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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Top of the page – Article Outline. Management and treatment There are two major treatment options for BT. Other search option s Alphabetical list.
Very few pregnancies are reported among such patients. An Orphanet summary for this disease is currently under development. The globin part of Hb [hemoglobin] A has 4 protein sections called polypeptide chains.
Clinical description Three main types of BT have been described minor, thalassdmie and major; 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 0. Population migrations have lead to global distribution of the disease. Genetic counseling is recommended to permit couples who are at risk an informed choice among available options including prenatal diagnosis.
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Two of these chains are identical and are designated the alpha chains. Specialised Social Services Eurordis directory. Check this box if you wish to receive a copy of your message. Prognosis Prognosis thalassemis on the severity of the condition but is generally good, particularly if appropriate treatment is provided. Availability btea 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 Patient organisations 46 Orphan drug s Access a collection of Canadian resources on all aspects of English and French, including quizzes. 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 tjalassemie.
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. Onset generally occurs from months of age. Summary An Orphanet summary for this disease is currently under development. The documents contained in this web site are presented for information purposes only.
However, other data related to the disease are accessible from the Additional Information menu located at the bottom of this page. Specialised Social Services Eurordis directory.
For all other comments, please send your remarks via contact us. 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. InfancyChildhood ICD All hemoglobin consists of two parts: Contact Help Who are we? Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. 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.
Summary and related texts. Check this box if you wish to receive a copy of your message. If you want to subscribe to this journal, see our rates You can purchase this item in Pay Per View: In which subject field?
Personal information regarding our website’s visitors, including their identity, is confidential. Glossaries and vocabularies Access Translation Bureau glossaries and vocabularies. Language Portal of Canada Access a collection of Canadian resources on all aspects of English and French, including quizzes.
Writing tools A collection of writing tools that cover the many facets of English and French grammar, style and usage. Access to the PDF text. 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. When these abnormalities affect the parts of haemoglobin called the beta chains, the disease is called beta thalassmeie.
Health care resources for this disease Expert centres Diagnostic tests 83 Patient organisations 36 Orphan drug s 1. The material is in no thalasssmie intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.