Homozygous beta thalassemia presenting as neonatal jaundice.

In hemolytic anaemia there is shorter than normal erythrocytic survival resulting in increased destruction of red blood cells (RBCs) which leads to raised bilirubin levels. Appearance of jaundice, however, depends not only Upon increased biirubin production but also on clearance mechanisms in the liver. These clearance mechanisms are not well developed in the neonates resulting in greater incidence of hemolysis related jaundice as compared with adults and older children. Anemias caused by the production of an abnormal type of haemoglobin or those that are a result of abnormal .haemoglobin synthesis, such as beta thalassaemia, rarely manifest themselves in the first week of life. At birth fetal hemoglobin (HbF) accounts for 50 to 60% of haemoglobin production. This declines to about 5% by the age of jhree months when most of it is replaced by adult type of hemoglobin containing two alpha and two beta globin chains. Gamma globin production is usually unimpaired in utero in individuals with severe beta thalassemia, thus, only when such newborns need to replace their fetal red cells with cells that contain predominantly haemoglobin A. does the defect in beta globin synthesis become apparent. We present a case of homozygous beta thalassemia which developed hyper bilirubinemia soon after birth and presented at the age of 3 months with persistent jaundice since birth.