During the 5 years period from Jan. 1, 1974 to Dec. 31, 1978, we experienced seven
cases of congenital hyperbilirubinemia diagnosed by the clinical, laboratory, and
histopathological findings. Of the nine cases, four cases were Dubin-Johnson syndrome,
two cases Rotor's syndromes, and one case Gilbert's disease. The four Dubin-Johnson
syndroms occurred in one family, and the jaundice developed intermittently and remitted
spontaneously in all four cases.
Among the four cases, two cases revealed conjugated hyperbilirubinemia, and one case
unconjugated hyperbilirubinemia. Remaining one case had a history of chronic,
intermittent jaundice. The specimens of the two cases were obtained by needle biopsy,
and these specimens were grossly tan brown to black. Microscopically, the basic liver
architecture was preserved. Abundant brown pigments were found in the liver cells of
the centrolobular area, and often in kupffer cells also. With Fontana-Masson stain, these
pigments stained black, whereas Prussian blue reactions was negative. The Rotor's
syndromes of two cases without family history had a chronic intermittent jaundice with
raised conjugated bilirubin in the plasma. In one case, BSP retention was 51%/45min.
After the injection, and there was no secondary rise of BSP retention on 90min.(15%).
In the microscopical findings of liver biopsies, lobular architecture was preserved. There
was a normal morphology of liver cells without pigments, inflammation and fibrosis.
However, in one case, few ,fat vacuoles are found. The case of Gilbert's disease
manifested icteric sclera and skin without any discomfort.
Liver function tests and liver scan were normal except for the raised plasma
unconjugated bilirubin concentration. In the microscopical findings, lobular architecture
and morphology of the liver cells were normal. Pigments, inflammation and fibrosis were
not found.
|