What a Clinician Needs to Know About Genome Editing: Status and Opportunities for Inborn Errors of Immunity.

During the past 20 years, gene editing has emerged as a novel form of gene therapy. Since the publication of the first potentially therapeutic gene editing platform for genetic disorders, increasingly sophisticated editing technologies have been developed. As with viral vector-mediated gene addition, inborn errors of immunity are excellent candidate diseases for a corrective autologous hematopoietic stem cell gene editing strategy.

Frontstage nursing and backstage growth: The emotional labour of student nurses in Dutch nursing homes.

The complex emotional work of nurses calls for more recognition of emotional labour and the incorporation of emotional labour in nursing education. Based on participant observation and semistructured interviews, we describe the experiences of student nurses in two nursing homes for elderly people with dementia in the Netherlands. We analyse their interactions using Goffman's dramaturgical view on the front and backstage behaviour and the distinction between surface acting and deep acting.

Gene therapy for inborn error of immunity - current status and future perspectives.

Purpose Of Review: Development of hematopoietic stem cell (HSC) gene therapy (GT) for inborn errors of immunity (IEIs) continues to progress rapidly. Although more patients are being treated with HSC GT based on viral vector mediated gene addition, gene editing techniques provide a promising new approach, in which transgene expression remains under the control of endogenous regulatory elements.

Recent Findings: Many gene therapy clinical trials are being conducted and evidence showing that HSC GT through viral vector mediated gene addition is a successful and safe curative treatment option for various IEIs is accumulating.

Lentiviral Gene Transfer Corrects Immune Abnormalities in XIAP Deficiency.

Background: X-linked inhibitor of apoptosis protein (XIAP) deficiency is a severe immunodeficiency with clinical features including hemophagocytic lymphohistiocytosis (HLH) and inflammatory bowel disease (IBD) due to defective NOD2 responses. Management includes immunomodulatory therapies and hematopoietic stem cell transplant (HSCT). However, this cohort is particularly susceptible to the chemotherapeutic regimens and acutely affected by graft-vs-host disease (GvHD), driving poor long-term survival in transplanted patients.

Evolution of Our Understanding of XIAP Deficiency.

X-linked inhibitor of apoptosis (XIAP) deficiency is a rare inborn error of immunity first described in 2006. XIAP deficiency is characterised by immune dysregulation and a broad spectrum of clinical manifestations, including haemophagocytic lymphohistiocytosis (HLH), inflammatory bowel disease (IBD), hypogammaglobulinemia, susceptibility to infections, splenomegaly, cytopaenias, and other less common autoinflammatory phenomena. Since the first description of the disease, many XIAP deficient patients have been identified and our understanding of the disease has grown.

Motivational factors for initiating and maintaining physical activity among adults aged over fifty targeted by a tailored intervention.

To provide insight into the motivational working mechanisms (i.e. mediators) of an effective physical activity (PA) intervention for adults aged over fifty.

Disease duration as an indicator of the efficacy of liraglutide in patients with type 2 diabetes mellitus.

Comment on the article of Usui et al. Retrospective cohort study of obese patients with type 2 diabetes mellitus (n = 69) demonstrates that the glucose-lowering effect of liraglutide as add on therapy to insulin relies on the remaining beta-cell function in type 2 diabetes. Shorter disease duration implies a more favourable prognosis for response on instantaneous substitution of insulin with liraglutide (HR 2.

Feasibility and acceptability of a telephone- and face-to-face-delivered counseling intervention for smoking cessation in Dutch patients with coronary heart disease.

Intensive behavioral counseling interventions combined with nicotine replacement therapy have increased smoking abstinence rates in cardiac patients, but little is known about their feasibility when initiated upon hospital admission and continued post-discharge. The current study was an evaluation of the use, appreciation, and fidelity of two post-discharge counseling interventions designed for cardiac patients to quit smoking that differed in their delivery mode. In a controlled trial with cross-over randomization at the cardiac unit level, hospitalized smokers in eight cardiac units of eight Dutch hospitals were assigned either telephone counseling (n = 223) or nurse-administered face-to-face counseling (n = 157) using the Ask-Advise-Refer strategy.

Modeling Longitudinal Relationships Between Habit and Physical Activity: Two Cross-Lagged Panel Design Studies in Older Adults.

These longitudinal studies in older adults targeted mediated relationships between habit and physical activity (PA). In The Netherlands two independent studies were conducted among 1976 (Study 1: Mage = 63.63, SD = 8.

High intensity smoking cessation interventions: Cardiac patients of low socioeconomic status and low intention to quit profit most.

Background: Without assistance, smokers being admitted to the hospital for coronary heart disease often return to regular smoking within a year.

Objective: This study assessed the 12-month effectiveness of a telephone and a face-to-face counselling intervention on smoking abstinence among cardiac patients. Differential effects for subgroups varying in their socioeconomic status and intention to quit smoking were also studied.

Eosinophilic esophagitis: published evidences for disease subtypes, indications for patient subpopulations, and how to translate patient observations to murine experimental models.

Eosinophilic esophagitis (EoE) is a chronic inflammatory disorder of the esophagus and commonly classified as a Th2-type allergy. Major advances in our understanding of the EoE pathophysiology have recently been made, but clinicians struggle with highly unpredictable therapy responses indicative of phenotypic diversity within the patient population. Here, we summarize evidences for the existence of EoE subpopulations based on diverse inflammatory characteristics of the esophageal tissue in EoE.

The influence of oral contraceptives on overnight 1 mg dexamethasone suppression test.

Background: In suspected hypercortisolism, the 1 mg dexamethasone suppression test is the usual initial test. In fertile women, false-positive test results are often due to the use of oral contraceptives. By elevating cortisol-binding globulin these contraceptives increase the total serum cortisol concentration.

Smoking cessation in cardiac patients: the influence of action plans, coping plans and self-efficacy on quitting smoking.

Smoking cessation is the most effective action for cardiac patients who smoke to improve their prognosis, yet more than one-half of cardiac patients continue to smoke after hospital admission. This study examined the influence of action plans, coping plans and self-efficacy on intention to quit and smoking cessation in cardiac patients. Cardiac patients completed a baseline questionnaire (N = 245) assessing demographic characteristics, smoking behavior, intention, self-efficacy, relapse self-efficacy and action and coping plans.

Self-Reporting of Smoking Cessation in Cardiac Patients: How Reliable Is It and Is Reliability Associated With Patient Characteristics?

Objectives: The objectives of this study were to determine the accuracy of smoking cessation self-reports by cardiac patients who participated in a smoking cessation program, and to determine which patient characteristics are associated with an inaccurate self-report during a follow-up interview 12 months after the start of the program.

Methods: Smoking cessation self-reports (point prevalence abstinence) were validated against salivary cotinine levels. Using χ analyses, patients who reported accurately being a nonsmoker were compared with those who reported inaccurately being a nonsmoker (biochemically verified as smokers) on factors the literature has indicated to be associated with inaccurate self-report in smoking.

Economic evaluation of a telephone- and face-to-face-delivered counseling intervention for smoking cessation in patients with coronary heart disease.

Objective: This study examined the cost-effectiveness and cost-utility of two smoking cessation counseling interventions differing in their modality for patients diagnosed with coronary heart disease from a societal perspective.

Methods: In a randomized controlled trial conducted in Dutch hospital wards, cardiac patients who smoked prior to admission were allocated to usual care (n = 245), telephone counseling (n = 223) or face-to-face counseling (n = 157). The counseling interventions lasted for 3 months and were complemented by nicotine patches.

Long-term health outcomes and cost-effectiveness of a computer-tailored physical activity intervention among people aged over fifty: modelling the results of a randomized controlled trial.

Background: Physical inactivity is a significant predictor of several chronic diseases, becoming more prevalent as people age. Since the aging population increases demands on healthcare budgets, effectively stimulating physical activity (PA) against acceptable costs is of major relevance. This study provides insight into long-term health outcomes and cost-effectiveness of a tailored PA intervention among adults aged over fifty.

Cost-effectiveness and cost-utility of a Web-based or print-delivered tailored intervention to promote physical activity among adults aged over fifty: an economic evaluation of the Active Plus intervention.

Background: The adverse health effects of insufficient physical activity (PA) result in high costs to society. The economic burden of insufficient PA, which increases in our aging population, stresses the urgency for cost-effective interventions to promote PA among older adults. The current study provides insight in the cost-effectiveness and cost-utility of different versions of a tailored PA intervention (Active Plus) among adults aged over fifty.

The differentiated effectiveness of a printed versus a Web-based tailored physical activity intervention among adults aged over 50.

This study provides insight in the effectiveness of a print-delivered and a Web-based physical activity (PA) intervention (with or without additional environmental information on local PA possibilities) among people aged over 50. Intervention groups (print-delivered basic [PB; n = 439], print-delivered environmental [PE; n = 435], Web-based basic [WB; n = 423], Web-based environmental [WE; n = 432]) and a control group (n = 411) were studied in a clustered randomized controlled trial. Participants received three times tailored advice within four months, targeting the psychosocial determinants of PA, and additional environmental information (in two conditions).

[Measuring the handgrip strength of geriatric patients].

The handgrip strength of geriatric patients can be measured when the patient is hospitalized. This article elaborates on the intrinsic and extrinsic factors which have a direct or indirect influence on handgrip strength. For the best results the tests need to be taken in the best circumstances with attention to individual differences and the age of the patient.

Correlates of the intention to implement a tailored physical activity intervention: perceptions of intermediaries.

The public health impact of health behaviour interventions is highly dependent on large-scale implementation. Intermediaries-intervention providers-determine to a large extent whether an intervention reaches the target population, and hence its impact on public health. A cross-sectional study was performed to identify the correlates of intermediaries' intention to implement a computer-tailored physical activity intervention.

Long-term efficacy of a printed or a Web-based tailored physical activity intervention among older adults.

Background: This study provides insight into the long-term efficacy (i.e. 12 month results) of the Web-based or print-delivered tailored Active Plus intervention (with and without environmental approach) to promote physical activity (PA) among the over-fifties.

Differences in the use and appreciation of a web-based or printed computer-tailored physical activity intervention for people aged over 50 years.

This study provides insight into the use and appreciation of a tailored physical activity intervention for people aged over 50 years in different intervention conditions (i.e. printed versus web-based and basic versus environmental).

Effectiveness of a telephone delivered and a face-to-face delivered counseling intervention for smoking cessation in patients with coronary heart disease: a 6-month follow-up.

Smoking cessation interventions for cardiac patients need improvement given their weak effects on long-term abstinence rates and low compliance by nurses to implementation. This study tested the effectiveness of two smoking cessation interventions against usual care in cardiac patients, and conditional effects for patients' motivation to quit and socio-economic status (SES). An experimental study was conducted from 2009 to 2012 for which Dutch cardiac patient smokers were assigned to: usual care (UC; n = 245), telephone counseling (TC; n = 223) or face-to-face counseling (FC; n = 157).

Differences in reach and attrition between Web-based and print-delivered tailored interventions among adults over 50 years of age: clustered randomized trial.

Background: The Internet has the potential to provide large populations with individual health promotion advice at a relatively low cost. Despite the high rates of Internet access, actual reach by Web-based interventions is often disappointingly low, and differences in use between demographic subgroups are present. Furthermore, Web-based interventions often have to deal with high rates of attrition.

Habit as moderator of the intention-physical activity relationship in older adults: a longitudinal study.

This longitudinal study examined whether habit strength moderates the intention-physical activity (PA) relationship in older adults, within the framework of the attitude-social influences-efficacy (ASE) model and the theory of planned behaviour (TPB). A total of 1836 older adults (Mage = 62.95 years, SDage = 8.