Hepatic fibrosis and survival in biliary atresia

The extent of biliary proliferation in liver biopsies from patients with biliary atresia at portoenterostomy is associated with the postoperative prognosis

“…Such measures may be more reflective of liver disease severity than the absolute amount of collagen per se [40]. Previously, we were not able to find an association between Kasai postoperative prognosis and the collagen surface density [7]; and in this study, fibrosis extent evaluated by a BA specific score [11] remained nonassociated with 1-year native liver survival. Wildhaber et al [15] described the influence of a "bridging fibrosis" factor on postoperative outcome, observing that variable in only 49% of their patients.…”

“…The extent of fibrosis is probably one such influencing factor. However, its assessment as a method for BA prognostic determination has become controversial [11,15,35,36]. The presence of cirrhosis in the liver periphery does not imply surgical failure because regenerative hilar nodules containing patent bile ducts can sustain an adequate bile flow [37].…”

“…Wildhaber et al [15] described the influence of a "bridging fibrosis" factor on postoperative outcome, observing that variable in only 49% of their patients. The lowest fibrosis score (FS1) from the classification used in this study [11], however, included "bridging fibrosis involving b50% of portal tracts." In our study group, 39 of 47 (82.98%) presented bridging fibrosis involving more than 50% of the portal spaces, with or without cirrhosis; and 30 of 47 (63.83%) presented "bridging fibrosis with nodular architecture" (FS3), that is, cirrhosis.…”

“…Fibrosis extent in the biopsies was evaluated according to the fibrosis score (FS) for BA developed by Weerasooriya et al [11], in which fibrosis was defined as follows: mild (FS1), fibrosis ranging from portal fibrous expansion to bridging fibrosis involving less than 50% of portal tracts; moderate (FS2), bridging fibrosis with more than 50% of portal tracts involved without nodular architecture; and severe (FS3), bridging fibrosis with more than 50% of portal tracts involved and nodular architecture.…”

Special considerations in children

“…A combination of genetic and environmental factors has been proposed to explain it, giving rise to a particularly intense fibrogenesis driven by an ongoing immune activation that may persist even after surgical restoration of bile flow [5,6]. After the Kasai portoenterostomy >50% of patients will clear jaundice if surgery is performed before 60 days of age but, unfortunately, almost all will already show an advanced stage of fibrosis or cirrhosis by that time (Figure 28.2) [7].…”