@entity2395 in southeast Asia: determination of different degrees of severity of anemia in XXXX .

@entity7723 @entity1 @entity224 @entity2395
beta (0)- @entity2395 /Hb E in Southeast Asia varies greatly in severity, with hemoglobin levels ranging from 2.5 to 13.5 g/dl, averaging 7.7 g/dl. Results of systematic investigations to find out what determines different levels of severity are reviewed. Concomitant inheritance of @entity7723 significantly decreases the severity. Different degrees of severity in the majority of cases, however, is not due to @entity7723 . Concordance of hemoglobin levels among @entity1 who are sibs prevails, suggesting polygenic factor determinants. Potential factors ruled out as determinants for different levels of severity are discriminating fetal hemoglobin production, erythrocyte @entity224 dismutase activity, reticulo-endothelial function, and failure of erythropoiesis compensation. Red cell survival and globin synthesis studies indicate that different degrees of excess of alpha-chains leading to different red cell pathology and survival are responsible for variable severity. Degrees of excess of alpha-chains in this circumstance are probably mainly determined by erythrocyte proteolytic activity. The relationship between the hemoglobin levels and erythrocyte cytosol proteolytic activity in 15 beta(0) - @entity2395 /Hb E disease @entity1 in whom a deletional type of @entity7723 had been ruled out by DNA mapping is striking, with a correlation coefficient of 0.78. This finding suggests that modulation of erythrocyte proteolysis is another approach for treatment of @entity2395 .
