Cardinal chains level 704/9/2023 ![]() Variable, sometimes transfusion-dependent chronic hemolytic anemia HbX = approximately 150 different variants HbX/HbA Hemolysis caused by infections/ medical drugs As a result, medical treatment is becoming more and more part of adult medicine, rather than pediatrics alone. Topical grounds for the publication of this article are the increase in the number of people affected, which has implications for care provision, and the fact that optimum treatment can give patients a steadily-increasing projected life span. It aims to provide a brief summary of the most important clinical pictures and indicate the features that can be used to identify those with these diseases with low-level symptoms, but not gene carriers in good health, in general practice ( Tables 2, ,3). This review article should be considered an extension of the original article “Hemoglobinopathies in Germany-a longitudinal study over four decades,” also published in Deutsches Ärzteblatt International ( 6). The total number of patients diagnosed with these diseases in the author’s laboratory from 1970 to 2010 is 5831. The following statements can be made regarding gene carriers: prevalence among the 9 million immigrants from countries with a high risk of hemoglobinopathies as a whole is 4.5%, giving a figure of 400 000 for the number of hemoglobinopathy gene carriers ( 6). There are no epidemiological studies on their frequency. Germany is one of the countries in which hemoglobinopathies have increased in recent years ( 4– 7). ![]() Optimally treated patients have a projected life span of 50 to 60 years. More than 90% of patients currently survive into adulthood. ![]() Blood transfusions should be given only when strictly indicated. Drugs to treat the symptoms of sickle-cell disease include analgesics, antibiotics, ACE inhibitors and hydroxyurea. Supportive, rather than curative, treatment consists of periodic blood transfusions for life, combined with iron chelation. Stem-cell transplantation is the preferred treatment for the severe forms of thalassemia. 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. There are many subtypes and combined types in each group. α- and β-thalassemia are the main types of thalassemia the main structural hemoglobin variants are HbS, HbE and HbC. They fall into two main groups: thalassemia syndromes and structural hemoglobin variants (abnormal hemoglobins). The hemoglobinopathies encompass all genetic diseases of hemoglobin.
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