β- and α-Thalassemia intermedia in Basra, Southern Iraq. 2013

Dhurgham A Abdulwahid, and Mea'ad K Hassan
Department of Pediatrics, Basra Maternity and Children Hospital , Barsa , Iraq.

Hemoglobinopathies are common in Iraq and β-thalassemia major (β-TM) is a leading health problem in Basra, Southern Iraq. However, β- and α-thalassemia intermedia (β- and α-TI) have not been so well studied. This is a descriptive study of 152 consecutive β- and α-TI patients registered at the Centre for Hereditary Blood Diseases (CHBD) in Basra, Southern Iraq from October 1 2010 through June 30 2012 including age at diagnosis, blood transfusions and complications. β-Thalassemia intermedia was found in 80 (52.6%) patients with a mean age at diagnosis of 7.10 ± 8.0 years. This was significantly different from that of Hb H (β4) disease (12.95 ± 14.8 years), p <0.05. Patients with β-TI received significantly more blood transfusions (3.39 ± 3.85)/year compared to those with Hb H disease (1.07 ± 1.39)/year, p <0.05. Short stature, extramedullary erythropoiesis, pulmonary hypertension and iron overload were significantly higher among patients with β-TI compared to those with Hb H disease, p <0.05. Iron overload is a significant risk factor for growth retardation among patients with Hb H and β-TI. While age is a significant risk factor for osteoporosis in both types of thalassemia intermedia. Both α- and β-thalassemia (α- and β-thal) have been reported in Basra. Although β-TI is associated with a more severe disease than α-TI, both are associated with considerable complications. Thus, genetic studies are needed to determine the types of mutation producing β-TI and the exact α-thal determinants producing Hb H disease as they are important in the prediction of the phenotype severity.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007493 Iraq A country bordering the Persian Gulf, between Iran and Kuwait. The capital is Baghdad. Republic of Iraq
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010641 Phenotype The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment. Phenotypes
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D003241 Consanguinity The magnitude of INBREEDING in humans. Inbreeding, Human,Consanguineous Marriage,Consanguinous Mating,Consanguineous Marriages,Consanguinities,Consanguinous Matings,Human Inbreeding,Human Inbreedings,Inbreedings, Human,Marriage, Consanguineous,Marriages, Consanguineous,Mating, Consanguinous,Matings, Consanguinous
D004909 Erythrocyte Indices ERYTHROCYTE size and HEMOGLOBIN content or concentration, usually derived from ERYTHROCYTE COUNT; BLOOD hemoglobin concentration; and HEMATOCRIT. The indices include the mean corpuscular volume (MCV), the mean corpuscular hemoglobin (MCH), and the mean corpuscular hemoglobin concentration (MCHC). Erythrocyte Hemoglobin, Mean Cell,Erythrocyte Size Determination,Erythrocyte Volume, Mean Cell,Hemoglobin, Erythrocyte, Mean Cell,Mean Corpuscular Volume,Red Cell Indices,Erythrocyte Diameter,Erythrocyte Index,Erythrocyte Indexes,Erythrocyte Thickness,Mean Cell Hemoglobin Concentration,Mean Cell Volume,Mean Corpuscular Hemoglobin,Mean Corpuscular Hemoglobulin Concentration,Red Cell Distribution Width,Red Cell Index,Red Cell Indexes,Cell Volumes, Mean,Corpuscular Volumes, Mean,Determination, Erythrocyte Size,Determinations, Erythrocyte Size,Diameter, Erythrocyte,Diameters, Erythrocyte,Erythrocyte Diameters,Erythrocyte Size Determinations,Hemoglobin, Mean Corpuscular,Hemoglobins, Mean Corpuscular,Index, Erythrocyte,Index, Red Cell,Indexes, Erythrocyte,Indexes, Red Cell,Indices, Erythrocyte,Indices, Red Cell,Mean Cell Volumes,Mean Corpuscular Hemoglobins,Mean Corpuscular Volumes,Size Determination, Erythrocyte,Size Determinations, Erythrocyte,Thickness, Erythrocyte,Volume, Mean Cell,Volume, Mean Corpuscular,Volumes, Mean Cell,Volumes, Mean Corpuscular
D005260 Female Females

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