ORIGINAL ARTICLE Prasert Sathienkijakarnchai ® Voranush Chongsrisawat Jongrak Charoensapaya ® Taveesin Tanprayoon Bidhya Chandrakamol ® Pongspeera Suwangool Yong Poovorawan The relationship between procollagen-III-peptide and the severity of esophageal varices in children with biliary atresia after Kasai operation Accepted: 8 January 2001 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. Chil- dren below 15 years of age (n ¼ 29) with BA after a Kasai operation were evaluated for EV by endoscopy. Healthy (n ¼ 26) children of the same age and sex dis- tribution who participated in the hepatitis B vaccination program served as the controls. Serum PIIIP was de- termined by radioimmunoassay. The BA patients were classified on the basis of severity of EV (Paquet’s clas- sification) into three groups: group 1 (n ¼ 15) had grade 0, group 2 (n ¼ 8) grade 1–2, and group 3 (n ¼ 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 in- creased with severity of the EV in the BA patients. Hence, high PIIIP levels may serve as a non invas- tive indicator of EV developing in postoperative BA patients. Keywords Procollagen-III-peptide ® Esophageal varices ® Biliary atresia ® Kasai operation Introduction Biliary atresia (BA) represents a biliary-tract anomaly that destroys the liver parenchyma, causing cirrhosis and subsequent death unless corrective surgery is performed. Postnatal biliary-tract inflammation has been assumed to be associated with reovirus 3 [1, 2], rotavirus group C infection [3], a malformed bile duct [4] or biliary-tract ischemia [5] during the neonatal period. Without timely corrective surgery, fibrogenesis at the portal tracts in- creases, culminating in cirrhosis. It has been observed that even after successful Kasai operation (improved symptoms and regression of jaundice), most patients develop complications such as portal hypertension (PH) and esophageal varices (EV) [6]. It has been established that liver fibrosis (HF) is caused by alcohol, chronic viral infection, or metabolic liver disease. An increase of extracellular matrix protein within the perisinusoidal and periportal space between hepatocytes and matrix containing non-parenchymal cells leads to fibrosis [7, 8]. Thus, activated hepatic stellate cells enter a transitional stage and subsequently turn into myofibroblast-like cells, which play an essen- tial role in fibrogenesis. In the course of fibrogenesis, the levels of collagens type I (C-I) and III (C-III) rise [9]. In normal circumstances the collagen level remains con- stant except during active fibrosis, when C-III undergoes a marked increase, particularly during the initial phase [10]. Collagen itself is insoluble, but its precursor, pro- collagen, comprises soluble carboxyl and amino groups that can be detected in the blood. Fibrogenesis eventually causes HF and PH; the pa- tient presents with EV and variceal hemorrhage. Various investigators have attempted to correlate the serum markers for HF, including collagen type IV [11], laminin [12], endothelin [13], and hyaluronic acid [9], with the degree of severity in children with BA. There have been reports comparing this complex of macromolecules with other chronic liver diseases [14]. Without surgery, more than 90% of BA patients die before the age of 2 years Pediatr Surg Int (2002) 18: 32–35 Ó Springer-Verlag 2002 P. Sathienkijakarnchai ® V. Chongsrisawat ® J. Charoensapaya Y. Poovorawan (&) Viral Hepatitis Research Unit, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University and Hospital, Bangkok 10330, Thailand T. Tanprayoon ® B. Chandrakamol Department of Surgery, Chulalongkorn University and Hospital, Bangkok, Thailand P. Suwangool Department of Pathology, Chulalongkorn University and Hospital, Bangkok, Thailand