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Bruton's tyrosine kinase (BTK) is a central mediator of B-cell receptor (BCR) signaling essential for normal B-cell development. Ibrutinib is an oral BTK inhibitor that induces apoptosis and inhibits migration and adhesion of malignant B-cells. Updated results of this international, multicenter, phase 2 study of single agent ibrutinib in relapsed or refractory MCL will be presented.Ibrutinib 560 mg PO QD was administered continuously until disease progression. Tumor response was assessed every 2 cycles (one cycle = 28 days). The study enrolled 115 patients (65 bortezomib-naïve, 50 bortezomib-exposed); 111 patients were treated; 110 were evaluable for response. Baseline characteristics included: median age 68 years, time since diagnosis 42 months, number of prior treatments 3; bulky disease (>10 cm) 13%, prior stem cell transplant 10%, high risk MIPI 49%.Median time on treatment was 9.2 months; 53% of patients remain on therapy. Median PFS was 13.9 months and DOR has not yet been reached. Responses increased with longer treatment: comparing to previous data described at ASH 2011, the CR rate increased from 16% to 39%, and the ORR increased from 69% to 75%.  相似文献   
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IntroductionThe aim of the study was to evaluate the correlation between the nutritional status of patients with anorexia nervosa (AN) and levels of vaspin (VASP), neuropeptide B (NPB), neuropeptide W (NPW) and total antioxidant status (TAS).Material and methodsNinety serum samples collected from 30 teenage female patients during the acute stage of AN and 30 healthy persons (CONTR) were subjected to biochemical analysis; patients with AN were examined at the beginning of the study (AN-I) and after hospitalization (AN-II), as a result of which partial stabilization of anthropometric measurements was achieved (an increase of body mass index (BMI) by 3.5 kg/m2).ResultsVaspin levels dropped at the end of the hospitalization (compared to AN-I, p < 0.05), achieving values comparable to the CONTR; moreover there was a positive correlation between VASP level and the achieved body weight in AN-II (p < 0.05). Positive correlations were also noted with regard to VASP vs. NPB in AN-I (p < 0.02) (and AN-II, p < 0.013), as well as in the case of VASP vs. NPW in the same groups (p < 0.02 and p < 0.015, respectively). NPB concentration was higher in AN-I (p < 0.05) and AN-II (p < 0.018) than in CONTR, whereas there were no differences (p > 0.05) with regard to levels of VASP, NPW, or TAS.ConclusionsThe high level of NPB despite treatment and normalization of VASP level may suggest that there are chronic neuroendocrine disorders at play in anorexia nervosa.  相似文献   
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IntroductionImmunoglobulin A nephropathy (IgAN) may lead to end stage renal disease and severely affect patient functioning and wellbeing. The aim of the study was to evaluate health-related quality of life (HRQoL) in children and adolescents with IgAN, and compare HRQoL in relation to the disease course, social status and psychological factors, such as expressing anger and perceived personal competence.Material and methodsThe multicentre cross-sectional study included 51 patients ≥ 8 years from 7 paediatric nephrology centres in Poland. Psychometric analysis was performed using the Kidscreen-52 questionnaire to evaluate HRQoL, the Anger Expression Scale to evaluate the severity of anger and the Personal Competence Scale to measure general perception of personal competence.ResultsMean age of patients was 14.54 ±3.69 years; duration since the diagnosis of IgAN was 4.98 ±3.9 years. Patients with IgAN rated their psychological wellbeing as significantly worse compared to healthy peers (p < 0.05). The presence of proteinuria was associated with significantly worse physical wellbeing (58.72 ±18.45 vs. 74.44 ±22.97; p < 0.05). Current therapy (steroids/immunosuppressive drugs) had no effect on HRQoL in the study group. Perceived personal competence was rated high by 49% of children in the study group. Children with IgAN were characterized by lower intensity of expressed anger (p < 0.001) and significantly higher intensity of suppressed anger (p < 0.01) compared to reference ranges. Severity of expressed anger correlated positively with the parent relations and school environment dimensions of HRQoL.ConclusionsWe found lower HRQoL in regard to physical and psychological wellbeing in a group of Polish children with IgAN compared to healthy peers. HRQoL should be monitored in this patient group.  相似文献   
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IntroductionPrimary arthroplasty of the hip joint is currently one of the most commonly performed procedures in orthopedics. In Poland we are observing significant changes in the age structure. With the prolonged life more and more elderly patients require musculoskeletal surgery to maintain comfortable and painless mobility. Reducing the duration of the procedure reduces the costs of anesthesiology, surgical and instrument teams, as well as the operating room technical team. The aim of the study was to compare the time required to perform hip joint arthroplasty by the direct anterior approach (DAA) with the postero-lateral approach (PLA) in our hospital.Material and methodsA retrospective analysis of 559 total and bipolar cemented and cementless hip replacement procedures based on two operative approaches – the minimally invasive DAA over the course of 2 years, and the standard PLA over the course of 3 years – was performed.ResultsStatistically significant differences were observed between the approaches used for cementless total arthroplasty with regard to the mean treatment times: 51.9 min for the 272 DAA cases, and 78.3 min for the 190 PLA cases (p < 0.0001). For the cementless hemi-arthroplasty procedure, the mean treatment times were 46.9 min in 36 patients for DAA, and 48.2 min for 61 patients for PLA (p = 0.57).ConclusionsMinimally invasive DAA significantly shortens the time of the procedure in elderly patients compared to PLA. Further study is needed to analyze other aspects of those two approaches.  相似文献   
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IntroductionThe misbalance between a family of inhibitor of apoptosis proteins (IAP), regulated by the nuclear factor kappa B (NF-κB) and their natural antagonist second mitochondrial-derived activator of caspases/direct IAP binding protein with low pI (Smac/DIABLO) are important to biology of acute myeloid leukemia (AML).Material and methodsThe aim of the study was to assess NF-κB and Smac/DIABLO proteins expression in blasts of 109 newly diagnosed AML patients using the multicolor flow cytometry and evaluate their influence on AML patients outcome.ResultsExpression of NF-κB and of Smac/DIABLO proteins were found in 95% and 98% of the patients, respectively. A negative correlation between Smac/DIABLO and NF-κB was observed. Age < 60 years old as well as higher Smac/DIABLO expression were associated with a higher probability of complete response achievement in the multivariate analysis. Longer overall survival (OS) in the univariate and multivariate analyses was influenced by age < 60 years old, a favorable or intermediate-risk karyotype and high Smac/DIABLO expression. Additionally, in the survival analysis of the subgroups, the patients aged < 60 years old, with high Smac/DIABLO expression, lower NF-κB expression and < 50% of bone marrow blasts who were treated with standard treatment had better OS.ConclusionsLower NF-κB and higher Smac/DIABLO expression may influence AML patients outcome.  相似文献   
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Over the last decade, great progress has been made in the field of organic electronics. Advancements in organic syntheses as well as in device engineering enabled preparation of polymer solar cells with power conversion efficiency (PCE) exceeding 8%–9%. In search for new polymers suitable for photovoltaic applications, push–pull polymers containing thieno[3,4‐c]pyrrole‐4,6‐dione (TPD) motif as an electron deficient (pull) unit emerged as very promising candidates. This Trend Article summarizes research on TPD‐based polymers with a special emphasis on the structure–property relationships.  相似文献   
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