Hereditary spherocytosin in children – diagnosis and treatment
Journal Title: Postępy Nauk Medycznych - Year 2013, Vol 26, Issue 9
Abstract
Congenital hemolytic anemia are group of diseases characterized by shortening of the survival time of red blood cells. In clinical picture various degrees of anemia, jaundice and splenectomy are observed. One of them is hereditary spherocytosis.The main aim was to demonstrate the clinical application of new diagnostic methods, and to prove their usefulness in determining the correct diagnosis. The study group consisted 150 children with jaundice, various degrees of anemia and splenomegaly. These disturbances could have been caused by the most frequent hemolytic anemia, namely congenital spherocytosis. The screening flow cytometry EMA test was performed. In spherocytosis the reduction in fluorescence is between 25% and 30%. In 15 infants who developed severe anemia erythropoietin treatment (500IU/kg/week) was instituted.Congenital spherocytosis was rocognaized in 79 children (52.7%), a reduction in the fluorescence of red cells with the EMA dye to 70.87%. In this group was 15 infants. During erythropoietin treatment these infants received an average dose of 22 injection and maintained clinically acceptable levels of red cells.Given that clinical course of sherocytosis may often be not characteristic, which is especially evident for infants, there are still cases of qualification for splenectomy. This review presented the laboratory tests and erythropoietin therapy in order to avoid the need for transfusion
Authors and Affiliations
Anna Adamowicz-Salach
Keywords
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