There is considerable clinical evidence of hepatic involvement in sickle-cell anemia (SCA). Clinically, the liver is enlarged and at times tender in 40% of young children with SCA, and jaundice is present in about 50% of cases after 5 years of age.1 The total circulating plasma protein mass is increased in these patients.2 This places an extra strain on the liver as a source of production of serum albumin.
Despite clinical evidence of liver involvement in children with SCA, difficulty is usually encountered in quantitating hepatic dysfunction by the usual laboratory tests. This paper represents an attempt to obtain additional evidence of disturbance in liver metabolism in SCA.
The following tests were done: (a) traditional liver-function tests, (b) serum protein electrophoresis, (c) determination of the level of venous blood ammonia, (d) analysis of amino acid excretion in the urine, and (e) histological examination of liver tissue.A.