{"title":"地中海贫血的孩子长大成人。","authors":"Renzo Galanello","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>In the last decades, treatment of patients with beta-thalassemia has changed considerably, with advances in red cell transfusion and the introduction of iron chelation therapy. This progress has greatly increased the probability for a thalassemic child to reach adult age with a good quality of life. At present, the prognosis for thalassemia major patients is \"open-ended\". Compliance with the conventional treatment and psychological support are critical to obtain good results. The expectancy of a long survival of good quality encourages the patients to plan their future life, having a job, a family and often children. Optimal treatment of thalassemia major is expensive and for this reason, unfortunately, available only for a minority of patients in the world. Despite the significant advances, other progresses are expected to further improve survival and quality of life. The major aim is the cure of the disease, increasing the possibility of bone marrow transplantation using HLA-matched unrelated donors, and hopefully, in the future, gene therapy. However, even the conventional treatment and in particular iron chelation is expected to improve. Efforts should be made by the Western countries, and by the international health and economic organizations to provide continuous and concrete support for achieving a high standard of management for thalassemia in all places of the world.</p>","PeriodicalId":82483,"journal":{"name":"Reviews in clinical and experimental hematology","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2003-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"A thalassemic child becomes adult.\",\"authors\":\"Renzo Galanello\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>In the last decades, treatment of patients with beta-thalassemia has changed considerably, with advances in red cell transfusion and the introduction of iron chelation therapy. This progress has greatly increased the probability for a thalassemic child to reach adult age with a good quality of life. At present, the prognosis for thalassemia major patients is \\\"open-ended\\\". Compliance with the conventional treatment and psychological support are critical to obtain good results. The expectancy of a long survival of good quality encourages the patients to plan their future life, having a job, a family and often children. Optimal treatment of thalassemia major is expensive and for this reason, unfortunately, available only for a minority of patients in the world. Despite the significant advances, other progresses are expected to further improve survival and quality of life. The major aim is the cure of the disease, increasing the possibility of bone marrow transplantation using HLA-matched unrelated donors, and hopefully, in the future, gene therapy. However, even the conventional treatment and in particular iron chelation is expected to improve. Efforts should be made by the Western countries, and by the international health and economic organizations to provide continuous and concrete support for achieving a high standard of management for thalassemia in all places of the world.</p>\",\"PeriodicalId\":82483,\"journal\":{\"name\":\"Reviews in clinical and experimental hematology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2003-03-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Reviews in clinical and experimental hematology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Reviews in clinical and experimental hematology","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
In the last decades, treatment of patients with beta-thalassemia has changed considerably, with advances in red cell transfusion and the introduction of iron chelation therapy. This progress has greatly increased the probability for a thalassemic child to reach adult age with a good quality of life. At present, the prognosis for thalassemia major patients is "open-ended". Compliance with the conventional treatment and psychological support are critical to obtain good results. The expectancy of a long survival of good quality encourages the patients to plan their future life, having a job, a family and often children. Optimal treatment of thalassemia major is expensive and for this reason, unfortunately, available only for a minority of patients in the world. Despite the significant advances, other progresses are expected to further improve survival and quality of life. The major aim is the cure of the disease, increasing the possibility of bone marrow transplantation using HLA-matched unrelated donors, and hopefully, in the future, gene therapy. However, even the conventional treatment and in particular iron chelation is expected to improve. Efforts should be made by the Western countries, and by the international health and economic organizations to provide continuous and concrete support for achieving a high standard of management for thalassemia in all places of the world.