocytes contained lipofuscin-like lysosomal
bodies. In both cases of Rotor's syndrome, we found reduced number of microvilli
along the sinusoidal side of hepatocyte, like Gilbert syndrome, immature bile
canaliculi and pleomorphic megamitochondria and lipofuscin-like lysosomal bodies.
We concluded that hepatocytic hyperbilirubinemia syndromes could be differentiated
by ultrastructural study along with clinicopathologic correlation.
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 35 No.4 (1994)
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