β- and α-Thalassemia intermedia in Basra, Southern 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.
Department of Pediatrics, Basra Maternity and Children Hospital , Barsa , Iraq.
Dhurgham A
DA
Abdulwahid
Mea'ad K
MK
Hassan
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003241:Consanguinity; D004909:Erythrocyte Indices; D005260:Female; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D007223:Infant; D007493:Iraq; D019190:Iron Overload / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D013156:Splenectomy; D055815:Young Adult; D017085:alpha-Thalassemia / Q000097:blood / Q000150:complications / Q000235:genetics* / Q000628:therapy; D017086:beta-Thalassemia / Q000097:blood / Q000150:complications / Q000235:genetics* / Q000628:therapy
D006454:Hemoglobins