Alpha-Thalassemia

Common Name(s)

Alpha-Thalassemia

Alpha-thalassemia is a blood disorder that reduces the body's production of hemoglobin. Affected individuals have anemia, which can cause pale skin, weakness, fatigue, and more serious complications. Two types of alpha-thalassemia can cause health problems: the more severe type is known as Hb Bart syndrome; the milder form is called HbH disease. Hb Bart syndrome may be characterized by hydrops fetalis; severe anemia; hepatosplenomegaly; heart defects; and abnormalities of the urinary system or genitalia. Most babies with this condition are stillborn or die soon after birth. HbH disease may cause mild to moderate anemia; hepatosplenomegaly; jaundice; or bone changes. Alpha-thalassemia typically results from deletions involving the HBA1 and HBA2 genes. The inheritance is complex, and can be read about here. No treatment is effective for Hb Bart syndrome; for HbH disease, occasional red blood cell transfusions may be needed.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Alpha-Thalassemia" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Alpha-Thalassemia" returned 200 free, full-text research articles on human participants. First 3 results:

Alpha thalassemia among sickle cell anaemia patients in Kampala, Uganda.
 

Author(s): Irene Lubega, Christopher M Ndugwa, Edison A Mworozi, James K Tumwine

Journal: Afr Health Sci. 2015 Jun;15(2):682-9.

 

Sickle cell anaemia is prevalent in sub Saharan Africa. While α+-thalassaemia is known to modulate sickle cell anaemia, its magnitude and significance in Uganda have hitherto not been described.

Last Updated: 30 Jun 2015

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[Molecular bases of α-thalassemia in Argentina].
 

Author(s): Karen G Scheps, Liliana Francipane, Abigail Nash, Gloria E Cerrone, Silvia B Copelli, Viviana Varela

Journal: Medicina (B Aires). 2015 ;75(2):81-6.

 

The α-thalassemia is one of the most common hereditary disorders worldwide. Currently, molecular diagnostics is the only available tool to achieve an accurate diagnosis. The purpose of this study was to characterize the molecular bases of these syndromes in our environment and to ...

Last Updated: 29 Apr 2015

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Parvovirus B19 infection presenting with severe erythroid aplastic crisis during pregnancy in a woman with autoimmune hemolytic anemia and alpha-thalassemia trait: a case report.
 

Author(s): Chi-Ching Chen, Chin-Shan Chen, Wei-Yao Wang, Jui-Shan Ma, Hwei-Fan Shu, Frank S Fan

Journal:

 

Parvovirus B19 virus commonly causes subclinical infection, but it can prove fatal to the fetus during pregnancy and cause severe anemia in an adult with hemolytic diseases. We present the case of a woman with autoimmune hemolytic anemia who was diagnosed with parvovirus B19-induced ...

Last Updated: 19 Apr 2015

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Reviews from the PubMed Database

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The terms "Alpha-Thalassemia" returned 11 free, full-text review articles on human participants. First 3 results:

Sonographic markers of fetal α-thalassemia major.
 

Author(s): Xinyan Li, Qichang Zhou, Ming Zhang, Xiaoxian Tian, Yili Zhao

Journal: J Ultrasound Med. 2015 Feb;34(2):197-206.

 

α-Thalassemia prevails in Southeast Asia, where α-thalassemia major is a lethal type. Sonography is a helpful and cost-effective screening tool for detecting α-thalassemia major fetuses. The cardiothoracic ratio, placental thickness, and middle cerebral artery peak systolic velocity ...

Last Updated: 23 Jan 2015

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Clinical manifestations of α-thalassemia.
 

Author(s): Elliott P Vichinsky

Journal:

 

α-Thalassemia mutations affect up to 5% of the world's population. The clinical spectrum ranges from an asymptomatic condition to a fatal in utero disease. Hemoglobin H disease results from mutations of three α-globin genes. Deletional forms result in a relatively mild anemia, whereas ...

Last Updated: 2 May 2013

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Variable clinical phenotypes of alpha-thalassemia syndromes.
 

Author(s): Sylvia Titi Singer

Journal:

 

Genetic mutations of the alpha genes are common worldwide. In Asia and particularly Southeast Asia, they can result in clinically significant types of alpha-thalassemia, namely hemoglobin (Hb) H disease and Hb Bart's hydrops fetalis. The latter is generally a fatal intrauterine condition, ...

Last Updated: 20 Jul 2009

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Screening for Alpha Thalassemia in Healthy Volunteers
 

Status: Not yet recruiting

Condition Summary: Alpha Thalassemia

 

Last Updated: 11 May 2016

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Genetics of Alpha Thalassemia in Israeli Ethnic Groups
 

Status: Recruiting

Condition Summary: Alpha Thalassemia

 

Last Updated: 22 Jun 2011

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