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IntroductionVon Willebrand factor (vWF) cleaving protease ADAMTS-13 has a key role for maintaining normal size of vWF. A deficiency or dysfunction of vWF cleaving protease is associated with ultra large vWF multimers and thrombotic microangiopathy. Patients with cancers have reduced levels of vWF cleaving protease. In this pilot study, we have evaluated whether or not deficiencies of ADAMTS-13 were present in myelodysplastic syndromes (MDS). Moreover, we assessed if a reduction in basal levels of ADAMTS-13 may play a role in the prognosis of MDS.Patients and MethodsWe measured and compared the levels of vWF cleaving protease ADAMTS-13 in 100 patients with MDS and 35 healthy controls. Patients were divided into 2 groups according to the International Prognostic Scoring System: group I consisting of 44 patients with low-risk MDS and group II of 56 patients with high-risk MDS. Patients with high-risk and low-risk MDS presented significantly lower levels of ADAMTS-13 than controls (P < .001 and P = .0177, respectively). High-risk patients had significantly lower levels of ADAMTS-13 when compared with the low-risk group (P < .001).ResultsWe found that reduced levels of ADAMTS-13 have a relationship with overall survival (P < .001). Statistical analysis showed that ADAMTS-13 correlates with cytogenetics (P < .001) and a tendency of slight correlation with platelet count and basal levels of ADAMTS-13 (R, 0.35; P value, 0.001). Moreover, we found that levels of ADAMTS-13 have correlation with response to treatment (P < .001).ConclusionsADAMTS-13 in MDS might represent a surrogate marker of prognosis, response to therapy, or disease progression. Further studies are needed.  相似文献   
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BackgroundThe positive effect of a father’s involvement in children’s upbringing is now recognised. However, research on fathers raising children with autism spectrum disorder (ASD) are still few. This study examines the relationship between the perception, fathers of children with ASD have of the importance of their role in the development of their children and the feelings (self-efficacy, caregiving burden, satisfaction) they express about their parenting experience.MethodSixty-three Swiss Italian fathers of children with ASD completed The Role of the Father Questionnaire (ROFQ), three sub-scales of the Caregiver Survey, a subtest of the Child Adjustment and Parent Efficacy Scale and a home-made questionnaire measuring Perceived Social Support.ResultsThe results from hierarchical multiple regression analyses show that the importance that fathers attach to the paternal role predicts positively their caregiving satisfaction and their feeling of self-efficacy. The children’s challenging behaviours predict positively the caregiving burden whereas the assessment of social support predicts it negatively.ConclusionsThe perception of the importance of the paternal role needs to be considered in the support offered to families with a child with ASD. A better understanding of the fathers’ feelings could be of value for the programmes.  相似文献   
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Introduction

The fast track / ultra-fast-track protocols are techniques used to optimise the patient care process and a quick recovery after cardiac surgery. They are one of the mainstays of efficient practice. With their use, the length of hospital and intensive care unit (ICU) stays are reduced, with a direct impact on costs and the quality of the health service.

Objective

To compare the length of stay in the ICU, length of hospital stay, and post-operative mortality in ultra-fast-track extubated (uFTE) patients and those with conventional extubation (CE) after cardiac surgery.

Methods

Longitudinal, analytical, retrospective study was conducted, with the period between the time of surgery and discharge being included as the study period.

Results

A total of 396 patients older than 18 years who required cardiac surgery were included, of whom 207 patients had (uFTE) and 189 had CE. Although the groups were not comparable due to the statistical differences found, when performing the multivariate adjustment, uFTE maintained its statistical independence and was associated with lower cardiovascular morbidity, such as myocardial ischaemia (95% CI: 0.37-0.86; P = .01) and lower post-surgical vasopressor requirement (95% CI: 0.18-0.49; P < .01). No significant differences were found in the length of hospital stay, ICU stay, or post-operative mortality in the ICU.

Conclusion

Implementing the uFTE strategy, decreases cardiovascular morbidity and vasopressor requirement. The change to uFTE should be accompanied by changes in models and practices in patient recovery to standardised protocols. This study shows that uFTE did not reduce the length of ICU stay, hospital stay, or mortality.  相似文献   
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Worldwide, researchers routinely study children indirectly through adults who act as proxies for such children. The call for researchers to rather study children directly and adopt less intrusive child-friendly methodologies has become louder. The draw-and-write technique is regarded as a less intrusive child-friendly method of collecting data that can be used for this purpose. This paper reports the findings of a qualitative study that sought to establish the feasibility of the draw-and-write technique in exploring the resilience of children orphaned by AIDS. This study involved a convenience sample of 23 IsiXhosa-speaking orphaned children aged from 13 to 17. The participants were in grades 6 to 10 and they resided in two child and youth care centres in the Eastern Cape province of South Africa. As part of the draw-and-write technique the participants were asked to make drawings of what enabled them to cope with their lives and to write short narratives in which they explained their drawings. Inductive content analysis was used to analyse the data and this process yielded two main themes, namely: personal protective resources and socio-ecological protective resources. The findings show that complex combinations of personal and socio-ecological resilience resources enabled the resilience of the orphaned children. The findings challenge researchers not to be oblivious to alternative child-friendly methods of research such as the draw-and-write technique when young people are the unit of analysis. The findings of this study have implications for research as well as practice in the field of education and psychology.  相似文献   
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