CONGENITAL INTRAHEPATIC BILIARY ATRESIA

Abstract

It seems that there are certain clinical and laboratory findings which may suggest the diagnosis of intrahepatic biliary atresia. This diagnosis should be considered when all incompletely obstructive, chronic, fluctuating jaundice is present in an infant who is otherwise healthy. Pronounced elevation of serum lipids, especially the neutral fat fraction, and dissociated changes in tests relative to biliary obstruction, should be regarded as further suggestive evidence. These features may be encountered as early as 2 to 3 months of age, as demonstrated in the present case.

There is not enough evidence at the present time to suggest that the above features, or a combination of them are specific for intrahepatic biliary atresia. Likewise, there is not enough evidence that the absence of these features excludes the diagnosis. The findings in the present case, and those in the previous reports on this condition, would rather serve to stress the importance of detailed investigation of infants suffering from an obstructive jaundice and the need for follow-up studies with tests repeated at regular intervals.

A tentative diagnosis of intrahepatic biliary atresia is probably justified when the above criteria are fulfilled, but a definitive diagnosis is dependent on examination of a representative biopsy specimen.