Thalassemia Reports最新文献_第7页

Diagnostic strategies in hemoglobinopathy testing, the role of a reference laboratory in the USA 血红蛋白病检测中的诊断策略，美国参考实验室的作用

IF 0.3

Thalassemia Reports Pub Date : 2018-04-19 DOI: 10.4081/thal.2018.7476

J. Oliveira

引用次数: 0

Hepatitis C virus treatment advances for thalassaemia patients 地中海贫血患者丙型肝炎病毒治疗取得进展

IF 0.3

Thalassemia Reports Pub Date : 2018-04-19 DOI: 10.4081/THAL.2018.7491

G. Papatheodoridis

{"title":"Hepatitis C virus treatment advances for thalassaemia patients","authors":"G. Papatheodoridis","doi":"10.4081/THAL.2018.7491","DOIUrl":"https://doi.org/10.4081/THAL.2018.7491","url":null,"abstract":"Chronic infection with hepatitis C virus (HCV) is a major problem for thalassaemia patients, as blood transfusions before 1990 were associated with a high risk of HCV infection. Given the high prevalence of co-morbidities, thalassaemia patients are at an increased risk for dying from end-stage liver disease or hepatocellular carcinoma. HCV treatment in thalassaemia patients was challenging in the interferon-alfa (IFN) era not only due to its unfavourable safety and tolerability profile but due to necessary combined use of ribavirin (RBV) and the subsequent haemolysis and increased need for blood transfusions. The introduction of the current direct acting antivirals (DAAs), which can be used in IFNfree and RBV-free regimens, has dramatically improved the management of all HCV patients including those with thalassaemia. Currently, depending on HCV genotype and availability in each country, the main available DAAs combinations are the co-formulation of sofosbuvir with ledipasvir (nucleotide analogue NS5B polymerase inhibitor/NS5A inhibitor, one tablet of 400/90 mg once daily),, the co-formulation of paritaprevir boosted by ritonavir with ombitasvir (NS3/4 protease inhibitor/ritonavir/NS5A inhibitor, two tablets of 75/50/12.5 mg once daily) perhaps with addition of dasabuvir (non-nucleos(t)ide analogue NS5B polymerase inhibitor, one tablet of 250 mg twice daily), the co-formulation of grazoprevir with elbasvir (NS3/4 protease inhibitor/NS5A inhibitor, one tablet of 100/50 mg once daily) and the co-formulation of sofosbuvir with velpatasvir (nucleotide analogue NS5B polymerase inhibitor/NS5A inhibitor, one tablet of 400/100 mg once daily). In 2017, the co-formulation of glecaprevir with pibrentasvir (NS3/4 protease inhibitor/NS5A inhibitor, three tablets of 100/40 mg once daily) and the co-formulation of sofosbuvir with velpatasvir and voxilaprevir (nucleotide analogue NS5B polymerase inhibitor/NS5A inhibitor/ NS3/4 protease inhibitor, one tablet of 400/100/100 mg once daily) were also approved and started to be used in some countries. According to all international current guidelines, thalassaemia patients do not represent a special group for the current HCV treatment and can be treated with the same indications and regimens used for patients without haemoglobinopathies. However, in countries which still prioritize the use of DAAs according to the severity of liver disease, thalassaemia patients are often excluded from such prioritization and have access to DAAs therapy regardless of their fibrosis severity. Moreover, all guidelines recommend that thalassaemia patients should be preferentially treated not only with IFN-free but RBV-free DAAs regimens too. In a proper clinical trial, only a 12-week regimen of grazoprevir/ elbasvir has been evaluated and proven to be highly efficacious and well tolerated among patients with inherited blood disorders and HCV genotype 1 or 4 infection. In addition, different DAAs regimens have been reported to be safe an","PeriodicalId":22261,"journal":{"name":"Thalassemia Reports","volume":" ","pages":""},"PeriodicalIF":0.3,"publicationDate":"2018-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.4081/THAL.2018.7491","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48993945","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: 2

Challenges to management of pain in sickle cell disease 镰状细胞病疼痛管理面临的挑战

IF 0.3

Thalassemia Reports Pub Date : 2018-04-19 DOI: 10.4081/thal.2018.7482

J. Kanter

{"title":"Challenges to management of pain in sickle cell disease","authors":"J. Kanter","doi":"10.4081/thal.2018.7482","DOIUrl":"https://doi.org/10.4081/thal.2018.7482","url":null,"abstract":"Sickle Cell Disease (SCD) is one of the most common blood disorders in the world. Pain is the primary reason for which individuals with SCD interact with the healthcare system. Generally speaking, there are two types of SCD pain: vaso-occlusive pain (or sickle cell disease crisis) and chronic pain caused by an accumulation of organ and tissue damage over time. However, despite its frequency, we have limited understanding about what causes pain in sickle cell disease, how best to manage pain in SCD and (most importantly) how to prevent pain in SCD. For medical providers, pain is also an elusive target due to the difficulty in objectively measuring pain and the importance of relying on patient reported outcomes. To face the challenges in managing pain in SCD, we will review the current understanding of the pathophysiology of vaso-occlusion, the multiple dimensions of the pain experience, and the current methods of measuring and managing pain. We will also review new pharmacologic agents undergoing clinical trials in SCD that will help to prevent pain and improve outcomes in SCD. Challenges to management of pain in sickle cell disease Julie Kanter Pediatrics, Medical University of South Carolina, Charleston, SC, USA Correspondence: Julie Kanter, Pediatrics, Medical University of South Carolina, Charleston, SC, USA. This work is licensed under a Creative Commons Attribution 4.0 License (by-nc 4.0). ©Copyright J. Kanter, 2018 Licensee PAGEPress, Italy Thalassemia Reports 2018; 8:7482 doi:10.4081/thal.2018.7482 No n-c om me rci al us e o nly","PeriodicalId":22261,"journal":{"name":"Thalassemia Reports","volume":" ","pages":""},"PeriodicalIF":0.3,"publicationDate":"2018-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.4081/thal.2018.7482","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44812677","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: 1

Adherence to treatment: Doctor vs patient perspective 坚持治疗:医生与病人的观点

IF 0.3

Thalassemia Reports Pub Date : 2018-04-19 DOI: 10.4081/thal.2018.7484

Farrukh T Shah, G. Kaltsounis

引用次数: 0

Haemoglobinopathies care and cure: Have we reached the end? 血红蛋白病的护理和治疗：我们已经走到尽头了吗？

IF 0.3

Thalassemia Reports Pub Date : 2018-04-19 DOI: 10.4081/thal.2018.7470

J. Porter

引用次数: 0

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