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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Health care resources for this disease Expert centres Diagnostic tests 83 Patient organisations 36 Orphan drug s 1. If you want to subscribe to this journal, see our rates You can purchase this item in Pay Per View: Check this box if you wish to receive a copy of your message. Recently, a first patient was successfully treated with gene therapy.

The material is thalssemie no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

They have a very high obstetrical risk and a pluridiscplinary follow is necessary.

The language you choose must correspond to the language of the term you have entered. An Orphanet summary for this disease is currently under thalasseie.

The other two chains are also identical to one another but differ from the alpha chains and are termed the beta chains.

Access to the full text of this article requires a subscription. Summary and related texts. Summary An Orphanet summary for this disease is currently under development.


Annales de Biologie Clinique

If you are a subscriber, please sign in ‘My Account’ at the top right of the screen. Homozygous beta-thalassemia is a severe, transfusion dependent anaemia that also causes infertility. For all other comments, please send your remarks via contact us.

Rare autosomal dominant forms have also been described dominant beta-thalassemia; see this term. Only comments written in English can be processed.

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. Specialised Social Services Eurordis directory. The documents contained in this web site are presented for information purposes only. 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.

Outline Masquer le plan. In BT-minor, the levels of HbA2 are increased and the levels Hb are usually normal to low with microcytosis and hypochromia. Journal page Archives Contents list.

Orphanet: Drepanocytose beta thalassemie Thalassodrepanocytose

Management and treatment There are two major treatment options for BT. Access to the PDF text. In which subject field? Change the order of display of the official languages of Canada English first French first Option to display the non-official languages Bets or Portuguese Neither Spanish Portuguese Display definitions, contexts, etc.

InfancyChildhood ICD Glossaries and vocabularies Access Translation Bureau glossaries and vocabularies. Contact Help Who are we? Genetic counseling is recommended to thalaszemie couples who are at risk an informed choice among available options including prenatal diagnosis. Writing tools A collection of writing tools that cover the many facets of English and French grammar, style and usage. As per the Law relating to information storage and personal integrity, you have the right to oppose art 26 of that lawaccess art 34 of that law and rectify art 36 of that law your personal data.

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Onset generally occurs from months of age. Summary thalsasemie related texts. Access to the text HTML. Patients with BTI may or may not require occasional transfusions. 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.

Long-term transfusion of red cell concentrates results in iron overload veta hampers the vital prognosis mainly due to cardiac involvement and causes significant morbidity due to endocrinal and hepatic iron deposition.

Bêta-thalassémie majeure et grossesse. À propos de deux cas – EM|consulte

FAQ Frequently asked questions Display options. When these abnormalities affect the parts of haemoglobin called the beta chains, the disease is called beta thalassaemia. 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.

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.