Neonatal Aspects Of Hereditary Spherocytosis.

Abstract

Hereditary spherocytosis is a common inherited non-1mmune haemolytic anaemia in the newly born infant. The clinical manifestations are : anaemia , jaundice, splenomegaly, and haemolytic and aplastic crisis associated to viral infections. Choledocholithiasis is a manifestation of the disease which can be seen at an early stage. Because some patients, even those who later manifest either mild or moderate forms of the diseases, can present with a very severe phenotype in early infancy, a reliable strategy for diagnosis of hereditary spherocytosis in the neonates hove not b;':en firmly established. Some pa[ient manifest clinical signs during the neonatal period and require transfusion. The use of rhEPO during the first month may be an alternative therapy to red cell transfusion. The natural history of the Hereditary spherocytosis has received little attention. Careful monitoring and follow up ofneonates with hereditary spherocytosis who have a severe phenotype is important for appropriate clinical management.