Linked Life Data

3893274

Source:http://linkedlifedata.com/resource/pubmed/id/3893274

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:dateCreated
1985-8-12
pubmed:abstractText
beta (0)-Thalassemia/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 alpha-thalassemia significantly decreases the severity. Different degrees of severity in the majority of cases, however, is not due to alpha-thalassemia. Concordance of hemoglobin levels among patients 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 superoxide 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) -thalassemia/Hb E disease patients in whom a deletional type of alpha-thalassemia 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 thalassemia.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
0077-8923
pubmed:author
pubmed:issnType
Print
pubmed:volume
445
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
119-26
pubmed:dateRevised
2007-11-15
pubmed:meshHeading
pubmed:year
1985
pubmed:articleTitle
Thalassemia in southeast Asia: determination of different degrees of severity of anemia in thalassemia.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S.