Platelet and factor VIII (F-VIII) storage and phagocytic functions of the spleen were studied in 15 patients with β-thalassemia major who were not splenectomized and in 7 patients with Hb-S-b-thalassemia. Eight splenectomized patients, 4 patients with b-thalassemia major, and 11 healthy children served as controls. F-VIII elevation following adrenalin was not found to be a sensitive index in th...

A pedigree was studied in which five individuals with beta-thalassemia minor were found to have nontransfusional hemochromatosis. Three were children under the age of 10 and two were young male adults, ages 28 and 33. A 5-yr-old child without evidence of thalassemia also had hemochromatosis. Since hemochromatosis is transmitted as an HLA-linked autosomal recessive disorder, HLA haplotypes serve...

Thalassemia is the most common form of all inherited disorders of the red cell. It is estimated that 70 000 children are bornwith various forms of thalassemia each year, andmore than half of these births are affected by severe formsofb-thalassemia, ofwhich themost common subgroup is hemoglobin (Hb) E b-thalassemia. Thalassemia was originally confined to the tropical and subtropical regions of t...

Sickle cell disease (SCD) is a genetic disorder in which emoglobin S (Hb S) predominates in red blood cells. It is onsidered a significant public health issue in Brazil.1–3 Sickle cell anemia (SCA, Hb SS) is the most common subype of SCD in the world. Although its clinical course is ariable, patients with SCA generally have the most severe henotype. SCD also includes the heterozygous combinatio...

-Thalassemia is one of the major genetic disorders which can be transmitted from parents to their children. In India, there is a concept of consanguineous marriage apart from having various castes and sub-castes in all the states. There are many ways of screening -Thalassemia. Complete hemogram like MCV, MCH, Hb values and the NESTROFT. The sample which shows positive for NESTROFT can be chec...

BACKGROUND Thalassemia is the most common monogenic disease in South-East of Iran. Despite the 70% reduction in Iranian thalassemia cases after thalassemia control comprehensive program, 601 affected babies were born in Sistan and Balouchistan Province, Iran from 2002 to 2010, so this study aims at investigating the causes of new thalassemia cases. METHODS Data from this retrospective cross-s...

To explore the knowledge of parents thalassemic children, their attitude towards prevention birth children and practices followed by them in relation to treatment adherence. : Parents 100 patients Thalassemia, who were coming regularly for blood transfusion Thalassemia day care centre at Rajindra Hospital, Patiala from January 2011 Nov 2012, interviewed regarding about inheritance types prenata...

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...

Background Beta-thalassemia is the most common hematology disease in human and leptin is one of the hormone that produce by adiposities cells. The purpose of this study was to investigate the relationship between serum leptin level and thyroid hormones in children with major beta-thalassemia. Materials and Methods This descriptive-cross sectional study was performed on 90 children aged 6-16...