Beta-thalassemia intermedia (β-TI) is a genetic variant of beta-thalassemias with a clinical disorder whose severity falls between thalassemia minor and thalassemia major. Different genetic defects are involved in this disorder and, based on severity of disease, clinical complications like skeletal deformities and growth retardation, splenomegaly, extramedullary hematopoiesis, heart failure, an...

IN PREVIOUS CO? -IMUNICATIONS’-3 the hematologic effects of the gene for an abnormal hemoglobin, since designated as hemoglobin C, were described in two combinations: A-C, an asymptomatic carrier state in which the hemoglobin consists of a mixture of A (normal adult type) hemoglobin amid C; secondly S-C, a variant of sickle cell disease in which sickle hemoglobin S is produced together with hem...

Background: Thalassemia syndromes are the commonest genetic disorders of blood and constitute a vast public health problem with 2.78 to 4% prevalence in India. The most effective feasible approach reduce incidence thalassemia major is implementation carrier screening program screen antenatal women early pregnancy. Methods: Institutional based cross sectional observational study was conducted. W...

OBJECTIVE The two most frequent hypochromic microcytic anemias are β- thalassemia minor (BTM) and iron deficiency anemia (IDA). Several discrimination indices have been proposed to quickly discriminate these similar entities via parameters obtained from automated blood count analyzers. The aim of this study to evaluate the diagnostic reliability of ten discrimination indices in the differentiat...

Structural hemoglobin (Hb) variants typically are based on a point mutation in a globin gene that produce a single amino acid substitution in a globin chain. Although most are of limited clinical significance, a few important subtypes have been identified with some frequency. Homozygous Hb C and Hb S (sickle cell disease) produce significant clinical manifestations, whereas Hb E and Hb D homozy...

congenital hepatic fibrosis (chf) is a developmental disorder of the biliary system, characterized by defective remodeling of the ductal plate. herein a family of three children, from consanguineous parents, with minor thalassemia is presented who suffered from congenital hepatic fibrosis (chf). prompt diagnosis and appropriate treatment are necessary to avoid further complications in the affec...