En fonction de la nature de la chaîne touchée, on parlera d’alpha (α), de bêta (β), de delta (δ) ou de gamma (γ)-thalassémie. Cependant, en pratique clinique. la β-thalassémie intermédiaire avec des besoins transfusionnels occasionnels sur un faible effectif de patients ayant une bêta-thalassémie intermédiaire ou. Many translated example sentences containing “bêta-thalassémie” – English- French dictionary and search engine for English translations.
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Availability of new oral iron chelators and monitoring of cardiac iron overload by MRI result in further clinical improvement and current evaluation is ongoing to determine their impact on morbidity and mortality. Health care resources for this disease Expert centres Diagnostic tests 83 Patient organisations 36 Orphan drug s 1.
Two of these chains are identical and are designated the alpha chains. The documents contained in this web site are presented for information purposes only. Population migrations have lead to global distribution of the disease.
Prognosis Prognosis depends on the severity of the condition but is generally good, particularly if appropriate treatment is provided. Language Portal of Canada Access a collection of Canadian resources on all aspects of English and French, including quizzes. Thalassaemia is caused by abnormalities in the genes units of heredity that control production of haemoglobin. Contact Help Who are we? You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not be used or stored, be corrected, clarified, updated or deleted.
The marketing authorization for deferiprone, another orally active iron chelator, particularly effective in removing heart iron, is restricted to cases in which treatment with deferoxamine fails or is contraindicated. All hemoglobin consists of two parts: Access a collection of Canadian resources on all aspects of English and French, including quizzes. Homozygous beta-thalassemia is a severe, transfusion dependent anaemia that also causes infertility. The other two chains are also identical to one another but differ from the alpha chains and are termed the beta chains.
The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.
Annales de Biologie Clinique
Access to the full text of this article requires a subscription. A collection of writing tools that cover the many facets of English and French grammar, style and usage. Detailed information Article for general public Deutsch If you want to subscribe to this journal, see our rates You can purchase this item in Pay Per View: An Orphanet summary for this disease is currently under development.
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Glossaries and vocabularies Access Translation Bureau glossaries and vocabularies. When these abnormalities affect the parts of haemoglobin called the beta chains, the disease is called beta thalassaemia.
Hypersplenism, cholelithiasis, extramedullary hematopoiesis, thrombotic complications and progressive iron overload are the main clinical features that may complicate the course of BTI. Summary and related texts. Summary An Orphanet summary for this disease is currently under development. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment. Health care resources for this disease Expert centres Diagnostic tests Patient organisations 46 Orphan drug s Clinical description Three main types of BT have been described minor, intermedia and tgalassemie see these terms.
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Recently, a first patient was successfully treated with gene therapy. In which subject field? Access to the text HTML. The documents contained in this web site are presented for information purposes only. Rare autosomal dominant forms have also been described dominant beta-thalassemia; see this term.
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There are two major treatment options for BT. If you are a subscriber, please sign thakassemie ‘My Account’ at the top right of the screen.
Onset generally occurs from months of age. Summary and related texts. Additional information Further information on this disease Classification s 4 Gene s 1 Clinical signs and symptoms Publications in PubMed Other website s 9.
Orphanet: Drepanocytose beta thalassemie Thalassodrepanocytose
Patients with BTI may or may not require occasional transfusions. Only comments written in English can be thalassejie. For all other comments, please send your remarks via contact us.
Journal page Archives Contents list. Access to the PDF text. Management and treatment There are two major treatment options for BT. In BT-minor, the levels of HbA2 are increased and the levels Hb are usually normal to low with microcytosis and hypochromia.
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