Increased deposition and serum level of type IV collagen in patients with extrahepatic biliary atresia.
Increased deposition of fibrous connective tissue has been demonstrated in extrahepatic biliary atresia (EHBA). We have focused on type IV collagen, a major component of the perisinusoidal wall of the liver. We attempted to localize tyep IV collagen in liver specimens with monoclonal antibodies. Ultrastructural changes of the liver were observed by electronmicroscopy. Serum amount of type IV collagen was estimated by a recently developed sandwich enzyme immunoassay (EIA) method. Serum laminin and prolyl 4-hydroxylase were also determined by EIA for comparison with type IV collagen. Increased deposition of type IV collagen in the perisinusoidal wall was evident in the area with so-called sinusoidal capillarization. Electronmicroscopy showed a basement membrane structure underlying the endothelial cells in Disse's space. Serum type IV collagen increased in EHBA patients before living-related liver transplantation (LRLT), and returned to normal within 5 weeks after LRLT, except for some patients with postoperative complications. Serum laminin and prolyl 4-hydroxylase showed the same propensity, although less prominently so. It is suggested that the immunoassay of serum type IV collagen adopted in this study has potential application in estimating collagen synthetic activity of the liver and in monitoring the postoperative states of patients after LRLT.
Shirahase
I
I
Second Department of Surgery, Kyoto University, Faculty of Medicine, Japan.
Ooshima
A
A
Tanaka
K
K
Ozawa
K
K
Yamabe
H
H
Yamamoto
T
T
D001656:Biliary Atresia / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D003094:Collagen / Q000097:blood / Q000378:metabolism*; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D007223:Infant; D007668:Kidney / Q000503:physiopathology; D007797:Laminin / Q000097:blood; D008099:Liver / Q000737:chemistry* / Q000473:pathology / Q000503:physiopathology; D016031:Liver Transplantation; D008297:Male; D011393:Procollagen-Proline Dioxygenase / Q000097:blood
D007797:Laminin; D003094:Collagen; D011393:Procollagen-Proline Dioxygenase