β Thalassemia major due to acquired uniparental disomy in a previously healthy adolescent.

Uniparental disomy: a novel mechanism for thalassemia major.

The Association between Dental Health Status and Oral Health-Related Quality of Life of Children Diagnosed with -Thalassemia Major in Zahedan City, Iran

Background: b-thalassemia major is a common hereditary blood disease that can affect patients’ oral health and quality of life. The present study aims to determine the relationship between health of deciduous teeth and the Oral Health-Related Quality of Life (OHRQoL) in children diagnosed with β-thalassemia major compared with healthy children. Materials and Methods This cross-sectional study w...

Evaluation of Dento-maxillofacial Changes in Pediatric ß-Thalassemia Major Patients in Northern Iran

Background Thalassemia is a group of congenital disorders which is characterized by a deficient synthesis of alfa or beta globulin chains in hemoglobin molecules. Due to the problems resulting from changes in face, especially in dento-maxillary regions, such patients require special attention regarding oral health. Thus in this study, we aimed to assess the dento-maxillofacial changes in Beta-t...

β-thalassemia major evolution from β-thalassemia minor is associated with paternal uniparental isodisomy of chromosome 11p15

β-thalassemia major can be caused by homozygosity or compound heterozygosity for β-globin gene mutations (HBB gene). Most cases are inherited from parents who both have diseased alleles of the HBB gene. We report a patient with late-onset β-thalassemia major that evolved from β-thalassemia minor in which only one of her parents had the diseased HBB gene. To study the cause of β-thalassemia majo...

Frequency of Celiac Disease in Children with Beta Thalassemia major

Background We aimed to investigate the frequency of celiac disease in children with β-thalassemia major (B-TM) in Shiraz, southern Iran. Materials and Methods In this study, the prevalence of celiac disease in children with B-TM was evaluated. Children with BTM were screened for celiac disease by ant-tissue transglutaminase (anti-tTG) IgA antibody, IgA level and anti-tTG IgG. A total o...