Survival in beta-thalassaemia major in the UK: data from the UK Thalassaemia Register.
نویسندگان
چکیده
About 50% of UK patients with beta-thalassaemia major die before the age of 35 years, mainly because conventional iron-chelation therapy is too burdensome for full adherence. Patients require an individually-tailored treatment plan incorporating new, more tolerable approaches.
منابع مشابه
Improved survival of thalassaemia major in the UK and relation to T2* cardiovascular magnetic resonance
BACKGROUND The UK Thalassaemia Register records births, deaths and selected clinical data of patients with thalassaemia who are resident in the UK. A study of survival and causes of death was undertaken which aimed to include the possible impact of T2* cardiovascular magnetic resonance (CMR). METHODS The Register was updated to the end of 2003, copies of death certificates were obtained, and ...
متن کاملبررسی فراوانی فاکتور V لیدن (G1691A)، پروترومبین G20210A و تغییر C667T در ژن سازنده آنزیم متیلن تتراهیدروفولات ردوکتاز (MTHFR) در بیماران تالاسمی ماژور و اینترمدیا مراجعه کننده به مرکز تحقیقات تالاسمی در مقایسه با افراد سالم در شمال ایران
Background and purpose: Mutation in factor V Leiden (R506Q), mutation of G20210Â in prothrombin and mutation of Ç667T in methylenetetrahydrofolate reductase (MTFHR) are part of genetic variant that increase the risk of thrombosis. The purpose of this study was to define the frequencies of three risk factors among thalassaemia major and thalassaemia intermedia compared with the normal subjects...
متن کاملA national register for surveillance of inherited disorders: beta thalassaemia in the United Kingdom.
OBJECTIVE To demonstrate the value of a national register for surveillance of services for an inherited disorder. METHODS Data from the United Kingdom Thalassaemia Register and the United Kingdom Register of Prenatal Diagnosis for Haemoglobin Disorders were combined in a database; these registers include all fetuses known to have been diagnosed with beta thalassaemia major, beta thalassaemia ...
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 کاملPrenatal diagnosis of beta-thalassaemia by chorionic villous sampling.
OBJECTIVE To establish intrauterine diagnosis of thalassaemia major in couples with thalassaemia trait by chorionic villous sampling. METHODS A total of 60 couples with children suffering from transfusion dependent beta-thalassaemia or couples who were known carriers of beta-thalassaemia were included in this study. The standard procedure was followed for the collection of samples which was f...
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ورودعنوان ژورنال:
- Lancet
دوره 355 9220 شماره
صفحات -
تاریخ انتشار 2000