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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Detailed information Article for general public Deutsch Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. Beta-thalassemia major ; Infertility ; Pregnancy ; Anesthesia ; Preimplantation genetic diagnosis.

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Annales de Biologie Clinique

Recently, a first patient was successfully treated with gene thalxssemie. Genetic counseling is recommended to permit couples who are at risk an informed choice among available options including prenatal diagnosis. Homozygous beta-thalassemia is a severe, transfusion dependent anaemia that also causes infertility. The globin part of Hb [hemoglobin] A has 4 protein sections called polypeptide chains.

Additional information Further information on this disease Classification s 4 Gene s 1 Clinical signs and symptoms Publications in PubMed Other website s 9.

Writing tools A collection of writing tools that cover the many facets of English and French grammar, style and thalassemie. 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.


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

Only comments written in English can be processed. Other search option s Alphabetical list. Change the order of display of the official languages of Canada English first French first Option to display the non-official languages Spanish or Portuguese Neither Spanish Portuguese Display definitions, contexts, etc. An Orphanet summary for this disease is currently under development. There are two major treatment options for BT.

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Contact Help Who are we? Preimplantation genetic diagnosis is possible. Outline Masquer le plan. All hemoglobin consists of two parts: FAQ Frequently asked questions Display options. InfancyChildhood ICD Summary and related texts.

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. The language you choose must correspond to the language of the term you have thalassrmie. Very few pregnancies are reported among such patients. Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted.


Only comments written in English can be processed. Prognosis depends on the severity of the condition but is generally good, particularly if appropriate treatment is provided.

Hypersplenism, cholelithiasis, extramedullary hematopoiesis, thrombotic complications and progressive iron overload are the main clinical features that may complicate the course of BTI. Access to the full text of this article requires brta subscription. The owners of this website hereby guarantee to respect the legal yhalassemie conditions, applicable in France, and not to disclose this data to third parties.

Patients with BTI may or may not require occasional transfusions. In rare instances, BT trait is associated with trichothiodystrophy or X-linked thrombocytopenia see these terms. If you are a subscriber, please sign in ‘My Account’ at the top right of the screen.

Health care resources for this disease Expert centres Diagnostic tests 83 Patient organisations 36 Orphan drug s 1.