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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There are two major treatment options for BT. They have a very high obstetrical risk and a pluridiscplinary follow is necessary. Writing tools A collection of writing tools that cover brta many facets of English and French grammar, style and usage.
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.
When these abnormalities affect the parts of haemoglobin called the beta chains, the disease is called beta thalassaemia. In which subject field? If you want to subscribe to this journal, see our rates You can purchase this item in Pay Per View: Homozygous beta-thalassemia is a severe, transfusion dependent anaemia that also causes infertility.
Very few pregnancies are reported among such patients. Other search option s Alphabetical list. The documents contained in this web site are presented for information purposes only. Thalassaemia is caused by abnormalities in the genes units of heredity that control production of haemoglobin. Check this box yhalassemie you wish to receive a copy of your message. A collection of writing tools that cover the many facets of English and French grammar, style and usage.
All hemoglobin consists of two parts: Health care resources for this disease Expert centres Diagnostic tests 83 Patient organisations 36 Orphan drug s 1. The owners of this website hereby guarantee to respect the legal confidentiality conditions, applicable in France, and not to disclose this data to third parties.
Prognosis depends on the severity of the condition but is generally good, particularly if appropriate treatment is provided. In rare instances, BT trait is associated with trichothiodystrophy or X-linked thrombocytopenia see these terms. For all other comments, please send your remarks via contact us.
Additional information Further information on this disease Classification s 2 Gene s 1 Other website s thalassemiee. If you are a subscriber, please sign in ‘My Account’ at the top right of the screen. Summary and related texts. FAQ Frequently asked questions Display options. Rare autosomal dominant forms have also been described dominant beta-thalassemia; see this term.
Top of the page – Article Outline. Access to the full text of this article requires a subscription. Other search option s Alphabetical list.
Annales de Biologie Clinique
Beta-thalassemia major ; Infertility ; Pregnancy ; Anesthesia ; Preimplantation genetic diagnosis. Long-term transfusion of red cell concentrates results in iron overload which hampers the vital prognosis mainly due to cardiac involvement and causes significant morbidity due to endocrinal and hepatic iron deposition.
InfancyChildhood ICD For all other comments, please send your remarks via contact us. Check this box if you wish to receive a copy of your message. Patients with BTI may or may not thalassemid occasional transfusions. 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 thxlassemie mortality.
Change the order of display of the official languages of Canada English first French thalaszemie Option to display the non-official languages Spanish or Portuguese Neither Spanish Portuguese Display definitions, contexts, etc.
You may thus request that your data, should it be inaccurate, incomplete, unclear, outdated, not thapassemie used or stored, be corrected, clarified, updated or deleted. Indeferasirox, a once-daily orally administered iron chelator, obtained EU marketing authorization as an Orphan drug for first-line treatment of BT-related iron overload.
Population migrations have lead to global distribution of the disease.
Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.
An Orphanet summary for this disease is currently under development.
Only comments seeking to improve the quality and accuracy of information on the Orphanet thwlassemie are accepted. Onset generally occurs from months of age. Detailed information Article for general public Deutsch Health care resources for this disease Expert centres Diagnostic tests Patient organisations 46 Orphan drug s Access to the PDF text.
Orphanet: Drepanocytose beta thalassemie Thalassodrepanocytose
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. Journal page Archives Contents list. Two of these chains are identical and are designated the alpha chains. Only comments written in English can be processed. Language Portal of Canada Access a collection of Canadian resources on all aspects of English and French, including quizzes. Management and treatment There are two major treatment options for BT.