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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
biochemistry, genetics and molecular biology
Molecular basis of Hb H disease in southwest Iran
Hemoglobin, Volume 29, No. 1, Year 2005
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Description
Although α-thalassemia (thal) defects are not very frequent in the Iranian population, Hb H disease does occur in the country. We have analyzed the α gene cluster of 13 patients showing the presence of Hb H to establish the molecular background of this disease in southwest Iran (Shiraz and Hormozgan provinces). Using gap-polymerase chain reaction (gap-PCR) and direct DNA sequencing we have found the --MED-I deletion, the polyadenylation signal (poly A) mutations αT-Saudiα and αT-Turkishα, and Hb Constant Spring (Hb CS) in association with the common -α3.7 deletion. This study has revealed that: 1) at least six genotypes are responsible for Hb H disease in the area: -α3.7/ --MED-I; -α3.7/ αT-Saudiα; αT-Saudiα/ αT-Saudiα; αCSα/-- MED-I; --MED-I/αT-Turkishα; and the atypical forms of Hb H disease -α3.7/α CSα. 2) The molecular background of Hb H disease in the southwest area of Iran is more similar to the Mediterranean type than to the Southeast Asian. 3) Hb Bart's hydrops fetalis syndrome and mild, intermediate or severe postnatal Hb H disease conditions can be expected, but at a relatively low incidence. 4) The diagnostic flowchart for patients with microcytic hypochromic anemia should include iron deficiency, β-thal, α+- and α0-thal analyses. Copyright © 2005 Taylor & Francis, Inc.
Authors & Co-Authors
Yavarian, Majid
Netherlands, Leiden
Leids Universitair Medisch Centrum
Karimi, Mehran A.
Iran, Shiraz
School of Medicine
Zoraï, Amine
Tunisia, Tunis
Institut Pasteur de Tunis
Harteveld, Cornelis Leonard
Netherlands, Leiden
Leids Universitair Medisch Centrum
Giordano, Piero Carlo
Netherlands, Leiden
Leids Universitair Medisch Centrum
Statistics
Citations: 5
Authors: 5
Affiliations: 3
Identifiers
Doi:
10.1081/HEM-200047019
ISSN:
03630269
Research Areas
Genetics And Genomics
Maternal And Child Health
Study Design
Cross Sectional Study
Cohort Study