Pilot RCT of a telehealth intervention to reduce symptoms of depression and anxiety in adults with cystic fibrosis

BackgroundAdults with cystic fibrosis (awCF) have higher levels of depression and anxiety than community samples. The Coping and Learning to Manage Stress with CF (CALM) intervention was developed for awCF reporting elevated symptoms of depression or anxiety.MethodsIn this pilot study, awCF were randomly assigned to either six telehealth sessions (CALM; n = 15) or treatment-as-usual (TAU; n = 16). Primary outcomes were depression and anxiety. Secondary outcomes were coping self-efficacy and health-related quality of life (HrQOL). Tertiary outcomes were feasibility, acceptability, and satisfaction. Assessments were completed at baseline, post-intervention, and 3-month follow-up. Group differences were examined via independent samples t-tests. Effect size (ES) was calculated via Cohen's d to provide a measure of the magnitude of the treatment effect.ResultsAt post-intervention, the CALM group had a lower mean score than the TAU group for depression (medium ES) and anxiety (large ES). The CALM group had higher (i.e., better) mean scores than the TAU group for coping (large ES) and HrQOL domains of Social Functioning (large ES) and Vitality (large ES). Most treatment gains were not sustained at 3-month follow-up. CALM was feasible, requiring <12 min. for setup and scheduling, and allowed seamless participation when hospitalized. Mean scores for acceptability and satisfaction indicated that most participants either agreed or strongly agreed that CALM was acceptable and satisfactory.ConclusionsCALM shows promise as an intervention to reduce symptoms of depression and anxiety and improve coping and HrQOL. Next steps are to add a booster session and examine CALM via a multi-site RCT.     

Clinical presentation and basic defect of the CFTR genotype p.Phe508del / p.Arg117His in a mother and her monozygous twin daughters

A non-consanguineous two-generation family of parent and monozygous twins who all three share the same CFTR mutation genotype p.Phe508del / p.Arg117His, was examined in clinical features, sweat test, nasal potential difference and intestinal current measurements. As expected the twins were very much alike in anthropometry and appearance and shared the clinical manifestation of CFTR dysfunction albeit at different intensity but unexpectedly like in comparison to their mother they were discordant in their CFTR-mediated basic defect and the response thereof to CFTR potentiation by ivacaftor. This case report illustrates the strong impact of non-inherited factors on the electrophysiological phenotype of the most common CFTR mutation genotype of variable clinical significance.     

Systematic review and meta-analysis of immune response of double dose of hepatitis B vaccination in HIV-infected patients

IntroductionThe prevalence of co-infection of hepatitis B virus (HBV) and human immunodeficiency virus (HIV) is high and increases risk of hepatitis B chronicity and mortality. Despite guidelines for HIV-infected patients to be immunized against HBV, the immunogenicity of the HBV vaccination in HIV-infected patients is lower than that in the HIV-seronegative population.MethodIn this study, we performed a systematic review of the literature and meta-analysis of randomized clinical trials to investigate the response rate to an increased dose of HBV vaccination in HIV-infected patients. A fixed-effects model, with heterogeneity and sensitivity analyses, was used. We identified nine studies involving 970 HIV-positive vaccine recipients.ResultsThe study results were divided into two groups, depending on the time when antibody against hepatitis surface antigen was measured. Results showed a significant increase in response rates among patients who received a double dose of the vaccine versus the standard dose in both subgroups; the pooled odds ratio (OR) was 1.76 (95% confidence interval [CI]: 1.36–2.29) and 2.28 (95% CI: 1.73–3.01) for the rate that was measured 4–6 weeks and >12 months after completion of vaccination, respectively. The total OR was 1.99 (95% CI: 1.64–2.41). No heterogeneity was found.DiscussionOur meta-analysis shows that a double dose of the HBV vaccine may significantly improve the immune response in HIV-infected patients. Higher immunogenicity was observed, when it was measured 4–6 weeks and >12 months after completion of the vaccination.     

A pair of DICER1‐positive monozygotic twins: One with pleuropulmonary blastoma,another with acute transient hepatitis

Pleuropulmonary blastoma (PPB) is the most common primary malignant neoplasm of the lung in children that is associated with a germline mutation in DICER1. In this report, we share an interesting case of a pair of monozygotic twins: one of them developed PPB when she was 4‐year old, while the other developed acute transient hepatitis when she was 5‐year old. Next‐Gen sequencing for DICER1 mutations of their family revealed that both twins and their mother had c.C3675A mutation. The mother also had a history of multinodular goiter. Identification of DICER1 mutation carriers and close surveillance of individuals at risk for DICER1 syndrome may allow early detection and hence better outcome.     

A guide for health professionals to interpret and use recommendations in guidelines developed with the GRADE approach

An increasing number of organizations worldwide are using new and improved standards for developing trustworthy clinical guidelines. One of such approaches, developed by the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) working group, offers systematic and transparent guidance in moving from evidence to recommendations. The GRADE strategy concentrates on four factors: the balance between benefits and harms, the certainty of the evidence, values and preferences, and resource considerations. However, it also considers issues around feasibility, equity, and acceptability of recommendations. GRADE distinguishes two types of recommendations: strong and weak. Strong recommendations reflect a clear preference for one alternative and should apply to all or almost all patients, obviating the need for a careful review of the evidence with each patient. Weak recommendations are appropriate when there is a close balance between desirable and undesirable consequences of alternative management strategies, uncertainty regarding the effects of the alternatives, uncertainty or variability in patients' values and preferences, or questionable cost-effectiveness. Weak recommendations usually require accessing the underlying evidence and a shared decision-making approach. Clinicians using GRADE recommendations should understand the meaning of the strength of the recommendation, be able to critically appraise the recommendation, and apply trustworthy recommendations according to their strength.     

Preseason functional tests discriminate injury risk in female collegiate volleyball players

ObjectivePreseason functional tests have been previously associated with noncontact time-loss lower extremity injuries in a variety of athletic populations. This study aimed to determine the effectiveness of one or more functional tests to discriminate injury risk in a cohort of female collegiate volleyball players in the United States.DesignProspective cohort study.SettingNational Collegiate Athletic Association Division II & III, National Association of Intercollegiate Athletics female volleyball players;Participants130 female volleyball players (mean age: 19.31 ± 1.1 years).Main outcome measureLower extremity functional test, single limb hop for distance, standing long jump, previous injury history, lower quarter Y-balance test, and limb symmetry index were measured prior to the season beginning. Noncontact time-loss lower quarter injuries were tracked during the season.ResultsAthletes with suboptimal scores on the single limb hop test (<70% of height) and standing long jump (<80% of height), combined with a previous history of injury, were three times more likely to sustain an injury during the season (OR = 3.0; 95% CI: 1.09 to 8.30). Individual functional tests did not discriminate injury risk.ConclusionsA battery of preseason functional tests and injury history discriminates injury risk in female collegiate volleyball players.