PROGNOSTIC VALUE OF SERUM HYALURONIC-ACID AND TYPE-I AND TYPE-III PROCOLLAGEN PROPEPTIDES IN EXTRAHEPATIC BILIARY ATRESIA

Although portoenterostomy has greatly improved the prognosis of extrah epatic biliary atresia (EHBA), 10-20% of patients still die before 5 y of age, and the only treatment option is liver transplantation (LT). To investigate whether these patients may be identified at an early st age, when the chances of successful LT are optimal, we have measured s erum concentrations of hyaluronic acid (HA), the amino-terminal propep tide of type III procollagen (PIIINP) and the carboxy-terminal and ami no-terminal propeptides of type I procollagen (PICP, PINP) in 24 selec ted patients with EHBA, both before portoenterostomy and then every 6 mo until death (n = 10, age at death = 7-20 mo), LT (n = 6, age at LT = 1.1-4.8 y) or 5 y of age (n = 8). Raised serum HA above 200 mu g/L b efore portoenterostomy identified those patients who would die or requ ire LT in the first 5 y of life with a positive predictive value of 88 %; after portoenterostomy, longitudinal changes in HA reflected clinic al status in each patient. None of the other three markers was of prog nostic value, and only PIIINP showed any relationship with clinical st atus, and then only up to 1.5 y. Interestingly, PINP (but not PICP) te nded to be low in all patients before portoenterostomy and may reflect impaired bone collagen metabolism during early skeletal changes in EH BA. This study therefore suggests that measurement of serum HA may be a useful complementary test in EHBA, particularly in identifying, at a n early stage, those patients who should be considered for LT.