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PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
2009-3-27
pubmed:abstractText
Hemoglobin E (HbE) is one of the most prevalent beta-globin variant, which is widely distributed in Southeast Asia especially in Thailand. Homozygosity for this variant is common and may occur with iron deficiency. In order to study clinical and hematological phenotypes without the confounding effect of iron deficiency, investigations were carried out before and after iron supplementation for 2 months. The effect of G6PD deficiency and coinheritance of alpha-thalassemia in homozygous HbE were also studied. HbE homozygotes were clinically benign, never had been transfused and had no hepatosplenomegaly. Out of 76 HbE homozygotes, hematological parameters of 7 individuals with iron deficiency improved after iron supplementation. Hemoglobin analysis revealed that HbE was the main hemoglobin detected, but 12 subjects were found to have a substantial percentage of HbF, which might lead to misdiagnosis as HbE/beta-thalassemia. Both clinical and hematological phenotypes of simple homozygous HbE did not differ from those who also inherited alpha-thalassemia and/or G6PD deficiency. It is necessary to perform a comprehensive DNA analysis for alpha-thalassemia in cases of homozygous HbE when their partner is suspected of having alpha-thalassemia 1 gene.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Mar
pubmed:issn
0125-1562
pubmed:author
pubmed:issnType
Print
pubmed:volume
40
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
306-16
pubmed:meshHeading
pubmed:year
2009
pubmed:articleTitle
Clinical and hematological phenotype of homozygous hemoglobin E: revisit of a benign condition with hidden reproductive risk.
pubmed:affiliation
Hematology/Oncology Division, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
pubmed:publicationType
Journal Article