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Haematopoietic stem cell transplantation for the management of haemoglobinopathies in Greek patients 造血干细胞移植治疗希腊患者的血红蛋白病
Transfusion science Pub Date : 2000-12-01 DOI: 10.1016/S0955-3886(00)00103-X
J Peristeri, V Kitra, E Goussetis, D Petropoulos, M Theodosaki, A Kattamis, S Graphakos
{"title":"Haematopoietic stem cell transplantation for the management of haemoglobinopathies in Greek patients","authors":"J Peristeri, V Kitra, E Goussetis, D Petropoulos, M Theodosaki, A Kattamis, S Graphakos","doi":"10.1016/S0955-3886(00)00103-X","DOIUrl":"10.1016/S0955-3886(00)00103-X","url":null,"abstract":"","PeriodicalId":80242,"journal":{"name":"Transfusion science","volume":"23 3","pages":"Pages 263-264"},"PeriodicalIF":0.0,"publicationDate":"2000-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0955-3886(00)00103-X","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21924006","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 4
The design and properties of 3-hydroxypyridin-4-one iron chelators with high pFe3+ values 3-羟基吡啶-4- 1高pFe3+螯合剂的设计与性能
Transfusion science Pub Date : 2000-12-01 DOI: 10.1016/S0955-3886(00)00106-5
Zu D Liu, S Piyamongkol, Robert C Hider
{"title":"The design and properties of 3-hydroxypyridin-4-one iron chelators with high pFe3+ values","authors":"Zu D Liu, S Piyamongkol, Robert C Hider","doi":"10.1016/S0955-3886(00)00106-5","DOIUrl":"10.1016/S0955-3886(00)00106-5","url":null,"abstract":"","PeriodicalId":80242,"journal":{"name":"Transfusion science","volume":"23 3","pages":"Pages 269-270"},"PeriodicalIF":0.0,"publicationDate":"2000-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0955-3886(00)00106-5","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21923845","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 21
Primary and secondary haemochromatosis 原发性和继发性血色病
Transfusion science Pub Date : 2000-12-01 DOI: 10.1016/S0955-3886(00)00110-7
J.J.M Marx
{"title":"Primary and secondary haemochromatosis","authors":"J.J.M Marx","doi":"10.1016/S0955-3886(00)00110-7","DOIUrl":"10.1016/S0955-3886(00)00110-7","url":null,"abstract":"","PeriodicalId":80242,"journal":{"name":"Transfusion science","volume":"23 3","pages":"Pages 183-184"},"PeriodicalIF":0.0,"publicationDate":"2000-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0955-3886(00)00110-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21925244","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 3
The Sydney Children's Hospital experience with the oral iron chelator deferiprone (L1) 悉尼儿童医院口服铁螯合剂去铁酮(L1)的经验
Transfusion science Pub Date : 2000-12-01 DOI: 10.1016/S0955-3886(00)00092-8
Vasili Berdoukas , Tim Bohane , Craig Eagle , Rob Lindeman , Keshani DeSilva , Vivienne Tobias , Dorothy Painter , Ian Fraser
{"title":"The Sydney Children's Hospital experience with the oral iron chelator deferiprone (L1)","authors":"Vasili Berdoukas , Tim Bohane , Craig Eagle , Rob Lindeman , Keshani DeSilva , Vivienne Tobias , Dorothy Painter , Ian Fraser","doi":"10.1016/S0955-3886(00)00092-8","DOIUrl":"10.1016/S0955-3886(00)00092-8","url":null,"abstract":"","PeriodicalId":80242,"journal":{"name":"Transfusion science","volume":"23 3","pages":"Pages 239-240"},"PeriodicalIF":0.0,"publicationDate":"2000-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0955-3886(00)00092-8","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21923995","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 22
Desferrioxamine-chelatable iron (DCI), a component of serum non-transferrin-bound iron (NTBI) used for assessing iron chelation therapy 去铁胺螯合铁(DCI),血清非转铁蛋白结合铁(NTBI)的一个组成部分,用于评估铁螯合治疗
Transfusion science Pub Date : 2000-12-01 DOI: 10.1016/S0955-3886(00)00093-X
William Breuer , Marieke J.J Ermers , Pensri Pootrakul , Ayala Abramov , Chaim Hershko , Z Ioav Cabantchik
{"title":"Desferrioxamine-chelatable iron (DCI), a component of serum non-transferrin-bound iron (NTBI) used for assessing iron chelation therapy","authors":"William Breuer , Marieke J.J Ermers , Pensri Pootrakul , Ayala Abramov , Chaim Hershko , Z Ioav Cabantchik","doi":"10.1016/S0955-3886(00)00093-X","DOIUrl":"10.1016/S0955-3886(00)00093-X","url":null,"abstract":"","PeriodicalId":80242,"journal":{"name":"Transfusion science","volume":"23 3","pages":"Pages 241-242"},"PeriodicalIF":0.0,"publicationDate":"2000-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0955-3886(00)00093-X","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21923996","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 13
Index 指数
Transfusion science Pub Date : 2000-12-01 DOI: 10.1016/S0955-3886(00)00114-4
{"title":"Index","authors":"","doi":"10.1016/S0955-3886(00)00114-4","DOIUrl":"https://doi.org/10.1016/S0955-3886(00)00114-4","url":null,"abstract":"","PeriodicalId":80242,"journal":{"name":"Transfusion science","volume":"23 3","pages":"Page XXII"},"PeriodicalIF":0.0,"publicationDate":"2000-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0955-3886(00)00114-4","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134831877","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Index 指数
Transfusion science Pub Date : 2000-12-01 DOI: 10.1016/S0955-3886(00)00115-6
{"title":"Index","authors":"","doi":"10.1016/S0955-3886(00)00115-6","DOIUrl":"https://doi.org/10.1016/S0955-3886(00)00115-6","url":null,"abstract":"","PeriodicalId":80242,"journal":{"name":"Transfusion science","volume":"23 3","pages":"Pages XXXIII-XXXIV"},"PeriodicalIF":0.0,"publicationDate":"2000-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0955-3886(00)00115-6","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"134831879","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Transfusional iron overload and chelation therapy with deferoxamine and deferiprone (L1) 输注铁超载与去铁胺和去铁酮螯合治疗(L1)
Transfusion science Pub Date : 2000-12-01 DOI: 10.1016/S0955-3886(00)00089-8
George J Kontoghiorghes, Katerina Pattichi, Michael Hadjigavriel, Annita Kolnagou
{"title":"Transfusional iron overload and chelation therapy with deferoxamine and deferiprone (L1)","authors":"George J Kontoghiorghes,&nbsp;Katerina Pattichi,&nbsp;Michael Hadjigavriel,&nbsp;Annita Kolnagou","doi":"10.1016/S0955-3886(00)00089-8","DOIUrl":"10.1016/S0955-3886(00)00089-8","url":null,"abstract":"<div><p>Iron is essential for all living organisms. Under normal conditions there is no regulatory and rapid iron excretion in humans and body iron levels are mainly regulated from the absorption of iron from the gut. Regular blood transfusions in thalassaemia and other chronic refractory anaemias can result in excessive iron deposition in tissues and organs. This excess iron is toxic, resulting in tissue and organ damage and unless it is removed it can be fatal to those chronically transfused. Iron removal in transfusional iron overload is achieved using chelation therapy with the chelating drugs deferoxamine (DF) and deferiprone (L1). Effective chelation therapy in chronically transfused patients can only be achieved if iron chelators can remove sufficient amounts of iron, equivalent to those accumulated in the body from transfusions, maintaining body iron load at a non-toxic level. In order to maintain a negative iron balance, both chelating drugs have to be administered almost daily and at high doses. This form of administration also requires that a chelator has low toxicity, good compliance and low cost. DF has been a life-saving drug for thousands of patients in the last 40 years. It is mostly administered by subcutaneous infusion (40–60 mg/kg, 8–12 h, 5 days per week), is effective in iron removal and has low toxicity. However, less than 10% of the patients requiring iron chelation therapy worldwide are able to receive DF because of its high cost, low compliance and in some cases toxicity. In the last 10 years we have witnessed the emergence of oral chelation therapy, which could potentially change the prognosis of all transfusional iron-loaded patients. The only clinically available oral iron chelator is L1, which has so far been taken by over 6000 patients worldwide, in some cases daily for over 10 years, with very promising results. L1 was able to bring patients to a negative iron balance at doses of 50–120 mg/kg/day. It increases urinary iron excretion, decreases serum ferritin levels and reduces liver iron in the majority of chronically transfused iron-loaded patients. Despite earlier concerns of possible increased risk of toxicity, all the toxic side effects of L1 are currently considered reversible, controllable and manageable. These include agranulocytosis (0.6%), musculoskeletal and joint pains (15%), gastrointestinal complaints (6%) and zinc deficiency (1%). The incidence of these toxic side effects could in general be reduced by using lower doses of L1 or combination therapy with DF. Combination therapy could also benefit patients experiencing toxicity with DF and those not responding to either chelator alone. The overall efficacy and toxicity of L1 is comparable to that of DF in both animals and humans. Despite the steady progress in iron chelation therapy with DF and L1, further investigations are required for optimising their use in patients by selecting improved dose protocols, by minimising their toxicity and by identifying","PeriodicalId":80242,"journal":{"name":"Transfusion science","volume":"23 3","pages":"Pages 211-223"},"PeriodicalIF":0.0,"publicationDate":"2000-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0955-3886(00)00089-8","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21923992","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 118
Competition studies of L1-deferiprone with copper and iron. Possible implications on efficacy, toxicity and new therapeutic applications l1 -去铁蛋白与铜、铁的竞争研究。可能对疗效、毒性和新的治疗应用产生影响
Transfusion science Pub Date : 2000-12-01 DOI: 10.1016/S0955-3886(00)00102-8
I Pashalidis , G.J Kontoghiorghes
{"title":"Competition studies of L1-deferiprone with copper and iron. Possible implications on efficacy, toxicity and new therapeutic applications","authors":"I Pashalidis ,&nbsp;G.J Kontoghiorghes","doi":"10.1016/S0955-3886(00)00102-8","DOIUrl":"10.1016/S0955-3886(00)00102-8","url":null,"abstract":"","PeriodicalId":80242,"journal":{"name":"Transfusion science","volume":"23 3","pages":"Pages 259-261"},"PeriodicalIF":0.0,"publicationDate":"2000-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0955-3886(00)00102-8","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21924005","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 13
Combining iron chelators with the nucleoside analog didanosine in anti-HIV therapy 铁螯合剂与核苷类似物二腺苷联合抗hiv治疗
Transfusion science Pub Date : 2000-12-01 DOI: 10.1016/S0955-3886(00)00097-7
Niki A Georgiou , Tjomme van der Bruggen , Maroeska Oudshoorn , Hans H.L.M Nottet , Joannes J.M Marx , B Sweder van Asbeck
{"title":"Combining iron chelators with the nucleoside analog didanosine in anti-HIV therapy","authors":"Niki A Georgiou ,&nbsp;Tjomme van der Bruggen ,&nbsp;Maroeska Oudshoorn ,&nbsp;Hans H.L.M Nottet ,&nbsp;Joannes J.M Marx ,&nbsp;B Sweder van Asbeck","doi":"10.1016/S0955-3886(00)00097-7","DOIUrl":"10.1016/S0955-3886(00)00097-7","url":null,"abstract":"","PeriodicalId":80242,"journal":{"name":"Transfusion science","volume":"23 3","pages":"Pages 249-250"},"PeriodicalIF":0.0,"publicationDate":"2000-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0955-3886(00)00097-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"21924000","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 4
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