Abstract

The blood biochemistry of 84 young homozygous sickle cell patients aged 1 to 11 years was analysed for evidence of liver disease. A comparison of the values found in their blood with those seen in normal children from an identical age group, assessed at the same time, shows no convincing evidence of liver cell damage except in 12% of cases. The normal transaminase observed in many of the patients assessed, together with the high alkaline phosphatase activity which seemed to be out of proportion to plasma bilirubin, is a picture compatible with localised obstructive lesions of the liver or bone lesions, both of which are common in sickle cell disease. This biochemical pattern suggests that the conjugated bilirubin, which dominates the picture in 40% of patients who have 'haemolytic jaundice', is due largely, not to liver cell damage, but to a combination of two factors, namely, intrahepatic cholestasis and the presence of actively functioning liver cells. Adequate albumin synthesis found in these patients, together with normal thymol reactions, provides further evidence of the absence of severe liver cell damage.