Assessment of Immature Platelet Fraction in the Diagnosis of Wiskott-Aldrich Syndrome.

Children with Wiskott-Aldrich syndrome (WAS) are often first diagnosed with immune thrombocytopenia (ITP), potentially leading to both inappropriate treatment and the delay of life-saving definitive therapy. WAS is traditionally differentiated from ITP based on the small size of WAS platelets. In practice, microthrombocytopenia is often not present or not appreciated in children with WAS. To develop an alternative method of differentiating WAS from ITP, we retrospectively reviewed all complete blood counts and measurements of immature platelet fraction (IPF) in 18 subjects with WAS and 38 subjects with a diagnosis of ITP treated at our hospital. Examination of peripheral blood smears revealed a wide range of platelet sizes in subjects with WAS. Mean platelet volume (MPV) was not reported in 26% of subjects, and subjects in whom MPV was not reported had lower platelet counts than did subjects in whom MPV was reported. Subjects with WAS had a lower IPF than would be expected for their level of thrombocytopenia, and the IPF in subjects with WAS was significantly lower than in subjects with a diagnosis of ITP. Using logistic regression, we developed and validated a rule based on platelet count and IPF that was more sensitive for the diagnosis of WAS than was the MPV, and was applicable regardless of the level of platelets or the availability of the MPV. Our observations demonstrate that MPV is often not available in severely thrombocytopenic subjects, which may hinder the diagnosis of WAS. In addition, subjects with WAS have a low IPF, which is consistent with the notion that a platelet production defect contributes to the thrombocytopenia of WAS. Knowledge of this detail of WAS pathophysiology allows to differentiate WAS from ITP with increased sensitivity, thereby allowing a physician to spare children with WAS from inappropriate treatment, and make definitive therapy available in a timely manner.

Calidad de vida y cáncer de próstata: un programa progresivo de fuerza-resistencia = Quality of life and prostate cancer: a progressive resistance strength program

This article presents the design and implementation of a progressive resistance strength program adapted to prostate cancer. The initial model corresponds to the guide of the American College Sports Medicine Position Stand (ACSM, 2009). This program includes the most habitual symptoms related to the illness and its treatments. The study design is quasi-experimental. The sample is 33 subjects in treatment phase. Study variables are tumour classification TNM, anthropometric measures, resistance strength, hypertension, fatigue, incontinence, pain and quality of life. After 24 weeks a significant improvement on resistance strength capacity is observed. This result is more consistent in lower extremities. Also improves hypertension, urinary incontinence, pain and quality of life. As conclusion, the improvement of the quality of life is mediated by the functional and physical capacity of the ill person

Role of immune and angiogenic markers in the pathology of endometriosis

Background: Endometriosis is an estrogen-dependent, pro-inflammatory, pro-angiogenic condition that affects 5 to 10% of women of reproductive age. Its defining feature is the presence of endometrium-like tissue in sites outside the uterine cavity, primarily on the pelvic peritoneum and ovaries. The main clinical features are chronic pain, pain during intercourse and infertility. In patients with endometriosis, inflammatory and immune responses, angiogenesis and apoptosis are altered in favour of the survival and replenishment of endometriotic tissue. These basic pathological processes depend on the excessive formation of estrogen and prostaglandins. Recently, new cellular and molecular mechanisms for the resolution of inflammation have been discovered, revealing key roles for lipid mediators such as lipoxins, resolvins and protectins. It is possible that disequilibrium in the expression of these molecules exists in endometriosis. Objective: To compare the expression of two proteins involved in the synthe sis and in the function of lipid mediators; the Arachidonate 15-lipoxygenase (ALOXI5), implicated in the synthesis of lipoxins A4 and B4 and the Formyl peptide receptor 1 (FPRLI), the specific receptor for Lipoxin A4 and B4, between women who suffer from endometriosis and a control group. We wish to demonstrate the cellular localisation of these two molecules and to investigate if their expression is alteted in this pathology. Methods and Materials Using immunohistochemistry we will compare ALOXI5 and FPRLI staining, in endometrium, normal peritoneum and endometriotic lesions. The samples are being collected in the department of Gynaecology and Obstetrics at the Centre Hospitalier Universitaire Lausanne (CHUV). Women attending the department for laparoscopic investigation of pain/infertility, suspected endometriosis or for a hysterectomy, are invited to participate. Approval of the ethics committee (Commission d'Ethique de la recherché clinique) was obtained in March 2009. Clinical samples will only be obtained from subjects having consented. Expected results and interpretation: No published studies investigating the expression of these two molecules in endometriotic lesions exist. A better understanding of the mechanisms underlying this disease will result in the development of new medical therapies and new diagnostic tests, with the aim of ameliorating the quality of life of endometriosis patients.

Paediatric end-of-life care needs in Switzerland: current practices, and perspectives from parents and professionals. A study protocol.

AIM: To present a protocol for a multi-phase study about the current practice of end-of-life care in paediatric settings in Switzerland. BACKGROUND: In Switzerland, paediatric palliative care is usually provided by teams, who may not necessarily have specific training. There is a lack of systematic data about specific aspects of care at the end of a child's life, such as symptom management, involvement of parents in decision-making and family-centred care and experiences and needs of parents, and perspectives of healthcare professionals. DESIGN: This retrospective nationwide multicentre study, Paediatric End-of-LIfe CAre Needs in Switzerland (PELICAN), combines quantitative and qualitative methods of enquiry. METHODS: The PELICAN study consists of three observational parts, PELICAN I describes practices of end-of-life care (defined as the last 4 weeks of life) in the hospital and home care setting of children (0-18 years) who died in the years 2011-2012 due to a cardiac, neurological or oncological disease, or who died in the neonatal period. PELICAN II assesses the experiences and needs of parents during the end-of-life phase of their child. PELICAN III focuses on healthcare professionals and explores their perspectives concerning the provision of end-of-life care. CONCLUSION: This first study across Switzerland will provide comprehensive insight into the current end-of-life care in children with distinct diagnoses and the perspectives of affected parents and health professionals. The results may facilitate the development and implementation of programmes for end-of-life care in children across Switzerland, building on real experiences and needs. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT01983852.

Phylogenomics Controlling for Base Compositional Bias Reveals a Single Origin of Eusociality in Corbiculate Bees.

As increasingly large molecular data sets are collected for phylogenomics, the conflicting phylogenetic signal among gene trees poses challenges to resolve some difficult nodes of the Tree of Life. Among these nodes, the phylogenetic position of the honey bees (Apini) within the corbiculate bee group remains controversial, despite its considerable importance for understanding the emergence and maintenance of eusociality. Here, we show that this controversy stems in part from pervasive phylogenetic conflicts among GC-rich gene trees. GC-rich genes typically have a high nucleotidic heterogeneity among species, which can induce topological conflicts among gene trees. When retaining only the most GC-homogeneous genes or using a nonhomogeneous model of sequence evolution, our analyses reveal a monophyletic group of the three lineages with a eusocial lifestyle (honey bees, bumble bees, and stingless bees). These phylogenetic relationships strongly suggest a single origin of eusociality in the corbiculate bees, with no reversal to solitary living in this group. To accurately reconstruct other important evolutionary steps across the Tree of Life, we suggest removing GC-rich and GC-heterogeneous genes from large phylogenomic data sets. Interpreted as a consequence of genome-wide variations in recombination rates, this GC effect can affect all taxa featuring GC-biased gene conversion, which is common in eukaryotes.

Intervals between response choices on a single-item measure of quality of life.

BACKGROUND: A single overall rating of quality of life (QoL) is a sensitive method that is often used in population surveys. However, the exact meaning of response choices is unclear. In particular, uneven spacing may affect the way QoL ratings should be analyzed and interpreted. This study aimed to determine the intervals between response choices to a single-item QoL assessment. METHODS: A secondary analysis was conducted on data from the Lc65+ cohort study and two additional, population-based, stratified random samples of older people (N = 5,300). Overall QoL was rated as excellent, very good, good, fair or poor. A QoL score (range 0-100) was derived from participants' answers to a 28-item QoL assessment tool. A transformed QoL score ranging from 1 (poor) to 5 (excellent) was calculated. The same procedure was repeated to compute seven domain-specific QoL subscores (Feeling of safety; Health and mobility; Autonomy; Close entourage; Material resources; Esteem and recognition; Social and cultural life). RESULTS: Mean (95 % confidence intervals) QoL scores were 96.23 (95.81-96.65) for excellent, 93.09 (92.74-93.45) for very good, 81.45 (80.63-82.27) for good, 65.44 (62.67-68.20) for fair and 54.52 (45.31-63.73) for poor overall QoL, corresponding to transformed QoL scores of respectively 5.00, 4.70, 3.58, 2.05, and 1.00. Ordinality of the categories excellent to poor was preserved in all seven QoL subscores. CONCLUSIONS: The excellent-to-poor rating scale provides an ordinal measure of overall QoL. The intervals between response choices are unequal, but an interval scale can be obtained after adequate recoding of excellent, very good, good, fair and poor.

Trajectory and predictors of quality of life in first episode psychotic mania.

BACKGROUND: Little is known about the trajectory of quality of life (QoL) following a first episode of psychotic mania in bipolar disorder (BD). This 18-month longitudinal study investigated the trajectory of QoL, and the influence of premorbid adjustment and symptoms on 18-month QoL in a cohort of young people experiencing a first episode of psychotic mania. METHODS: As part of an overarching clinical trial, at baseline, sixty participants presenting with a first episode of psychotic mania (BD Type 1 - DSM-IV) completed symptomatic and functional assessments in addition to the Premorbid Adjustment Scale - General Subscale. Symptom measures were repeated at 18-month follow up. QoL was rated using the Quality of Life Scale (QLS) at designated time points. RESULTS: Mean QLS scores at initial measurement (8 weeks) were 61% of the maximum possible score, increasing significantly to 70% at 12 months, and 71.2% at 18-month follow-up. Premorbid adjustment and 18-month depressive symptoms were significantly associated with QoL at 18-month follow-up. LIMITATIONS: Study limitations include the small sample size, inclusion of participants with psychotic mania only, use of measures originally designed for use with schizophrenia spectrum disorders, and lack of premorbid or baseline measurement of QoL. CONCLUSIONS: Results suggest that QoL can be maintained early in BD, and reinforce the importance of assertively treating depressive symptoms throughout the course of this disorder. The emergence of a link between premorbid adjustment and poorer QoL in this cohort highlights the importance of assessing facets of adjustment when planning psychological interventions.

Indicadores sociales y psicosociales de calidad de vida de las personas mayores en un municipio = Social and psychosocial quality of life indicators of older persons in a municipality

A research about the quality of life of the older persons of a municipality starting from the analysis of the perceptions, evaluations and expectations related to concrete spheres of their lives (familiar characteristics, housing, health, nearby environment, activities, needs and dependencies and persons helping them to satisfy needs) is presented. Answers to 1988 questionnaires obtained from two representative samples of older per - sons living in private homes in the city have been analysed: persons over 65 years old and a specific one composed by a sub-sample of the general one, with persons over 75 years living alone. Data shaping contextual indicators related to housing have been analysed. The auto - nomy to drive and the evaluation of on’s health seem to be positive indicators to take in account while sutying quality of life at these ages. Sentinel indicators of the physical and relational conditions in the family and indicators of dependency are analysed as well. The evaluations of the older persons about their own life conditions are also analysed through psychosocial indicators connected to housing, nearby environmenent, their activities andincomes. The results obtained are applicable in order to improve the decision making process in social intervention programmes developed in the field of ageing aiming to take into account their perspectives

Improving Quality of Life of Patients With Schizophrenia In Acute Psychiatric Wards

The overall goal of this study was to identify means by which the quality of life (QoL) of patients with schizophrenia could be improved in acute psychiatric wards. First, subjective QoL of patients (n=35) was explored. Second, two different QoL instruments (EuroQoL-5D, EQ-5D; Quality of Life Enjoyment and Satisfaction Questionnaire Short Form, Q-LES-Q SF) were examined. Third, patients’ (n=35) and nurses’ (n=29) perceptions of nursing interventions to support patients’ QoL were examined. Fourth, the effect of three different patient education methods on patients’ QoL (n=311) was compared. The data were collected during the period 2005-2007. Patients named health, family, leisure activities, work or study, and social relationships most frequently as their important QoL areas. It emerged that patients’ QoL was impaired. Examination of two QoL instruments showed that the EQ-5D has moderate and the Q-LES-Q SF good internal consistency. Moreover, both instruments proved to be reasonably valid and feasible for use with patients with schizophrenia. Altogether six nursing interventions which nurses use to support patients’ QoL, and which should be further developed were identified from nurses’ descriptions: interventions related to care planning, empowering interventions, social interventions, activating interventions, security interventions, and interventions to support physical health. Evaluation of different patient education methods showed that patients’ QoL improved significantly during follow-up. No significant differences between groups were found. In light of the findings it is recommended to assess QoL of patients with schizophrenia as a basis for care planning and care evaluation in clinical settings. Valid and feasible instruments should be used in this assessment. Moreover, it is recommend that nursing interventions should be further developed to better improve patients’ QoL.

Evaluation of quality of life before and after videothoracoscopic simpathectomy for primary hyperhidrosis

Objective: To assess quality of life before and after thoracoscopic sympathectomy for treatment of primary hyperhidrosis.Methods: we conducted an observational, analytical, cross-sectional and quantitative study. We evaluated patients undergoing thoracoscopic sympathectomy for primary axillary hyperhidrosis, primary palmar hyperhidrosis, and axillary hyperhidrosis associated with palmar one. We applied a questionnaire on quality of life related to hyperhidrosis before and after the operation.Results: The questionnaire was administered to 51 patients with a mean age of 32.4 years, 45 women and six men. The average quality of life related to hyperhidrosis in a score of 0-100 before sympathectomy was 34.6 and after the operation it was 77.1. Compensatory hyperhidrosis occurred in 84.3% of patients.Conclusion: thoracoscopic sympathectomy improves the quality of life of patients with primary hyperhidrosis, with results supported over time. Compensatory hyperhidrosis occurred in most patients, but did not significantly influence the improved quality of life.

Importance of considering food waste in the development of sustainable food packaging systems

Meeting the needs of both present and future generations forms the foundation of sustainable development. Concern about food demand is increasing alongside the continuously growing population. In the pursuit of food security preventing food waste is one solution avoiding the negative environmental impacts that result from producing food unnecessarily. Packages offer one answer to preventing food waste, as they 1) preserve and protect food, 2) introduce the user to the correct way to handle and use the food and package and 3) allow the user to consume the food in its entirety. This thesis aims to enhance the sustainability of food packages by giving special emphasis to preventing food waste. The focus of this thesis is to assist the packaging designer in being able to take into account the requirements for the sustainability of food packages and to be able to integrate these requirements into the product development process. In addition, life cycle methods that can be used as a tool in the packaging design process or in assessing the sustainability of finished food-packaging combinations are evaluated. The methods of life cycle costing (LCC) and life cycle working environment (LCWE) are briefly discussed. The method of life cycle assessment (LCA) is examined more thoroughly through the lens of the literature review of food-package LCA case studies published in the 21st century in three relevant journals. Based on this review and on experiences learned from conducting LCAs, recommendations are given as to how the LCA practitioner should conduct a food packaging study to make most of the results. Two case studies are presented in this thesis. The first case study relates the results of a life cycle assessment conducted for three food items (cold cut (ham), sliced dark bread (rye) and Soygurt drink) and the alternative packaging options of each. Results of this study show that the packaging constitutes only 1–12 % of the total environmental impacts of the food-packaging combination. The greatest effect is derived from the food itself and the wasted food. Even just a small percentage of wasted food causes more environmental impacts than does the packaging. The second case study presents the results of LCC and LCWE analysis done for fruit and vegetable transport packages. In this thesis, the specific results of the study itself are not the focus, but rather the study methods and scope are analysed based on how these complement the sustainability assessment of food packages. This thesis presents reasons why prevention of food waste should be more thoroughly taken into account in food packaging design. In addition, the task of the packaging designer is facilitated by the requirements of sustainable food packaging, by the methods and step-by-step guidance on how to integrate sustainability issues into the design process, and by the recommendations on how to assess the sustainability of food packages. The intention of this thesis is to express the issues that are important in the field of the food packaging industry. Having recognised and implemented these issues, businesses can better manage the risks that could follow from neglecting these sustainability aspects.

Cesarean section - short term maternal complications related to the mode of delivery

Cesarean section (CS) is the most common major surgery performed on women worldwide. CS can save the life of the mother or the fetus, but is associated with the typical complications of any major surgery: hemorrhage, infection, venous thromboembolism and complications of anesthesia, sometimes leading to maternal death. Recently there have been several reports from well resourced countries on increased severe maternal morbidity and even mortality. Increased rates of CS, obesity and older mothers may explain this rise. The aim of this thesis is to study the rates and risk factors of short term maternal complications associated with CS. Also, we compared maternal morbidity by mode of delivery and over time. The complication rates were assessed in a prospective study involving 2496 CS performed in the 12 largest delivery units in Finland in 2005. The rates of severe complications were studied by mode of delivery in a register-based study comparing national cohorts in 1997 and 2002. The impact of several risk factors on severe maternal morbidity by mode of delivery was studied in a register-based study of all singleton deliveries in 2007-2011. In the prospective study, 27% of the women who underwent CS had one or more intraoperative or postoperative complications during their hospital stay, and 10% had a severe complication. In the register-based study the incidence of life-threatening maternal complications was 7.6 in 1000 deliveries. The incidence was lowest for vaginal delivery (VD), followed by instrumental VD and elective CS, and highest in emergency CS. An attempt of VD, including the risks associated with emergency CS, seems to be the safest mode of delivery, even for most high-risk women.

Relationship between disease severity and quality of life in patients with chronic obstructive pulmonary disease

Few studies have evaluated the relationship between Airways Questionnaire 20 (AQ20), a measure of the quality of life, scores and physiological outcomes or with systemic markers of disease in patients with chronic obstructive pulmonary disease (COPD). The aim of the present study was to investigate the relationship of forced expiratory volume in 1 s (FEV1), body mass index, fat-free mass index, 6-min walk test (6MWT) results, dyspnea sensation and peripheral oxygen saturation (SpO2) with the quality of life of COPD patients. Ninety-nine patients with COPD (mean age: 64.2 ± 9.2 years; mean FEV1: 60.4 ± 25.2% of predicted) were evaluated using spirometry, body composition measurement and the 6MWT. The baseline dyspnea index (BDI) and the Modified Medical Research Council (MMRC) scale were used to quantify dyspnea. Quality of life was assessed using the AQ20 and the St. George's Respiratory Questionnaire (SGRQ). The Charlson index was used to determine comorbidity. The body mass index/airflow obstruction/dyspnea/exercise capacity (BODE) index was also calculated. AQ20 and SGRQ scores correlated significantly with FEV1, SpO2, 6MWT, MMRC and BDI values as did with BODE index. In the multivariate analyses, MMRC or BDI were identified as predictors of AQ20 and SGRQ scores (P < 0.001 in all cases). Thus, the relationship between AQ20 and disease severity is similar to that described for SGRQ. Therefore, the AQ20, a simple and brief instrument, can be very useful to evaluate the general impact of disease when the time allotted for measurement of the quality of life is limited.

Classical and recent advances in the treatment of inflammatory bowel diseases

Crohn's disease (CD) and ulcerative colitis (UC) are intestinal disorders that comprise the inflammatory bowel diseases (IBD). These disorders have a significant effect on the quality of life of affected patients and the increasing number of IBD cases worldwide is a growing concern. Because of the overall burden of IBD and its multifactorial etiology, efforts have been made to improve the medical management of these inflammatory conditions. The classical therapeutic strategies aim to control the exacerbated host immune response with aminosalicylates, antibiotics, corticosteroids, thiopurines, methotrexate and anti-tumor necrosis factor (TNF) biological agents. Although successful in the treatment of several CD or UC conditions, these drugs have limited effectiveness, and variable responses may culminate in unpredictable outcomes. The ideal therapy should reduce inflammation without inducing immunosuppression, and remains a challenge to health care personnel. Recently, a number of additional approaches to IBD therapy, such as new target molecules for biological agents and cellular therapy, have shown promising results. A deeper understanding of IBD pathogenesis and the availability of novel therapies are needed to improve therapeutic success. This review describes the overall key features of therapies currently employed in clinical practice as well as novel and future alternative IBD treatment methods.

Examining Predictors of Quality of Life of Adults with Intellectual Disabilities After Deinstitutionalization

This study examined if a person’s quality of life could be predicted by six relevant factors in a sample of 114 individuals with intellectual disability who had moved from institutional settings to community living settings within Ontario. Further, two aspects of self-efficacy were tested to see if they moderated the relationship between the possible predictors and the quality of life indicator. The initial multiple regression model accounted for a very small amount of the variance in the outcome (r2 = .08). The second analysis included decision-making as a predictor (r2 = .35) but did not find it to be moderator. The third analysis used opportunities for change as a predictor (r2 = .28), and as a moderator with two significant interaction terms, health and years in an institutional setting (r2 = .35). These findings support the often-theorized influence of self-efficacy on quality of life for individuals with intellectual disability.