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J Pediatr Surg. 1994 May;29(5):639-44.

Immunohistochemical demonstration of collagen types III and IV and myofibroblasts in the liver of patients with biliary atresia.

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Second Department of Surgery, Kyoto University, Faculty of Medicine, Japan.


To clarify the pathogenesis of pericholangitis in biliary atresia, immunohistochemical and ultrastructural methods were used to quantify the amount of collagen types III and IV, and alpha-smooth muscle actin-positive myofibroblast present in the pericholangial tissue. Patients were divided into two groups: the early-stage group (n = 7; 15 to 30 days old at the time of the Kasai operation), and the advanced-stage group (n = 14; 8 months to 6 years old at the time of liver transplantation). The liver specimen was harvested at the time of the Kasai operation in the early-stage group, and during liver transplantation in the advanced-stage group. Liver tissue from the donor was used as the control. The specimen was visually inspected and classified into three grades to express the extent of proliferation. An increase of collagen types III and IV, and myofibroblast in the pericholangial area was seen even in 2- to 4-week-old patients, which became marked with the progression of the fibrotic disease process. This suggests that pericholangitis occurs in the very early stage of biliary atresia, perhaps before the development of bile duct obstruction.

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