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Alpha-thalassemia (α-thalassemia, α-thalassaemia) is a form of thalassemia involving the genes HBA1 and HBA2. Thalassemias are a group of inherited blood conditions which result in the impaired production of hemoglobin, the molecule that carries oxygen in the blood. Normal hemoglobin consists of two alpha chains and two beta chains; in alpha-thalassemia, there is a quantitative decrease in the amount of alpha chains, resulting in fewer normal hemoglobin molecules. Furthermore, alpha-thalassemia leads to the production of unstable beta globin molecules which cause increased red blood cell destruction. The degree of impairment is based on which clinical phenotype is present (how many genes are affected).

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dbo:abstract
  • الالفا ثلاسيما (بالإنجليزية: Alpha-thalassemia)‏ هو أحد أنواع الثلاسيميا يتصف بنقص أو انعدام في تكوين السلسلة ألفا، أحد مكونات الهيموغلوبين الأساسية. وهذا النوع من الثلاسيما يتصف بقلة شيوعه. (ar)
  • Alpha-Thalassämie (α-Thalassämie) ist eine Form der Thalassämie, welche die Gene HBA1 und HBA2 mit einbezieht. Alpha-Thalassämie ist auf eine verminderte Produktion von 1,2,3 oder 4 alpha-Globin-Ketten zurückzuführen, was zu einem relativen hohem Überschuss an beta-Globin-Ketten führt. Der Grad der Störung hängt davon ab, welcher klinische Phänotyp vorhanden ist bzw. wie viele Ketten betroffen sind. (de)
  • Alpha-thalassemia (α-thalassemia, α-thalassaemia) is a form of thalassemia involving the genes HBA1 and HBA2. Thalassemias are a group of inherited blood conditions which result in the impaired production of hemoglobin, the molecule that carries oxygen in the blood. Normal hemoglobin consists of two alpha chains and two beta chains; in alpha-thalassemia, there is a quantitative decrease in the amount of alpha chains, resulting in fewer normal hemoglobin molecules. Furthermore, alpha-thalassemia leads to the production of unstable beta globin molecules which cause increased red blood cell destruction. The degree of impairment is based on which clinical phenotype is present (how many genes are affected). (en)
  • La thalassémie alpha est une maladie génétique en rapport avec une anomalie de la synthèse de la chaîne alpha de l'hémoglobine, se traduisant par une anémie plus ou moins prononcée. Il s'agit de l'une des deux formes de thalassémies, l'autre étant la thalassémie bêta. (fr)
  • L'alfa-talassemia (α-talassemia) è un tipo di talassemia che coinvolge i geni che codificano per le emoglobine HbA e HbA2.. La malattia è caratterizzata dalla compromissione della produzione di una, due, tre o addirittura tutte e quattro le catene α dell'emoglobina, che correla direttamente con la gravità clinica della malattia. (it)
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  • Alpha-Thalassemia (en)
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dbp:caption
  • Alpha-thalassemia inheritance pattern (en)
dbp:causes
  • Deletions of chromosome 16p. (en)
dbp:diagnosis
  • Haemoglobin electrophoresis (en)
dbp:diseasesdb
  • 448 (xsd:integer)
dbp:emedicinesubj
  • article (en)
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  • Alpha-Thalassemia (en)
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  • Alpha-thalassemia (en)
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  • 846 (xsd:integer)
dbp:symptoms
  • Jaundice, Fatigue (en)
dbp:synonyms
  • α-thalassaemia (en)
dbp:treatment
  • Blood transfusion, possible splenectomy (en)
dbp:wikiPageUsesTemplate
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rdfs:comment
  • الالفا ثلاسيما (بالإنجليزية: Alpha-thalassemia)‏ هو أحد أنواع الثلاسيميا يتصف بنقص أو انعدام في تكوين السلسلة ألفا، أحد مكونات الهيموغلوبين الأساسية. وهذا النوع من الثلاسيما يتصف بقلة شيوعه. (ar)
  • Alpha-Thalassämie (α-Thalassämie) ist eine Form der Thalassämie, welche die Gene HBA1 und HBA2 mit einbezieht. Alpha-Thalassämie ist auf eine verminderte Produktion von 1,2,3 oder 4 alpha-Globin-Ketten zurückzuführen, was zu einem relativen hohem Überschuss an beta-Globin-Ketten führt. Der Grad der Störung hängt davon ab, welcher klinische Phänotyp vorhanden ist bzw. wie viele Ketten betroffen sind. (de)
  • Alpha-thalassemia (α-thalassemia, α-thalassaemia) is a form of thalassemia involving the genes HBA1 and HBA2. Thalassemias are a group of inherited blood conditions which result in the impaired production of hemoglobin, the molecule that carries oxygen in the blood. Normal hemoglobin consists of two alpha chains and two beta chains; in alpha-thalassemia, there is a quantitative decrease in the amount of alpha chains, resulting in fewer normal hemoglobin molecules. Furthermore, alpha-thalassemia leads to the production of unstable beta globin molecules which cause increased red blood cell destruction. The degree of impairment is based on which clinical phenotype is present (how many genes are affected). (en)
  • La thalassémie alpha est une maladie génétique en rapport avec une anomalie de la synthèse de la chaîne alpha de l'hémoglobine, se traduisant par une anémie plus ou moins prononcée. Il s'agit de l'une des deux formes de thalassémies, l'autre étant la thalassémie bêta. (fr)
  • L'alfa-talassemia (α-talassemia) è un tipo di talassemia che coinvolge i geni che codificano per le emoglobine HbA e HbA2.. La malattia è caratterizzata dalla compromissione della produzione di una, due, tre o addirittura tutte e quattro le catene α dell'emoglobina, che correla direttamente con la gravità clinica della malattia. (it)
rdfs:label
  • Alpha-thalassemia (en)
  • ثلاسيميا ألفا (ar)
  • Α-Thalassämie (de)
  • Thalassémie alpha (fr)
  • Alfa talassemia (it)
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  • Alpha-thalassemia (en)
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