Disorders of bilirubin metabolism

This chapter provides a brief summary of bilirubin metabolism and its inherited disorders, with emphasis on some significant developments. Serum bilirubin levels were found to be inversely related to the risk of obesity and metabolic syndrome and ischemic coronary artery disease in middle-aged men. Unconjugated bilirubin binds more tightly to albumin than conjugated bilirubin. Neonatal hyperbilirubinemia results from a combination of increased bilirubin production and lower hepatic bilirubin excretory capacity. Although clinicians are generally concerned with serum bilirubin levels as an indicator of liver function and disease, and the toxicity of bilirubin, within a near-physiological range of plasma concentrations, the antioxidative action of bilirubin may provide beneficial effects. Liver biopsy shows noninflammatory intrahepatic cholestasis without fibrosis, notwithstanding the number and severity of the attacks. The progressive familial intrahepatic cholestasis syndro.