Jujin We report here an updated review on epidemiologic and molecular data of the hemoglobinopathies in Tunisia. Just click on PDF on the contents page. Tunis Med ; Hemoglobins in Togolese newborns: Hemoglobin screening on newborns has shown a frequency of alpha-thal trait of 5. Molecular analysis on beta-thalassemia patients allowed to identify 21 different alleles. How to cite item. 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.
|Published (Last):||8 August 2017|
|PDF File Size:||16.72 Mb|
|ePub File Size:||17.77 Mb|
|Price:||Free* [*Free Regsitration Required]|
This article has been cited by other articles in PMC. Abstract Background Hemoglobinopathies are among the most common inherited diseases around the world. They have become much more common recently in northern and central Europe, including Germany, due to immigration. Method Selective review of the literature with consideration of national guidelines. Results The hemoglobinopathies encompass all genetic diseases of hemoglobin.
They fall into two main groups: thalassemia syndromes and structural hemoglobin variants abnormal hemoglobins. There are many subtypes and combined types in each group.
The highly variable clinical manifestations of the hemoglobinopathies range from mild hypochromic anemia to moderate hematological disease to severe, lifelong, transfusion-dependent anemia with multiorgan involvement. Stem-cell transplantation is the preferred treatment for the severe forms of thalassemia. Supportive, rather than curative, treatment consists of periodic blood transfusions for life, combined with iron chelation.
Drugs to treat the symptoms of sickle-cell disease include analgesics, antibiotics, ACE inhibitors and hydroxyurea. Blood transfusions should be given only when strictly indicated. Optimally treated patients have a projected life span of 50 to 60 years.
Adequate care of the affected patients requires a wide variety of diagnostic and therapeutic measures. They were originally found mainly in the Mediterranean area and large parts of Asia and Africa 3.
International migration has spread them from those areas all over the world. In many parts of Europe today, hemoglobin Hb defects are classified as endemic diseases 3 table 1.