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الوصف: Sickle cell disease patients often need regular blood transfusions to improve both the quality of life and survival from the veno-occlusive complications of the disease. Deferasirox, a convenient long acting oral agent, has recently been introduced in clinical practice with promising efficacy. This study aims to evaluate the association of liver stiffness and possible fibrosis with iron deposition and confirm the use of elastography as a validated test of responding to chelation with low cost and easy access. 15 patients with sickle cell disease and systemic or occasional transfusions were evaluated with MRI, transient elastography and biochemistry, for liver iron(LIC) and liver stiffness(LSM) before onset and one year after taking Deferasirox. All patients completed the study. Our results showed improvement in hepatic iron and hepatic stiffness after chelation therapy; Furthermore ALT, AST, LDH and ferritin levels have improved after 12 months of therapy with deferasirox. During the study no serious adverse events were encountered indicating the safety of the drug. Transient liver elastography findings correlate with serum ferritin and LIC in patients with sickle cell disease and it is a useful tool for assessing the response of liver iron chelation therapy. © 2018 Slovensko Kemijsko Drustvo. All Rights Reserved.
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=od______2127::16708019fa970d22c87b705beefa45a1Test
https://pergamos.lib.uoa.gr/uoa/dl/object/uoadl:3107824Test -
2دورية أكاديمية
المؤلفون: Sinakos E., Kountouras D., Koskinas J., Zachou K., Karatapanis S., Triantos C., Vassiliadis T., Goulis I., Kourakli A., Vlachaki E., Toli B., Tampaki M., Arvaniti P., Tsiaoussis G., Bellou A., Kattamis A., Maragkos K., Petropoulou F., Dalekos G.N., Akriviadis E., Papatheodoridis G.V.
المصدر: British Journal of Haematology ; https://www.scopus.com/inward/record.uri?eid=2-s2.0-85018745432&doi=10.1111%2fbjh.14640&partnerID=40&md5=6314541aca3316573098063af32c40edTest
مصطلحات موضوعية: daclatasvir, dasabuvir, deferiprone, deferoxamine, ledipasvir plus sofosbuvir, ombitasvir plus paritaprevir plus ritonavir, ribavirin, simeprevir, sofosbuvir, antivirus agent, BMS-790052, imidazole derivative, adult, antiviral therapy, Article, blood transfusion, chelation therapy, chronic hepatitis C, clinical article, combination drug therapy, drug efficacy, drug safety, drug tolerability, female, Hepatitis C virus genotype 1, Hepatitis C virus genotype 2, Hepatitis C virus genotype 3, Hepatitis C virus genotype 4, human, liver cirrhosis
الوصف: Interferon-based regimens for chronic hepatitis C (CHC) were often deferred in patients with β-thalasaemia major (β-TM) due to poor efficacy and tolerance. Current guidelines recommend direct-acting antivirals (DAAs) for these patients. The aim of this study was to assess the safety and efficacy of DAAs in patients with β-TM and advanced liver disease due to CHC. Patients were recruited from eight liver units in Greece. The stage of liver disease was assessed using transient elastography and/or liver histology. Five regimens were used: sofosbuvir (SOF) + ribavirin (RBV); SOF + simeprevir ± RBV; SOF + daclatasvir ± RBV; ledipasvir/SOF ± RBV and ombitasvir/paritaprevir-ritonavir + dasabuvir ± RBV. Sixty-one patients (median age 43 years) were included. The majority of patients was previously treated for hepatitis C (75%) and had cirrhosis (79%). Viral genotype distribution was: G1a: n = 10 (16%); G1b: n = 22 (36%); G2: n = 2 (3%); G3: n = 14 (23%); G4: n = 13 (22%). The predominant chelation therapy was a combination of deferoxamine and deferiprone (35%). Overall sustained virological response rates were 90%. All treatment regimens were well tolerated and no major adverse events or drug-drug interactions were observed. Approximately half of the patients who received RBV (7/16, 44%) had increased needs for blood transfusion. Treatment of CHC with DAAs in patients with β-TM and advanced liver disease was highly effective and safe. © 2017 John Wiley & Sons Ltd
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الوصف: Interferon-based regimens for chronic hepatitis C (CHC) were often deferred in patients with β-thalasaemia major (β-TM) due to poor efficacy and tolerance. Current guidelines recommend direct-acting antivirals (DAAs) for these patients. The aim of this study was to assess the safety and efficacy of DAAs in patients with β-TM and advanced liver disease due to CHC. Patients were recruited from eight liver units in Greece. The stage of liver disease was assessed using transient elastography and/or liver histology. Five regimens were used: sofosbuvir (SOF) + ribavirin (RBV); SOF + simeprevir ± RBV; SOF + daclatasvir ± RBV; ledipasvir/SOF ± RBV and ombitasvir/paritaprevir-ritonavir + dasabuvir ± RBV. Sixty-one patients (median age 43 years) were included. The majority of patients was previously treated for hepatitis C (75%) and had cirrhosis (79%). Viral genotype distribution was: G1a: n = 10 (16%); G1b: n = 22 (36%); G2: n = 2 (3%); G3: n = 14 (23%); G4: n = 13 (22%). The predominant chelation therapy was a combination of deferoxamine and deferiprone (35%). Overall sustained virological response rates were 90%. All treatment regimens were well tolerated and no major adverse events or drug-drug interactions were observed. Approximately half of the patients who received RBV (7/16, 44%) had increased needs for blood transfusion. Treatment of CHC with DAAs in patients with β-TM and advanced liver disease was highly effective and safe. © 2017 John Wiley & Sons Ltd
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=od______2127::316ae958786f3b151229b4867761c669Test
https://pergamos.lib.uoa.gr/uoa/dl/object/uoadl:3108562Test -
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مصطلحات موضوعية: genetic structures, sense organs, eye diseases
الوصف: Purpose: The purpose of this study was to assess the role of various diagnostic tests in early detection of retinal changes in β-thalassemia major patients. Methods: Thirty-eight visually asymptomatic β-thalassemia major patients receiving regular blood transfusions and iron-chelation therapy with deferoxamine (group A, n = 13), deferasirox (group B, n = 11) or deferoxamine with deferiprone (group C, n = 14) and fourteen age- and sex- matched healthy individuals were included in the study. All participants underwent ophthalmoscopy, full-field electroretinography (ERG), visual evoked potentials (VEP), multifocal electroretinography (mfERG), fundus autofluorescence (FAF) imaging and optical coherence tomography (OCT) scans. Results: Retinal pigment epithelium changes were present in two cases. Scotopic ERG demonstrated decreased a-wave amplitude in groups A, B and C (p = 0.03, p = 0.002 and p = 0.002, respectively) and decreased b-wave amplitude in groups B and C (p = 0.002 and p = 0.01, respectively) compared to controls. Photopic ERG showed delayed b-wave latency in groups A and C (p = 0.03 and p = 0.03, respectively) ERG maximal combined response and VEP response did not differ between groups. MfERG showed reduced retinal response density in ring 1 in groups A, B, C (p < 0.001, p < 0.001, p = 0.001, respectively) and ring 2 in group B (p = 0.02) and delayed latency in ring 5 in groups A and B (p = 0.04 and p = 0.04, respectively). Abnormal FAF images appeared in three cases and OCT abnormalities in one case, whereas no changes were observed in controls (p = 0.55 and p = 1.00, respectively). Conclusions: Full-field ERG and mfERG are more sensitive tools for detecting early retinal changes in β-thalassemia patients compared with ophthalmoscopy, VEP, FAF imaging and OCT scans. © 2017, Springer-Verlag Berlin Heidelberg.
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=od______2127::0c23f45e0afb3a91bb0f267c13e1e901Test
https://pergamos.lib.uoa.gr/uoa/dl/object/uoadl:2998294Test
