THE TRIPLICATED ALPHA-GENE LOCUS AND HETEROZYGOUS BETA THALASSAEMIA: A CASE OF THALASSAEMIA INTERMEDIA
نویسندگان
چکیده
منابع مشابه
Thalassaemia Intermedia: an Update
Our understanding of the molecular and pathophysiological mechanisms underlying the disease process in patients with thalassaemia intermedia (TI) has substantially increased over the past decade. TI encompasses a wide clinical spectrum of beta-thalassaemia phenotypes. Some TI patients are asymptomatic until adult life, whereas others are symptomatic from as young as 2 years of age. A number of ...
متن کاملBeta-Thalassaemia Intermedia: Evaluation of Endocrine and Bone Complications
OBJECTIVE Data about endocrine and bone disease in nontransfusion-dependent thalassaemia (NTDT) is scanty. The aim of our study was to evaluate these complications in β-TI adult patients. METHODS We studied retrospectively 70 β-TI patients with mean followup of 20 years. Data recorded included age, gender, haemoglobin and ferritin levels, biochemical and endocrine tests, liver iron concentrat...
متن کاملIron overload in Beta thalassaemia major and intermedia patients.
ABSTRACT BACKGROUND In beta thalassaemia major multiple blood transfusions, ineffective erythropoiesis and increased gastrointestinal iron absorption lead to iron overload in the body. Iron overload impairs the immune system, placing patients at greater risk of infection and illness. Iron overload can be determined by serum ferritin measurement. OBJECTIVE The aim of the present study is to...
متن کاملModern treatment of thalassaemia intermedia.
The term thalassaemia intermedia includes a large spectrum of conditions of varying severity. Blood transfusion and chelation are necessary in some patients, especially during childhood, in order to promote growth and prevent bone deformities. Alloimunisation, however, is frequent and can be difficult to control. Splenectomy is usually needed at some time because of hypersplenism and mechanical...
متن کاملAnaesthetic management of two patients with beta-thalassaemia intermedia.
There is paucity of literature regarding the anaesthetic management of patients with thalassaemia intermedia. In this case study, the anaesthetic management and concerns in two children with thalassaemia intermedia aged eleven and nine years undergoing herniotomy and splenectomy respectively is reported. Both children had unanticipated difficulty in airway management and high intraoperative blo...
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ژورنال
عنوان ژورنال: British Journal of Haematology
سال: 1983
ISSN: 0007-1048,1365-2141
DOI: 10.1111/j.1365-2141.1983.tb02854.x