Furthermore, we identified seven novel variants in PYGL gene. Conclusions Our study enriched the genotypic and phenotypic spectrum of GSD VI, and provided a new clue for management of GSD VI.Background Despite medical technology advancement, postpartum hemorrhage remains the top universal cause of maternal mortality. Factors note the inconsistency in recognition and timely treatment of women experiencing it, which suggests healthcare professionals' mentorship about postpartum hemorrhage. Methods The study recruited 141 nurses and midwives and used instruments adapted to knowledge and self-efficacy to assess the impact of mentorship on nurses' and midwives' knowledge and self-efficacy in managing postpartum hemorrhage. Results There was an increase in knowledge from 68% prior to mentorship up to 87% and self-efficacy from 6.9 to 9.5 average score out of 10. Knowledge and self-efficacy correlated moderately positive at pre-mentorship (r=0.214) and strongly positive at post-mentorship (r=0.585). The number of mentorship visits attended was associated with post-mentorship knowledge scores (r=0.539) and post-mentorship self-efficacy (r=0.623). Conclusions Mentorship about management of postpartum hemorrhage increases nurses' and midwives' knowledge and self-efficacy in managing postpartum hemorrhage.Objectives Novice nurses are struggling to exercise the critical thinking skill set needed to make competent clinical decisions in today's complex health care environment. This poses immense threats to the health and safety of patients. To address this alarming concern, many prominent organizations have called for a widespread overhaul of nursing curricula. Flavopiridol Methods A consistent theme among the recommendations for revising nursing curricula is the utilization of innovative curricula designs that focus on enhancing the critical thinking ability of nursing students, such as a concept-based curriculum. Results Planning and implementing a curriculum revision is an overwhelming undertaking. Conclusions Providing nurse educators with a guide on how to transition to a concept-based curriculum may help to facilitate a course revision.Progression-free survival (PFS), defined as the time from randomization to progression of disease or death, has been indicated as an endpoint to support accelerated approval of certain cancer drugs by the U.S. FDA. The standard Kaplan-Meier (KM) estimator of PFS, however, can result in significantly biased estimates. A major source for the bias results from the substitution of censored progression times with death times. Currently, to ameliorate this bias, several sensitivity analyses based on rather arbitrary definitions of PFS censoring are usually conducted. In addition, especially in the advanced cancer setting, patients with censored progression and observed death times have the potential to experience disease progression between those two times, in which case their true PFS time is actually between those times. In this paper, we present two alternative nonparametric estimators of PFS, which statistically incorporate survival data often available for those patients who are censored with respect to progression to obtain less biased estimates. Through extensive simulations, we show that these estimators greatly reduce the bias of the standard KM estimator and can also be utilized as alternative sensitivity analyses with a solid statistical basis in lieu of the arbitrarily defined analyses currently used. An example is also given using an ECOG-ACRIN Cancer Research Group advanced breast cancer study.Objective School children are exposed to various social, environmental, nutritional and cultural changes which will influence their lifestyle and can impose behavioral change. This study was formulated to assess the lifestyle factors influencing the academic performance among secondary school students in an urban area of south India. Materials and Methods A cross sectional study was conducted among secondary school students of grade 8, 9 and 10 in public and private schools of an urban area. Data was collected using a pre-designed, pretested, questionnaire. Demographic data and socio-economic status was assessed. Lifestyle assessment included the dietary habits, physical activity, tobacco and other substance use and anthropometric measurements. Academic performance was assessed by attendance, past annual examination grades. Results were expressed in percentages and analysis was done using Chi-square test and Fischer exact test. Results Study participants included were 613. Overall prevalence of tobacco use 14.0%. Skipping breakfast was associated with poor performance (p=0.002) among public school students. Private school students' academic performance was significantly associated with the BMI (p=0.03). Prevalence of underweight, overweight and obesity was 70.5, 5.0 and 5.5% respectively. Physical activity didn't show any significant influence on academic performance (p=0.69, 0.76). Conclusion Academic performance of secondary school students is influenced by various factors including, regularity of attendance, socio-economic status, skipping breakfast and consumption of tobacco and other substances. Study establishes the need for proper motivation and reinforcement of safe healthy lifestyle practices to achieve complete academic excellence. Emicizumab, a monoclonal antibody mimicking the function of factor (F) VIII in the activation of FX by FIXa, is widely used for prophylaxis in hemophilia patients with or without inhibitors to FVIII. Although it is administered at fixed dose, its measurement could be occasionally required. In principle, the emicizumab procoagulant effect could be assessed by the one-stage assay (OSA) currently used to measure FVIII. However, the OSA for FVIII presents with limitations. Furthermore, owing to its potent FVIII-like activity, emicizumab interferes with the measurement of the inhibitor to FVIII, which is often needed in patients on emicizumab. We prepared test samples by spiking a FVIII-deficient plasma with graded amounts of emicizumab. We modified the OSA for FVIII and tested plasma samples for emicizumab concentrations. Furthermore the chromogenic assay (CA) for FVIII with bovine reagents was used to assess for the FVIII inhibitor in patients on emicizumab. Slight modification of the OSA for FVIII (i.e., higher test plasma dilution and longer coagulometer acquisition time) made the regular OSA as a reliable laboratory tool to measure emicizumab concentration as shown by the identity of the regression (observed vs.