The relationship between procollagen-III-peptide and the severity of esophageal varices in children with biliary atresia after Kasai operation

Abstract

Biliary atresia (BA) is a common cause of infantile cholestasis. Disease progression leads to intra hepaticfibrosis, and thus to the development of PH and EV. Our objective has been to study the relationship between procollagen-III-peptide (PIIIP) and the severity of EV in children with BA after Kasai operation. Children below 15 years of age (n=29) with BA after a Kasai operation were evaluated for EV by endoscopy. Healthy (n=26) children of the same age and sex distribution who participated in the hepatitis B vaccination program served as the controls. Serum PIIIP was determined by radioimmunoassay. The BA patients were classified on the basis of severity of EV (Paquet's classification) into three groups: group 1 (n=15) had grade 0, group 2 (n=8) grade 1-2, and group 3 (n=6) grade 3-4 EV. In group 3, serum PIIIP (2.9 +/- 1.3 IU/ml) was significantly higher than in group 2 (1.5 +/- 0.4 IU/ml) (P < 0.05). Serum PIIIP levels were increased in group 2 compared with group 1 (1.2 +/- 0.4 IU/ml) and in group 1 compared with the control group (1.2 +/- 0.2 IU/ml), but this difference was not significant. PIIIP levels increased with severity of the EV in the BA patients. Hence, high PIIIP levels may serve as a non invastive indicator of EV developing in postoperative BA patients.

What's New in Biliary Atresia?

“…Other series subdivide patients with BA into groups with good, worse, and bad outcome, respectively. Herein c-kit, which is expressed from mast cells, reflects the degree of hepatic fibrosis as well the transforming growth factor-b-1 and procollagen-IIIpeptide (2,4,14,22,24,44). Plasmaendothelin-1, a potent vasoconstrictor, correlates with the severity of liver cirrhosis and portal hypertension, as well as intercellular (ICAM) and vascular cell adhesion molecules (VCAM) (11,14).…”