Unexpected Genotype in a Non-Transfusion Dependent Thalassemia Family
نویسندگان
چکیده
منابع مشابه
Bone density in transfusion dependent thalassemia patients in Urmia, Iran
Background Patients with thalassemia major and intermedia are susceptible to osteopenia and osteoporosis. The mechanism of osteoporosis in these patients is multifactorial. Transfusion related iron overload in endocrine organs leads to impaired growth hormone secretion, diabetes mellitus, hypothyroidism, hypoparathyroidism, lack of sex steroids and vitamin D deficiency that contribute t...
متن کاملPrevalence of Alloantibodies and Autoantibodies in Transfusion Dependent Thalassemia Patients
Background: The development of anti-red blood cell alloantibodies remains a major problem in transfusion of blood in thalassemia major patients. Also, Autoantibodies can result in clinical hemolysis and difficulty in cross-matching blood. We studied the frequency of red blood cell alloimmunization and autoimmunization among thalassemia patients who received regular transfusions in Ilam province...
متن کاملIron Overload and Chelation Therapy in Non-Transfusion Dependent Thalassemia
Iron overload (IOL) due to increased intestinal iron absorption constitutes a major clinical problem in patients with non-transfusion-dependent thalassemia (NTDT), which is a cumulative process with advancing age. Current models for iron metabolism in patients with NTDT suggest that suppression of serum hepcidin leads to an increase in iron absorption and subsequent release of iron from the ret...
متن کاملTreating iron overload in patients with non-transfusion-dependent thalassemia
Despite receiving no or only occasional blood transfusions, patients with non-transfusion-dependent thalassemia (NTDT) have increased intestinal iron absorption and can accumulate iron to levels comparable with transfusion-dependent patients. This iron accumulation occurs more slowly in NTDT patients compared to transfusion-dependent thalassemia patients, and complications do not arise until la...
متن کاملThe role of ineffective erythropoiesis in non-transfusion-dependent thalassemia.
Ineffective erythropoiesis is the hallmark of beta-thalassemia that triggers a cascade of compensatory mechanisms resulting in clinical sequelae such as erythroid marrow expansion, extramedullary hematopoiesis, splenomegaly, and increased gastrointestinal iron absorption. Recent studies have begun to shed light on the complex molecular mechanisms underlying ineffective erythropoiesis and the as...
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ژورنال
عنوان ژورنال: Journal of Medical Cases
سال: 2020
ISSN: 1923-4155,1923-4163
DOI: 10.14740/jmc3335