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The Korean Journal of Pathology 1980;14(3): 37-42.
선천성 과빌리루빈혈증 7예에 대한 병리학적 검색
A Pathological Study on Seven Cases of the Congenital Hyperbilirubinemia
ABSTRACT
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 syndromes 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.