Inborn errors of bilirubin metabolism

The disorders of bilirubin metabolism are, for the most part, completely innocuous. The single exception, Type 1 congenital nonhemolytic jaundice, while usually lethal, is extremely rare. Hence, this entire category of conditions can hardly be said to constitute a significant public health problem. Its importance lies in the fact that the study of these conditions has stimulated much of the current understanding of bilirubin metabolism and hepatic excretory function. Furthermore, the accurate diagnosis of individual cases is essential to avoid the unnecessary surgery and economic hardship which may otherwise afflict affected individuals.