Splenectomy for people with thalassaemia major or intermedia

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β-Thalassaemia intermedia masquerading as β-thalassaemia major.

To cite: Singh A, Varma S. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/ bcr-2014-207637 DESCRIPTION A 19-year-old man presented with dysmorphic facies, progressive fatigability and exertional breathlessness from the age of 15 years. On examination the patient had classical ‘Chipmunk facies’ (figure 1), short stature, delayed puberty and mild splenomegaly. X-ray of...

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Iron 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...

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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 ...

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Modern 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...

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Living a 'normal' life: young people coping with thalassaemia major or sickle cell disorder.

This qualitative paper explores the strategies and resources young people use to cope with sickle cell disorder or thalassaemia major, two haemoglobin disorders with serious implications for health and survival. By focusing on coping strategies, we explore how young people attempt to take control over their lives. The respondents, largely of South Asian and African Caribbean origin, aged betwee...

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ژورنال

عنوان ژورنال: Cochrane Database of Systematic Reviews

سال: 2019

ISSN: 1465-1858

DOI: 10.1002/14651858.cd010517.pub3