Thalassemia is one of the most common causes of inherited anemia worldwide. While significant advances has been made in clinical management of thalassemia patients over the past few decades, our knowledge on the factors affecting the quality of life of thalassemia patients is limited. The "IntercontinThal Study" is a collaborative effort to study the quality of life (QoL) and quality of care of thalassemia patients in populations across diverse social and health care systems. Data presented here are from the three participating centers in Canada, Lebanon and Iran. We have gathered study data through: a) QoL questionnaire SF-36 completed by patients, b) a specifically designed and validated questionnaire completed by patients which addressed patient's social status (marriage/relationship status, education, employment status, and access to social support and health care), and c) review of the patients' charts using a data collection form. This form included: patients' demographics, specifics of transfusion therapy and iron chelation, thalassemia-related and other clinical complications (endocrinopathies, bone disease, cardiac disease, hemolysis-related complications, etc.), tissue iron content (liver and cardiac) and/or serum ferritin within the past three years, and splenectomy status. All study questionnaires were translated into Persian (for Iranian patients) and Arabic (for Lebanese patients). Due to the variety of the clinical complications, all clinical complications were aggregated together for statistical analysis. We used univariate and multivariate regression analysis to study the association of predictors and patients' QoL Mental Component Summary (MCS) Score. Ninety seven patients [46 female, 59 transfusion-dependent beta-thalassemia (TDT) and 38 non-transfusion-dependent beta-thalassemia (NTDT)] were included in the analysis. All patients were older than 18 years of age (Mean 32 years, SD: 7 years). In univariate analysis age, access to social support and health care, marriage status, liver iron concentration and ferritin (strongly correlated with each other), and disease-related complications were found to be predictor of QoL MCS scores. In NTDT patients, splenectomy and lower baseline hemoglobin were also significantly associated with reduced QoL. In multivariate analysis, ferritin and age (and clinical complications in TDT patients) were found to independently be associated with reduced QoL. LIC was not found to be an independent factor likely due to the fewer number of patients who had recent LIC assessments. Of interest, patients with NTDT reported better QoL at younger age compared to TDT patients but there was a trend toward worse QoL at older age. Our results provide a better understanding of the factors that affect the QoL of thalassemia patients and highlights the importance of management of body iron in both TDT and specially in NTDT patients. In addition, it confirms the notion that while NTDT patients may not require regular transfusions based on conventional criteria, they may experience significant reduction in QoL especially at older ages. Further efforts to address the health and QoL of NTDT patients are required to improve the outcomes of this often neglected condition. (Funded by a research grant from the Thalassemia Foundation of Canada) Disclosures Taher: Celgene: Research Funding; Novartis: Honoraria, Research Funding.
We suggest that the reaction e +e -+T+T-on a +-like resonance may provide a method for testing theories of the weak neutral current. The method is based on the observation that the T decay products from this reaction will have a forward-backward asymmetry induced by the polarization sensitivity of the T decays. We point out that, to the extent that the +-like resonance stands above background, this reaction provides not only an improved counting rate but also relief from the radiative asymmetries that trouble proposals to measure neutral-current asymmetries in e +e -3
Objective Skin extracellular matrix (ECM) is a dense and well‐organized structure produced by fibroblasts. This ECM transduces environmental mechano‐signals to cell nucleus through the integrin–actin complex, thus triggering ECM protein syntheses. The aim of this study was to discover a novel peptide, structurally related to dermal matrikines, that promotes syntheses of ECM components. Methods and results Screening tests with 120 peptides were carried out by using normal dermal human fibroblasts (HF). As a result, one candidate of interest was isolated, the N‐Prolyl Palmitoyl Tripeptide‐56 Acetate (PP56), which increases collagen and fibronectin productions at gene and/or protein levels. Using liquid chromatography–tandem mass spectrometry (LC‐MS/MS), a recent and innovative analytical technology, in addition to more traditional techniques, it was showed that two metabolic pathways were significantly modulated: one for collagen production and one for actin. Moreover, this peptide up‐regulated the transcription of Forkhead Box O (FOXO) and sestrin messenger RNAs (mRNA), leading to production of proteins involved into longevity and more recently in collagen production. Results Results indicated that this peptide is a potential candidate to improve ECM density and organization in a new way.
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