The PompeQoL questionnaire: Development and validation of a new measure for children and adolescents with Pompe disease.

Genetic disorders pose great challenges for affected individuals and their families, as they must cope with the irreversible nature of the disease and a life-long dependence on medical assistance and treatment. Children and adolescents dealing with Pompe disease (PD) often struggle to keep up with their peers in physical activities. To gain valuable insights into their subjective experiences and better understand their perception and coping related to daily challenges linked to their condition and treatment, the use of standardized questionnaires is crucial. This study introduces the novel PompeQoL 1.0 questionnaire for children and adolescents with PD, designed for comprehensive assessment of both disease-specific FDH and HRQoL through self- and proxy reports. Content validity was ensured through patients' and parents' involvement at the initial stages of development and in subsequent cognitive debriefing process. Participants found the questionnaire easy to understand, answerable, relevant, and comprehensive. Adjustments based on feedback from patients and their parents improved its utility as a patient- and observer-reported outcome measure. After careful item examination, 52 items were selected, demonstrating moderate to excellent test-retest reliability for most scales and initial evidence for satisfactory construct validity. The PompeQoL questionnaire stands as a valuable screening instrument for both clinical and research purposes. Future research should prioritize additional revisions and larger validation studies, focusing on testing the questionnaire in clinical practice and trials. Nevertheless, the PompeQoL 1.0 stands out as the first standardized measure providing insights into disease-specific FDH and HRQoL among children and adolescents with various forms of PD.

Neuropsychiatric drugs and a neurophysiological marker as predictors of health-related quality of life in patients with phantom limb pain.

OBJECTIVE: To explore the relationship between sociodemographic, clinical, and neurophysiological variables and health-related quality of life (HR-QOL) of patients with phantom limb pain. METHODS: This is a cross-sectional analysis of a previous clinical trial. Univariate and multivariate linear and logistic regression analyses were used to model the predictors of HR-QOL. We utilized a sequential modeling approach with increasing adjustment levels, controlling for age and sex, and other relevant clinical variables (time since amputation, level of amputation, and pain). HR-QOL was assessed by the SF-36 Health Survey and its eight subdomains. RESULTS: We analyzed baseline data from 92 patients with lower-limb amputations. They were mostly male (63%), 45.2 ± 15.6 years, with a mean time since amputation of 82.7 ± 122.4 months, and an overall SF-36 score of 55.9 ± 21.5. We found an association between intracortical facilitation in the affected hemisphere (ICF), gabapentin usage, and HR-QOL. ICF is a predictor of better HRQOL, whereas gabapentin usage was associated with a poorer HR-QOL, with the main model explaining 13.4% of the variance in the outcome. For the SF-36 subdomains, ICF was also a positive predictor for social functioning, bodily pain, and vitality, while medication usage was associated with lower scores in mental health, general health perception, bodily pain, and vitality. CONCLUSION: We found firsthand two new independent predictors of HR-QOL in individuals with PLP, namely, the neurophysiological metric ICF and gabapentin usage. These results highlight the role of the motor cortex excitability in the HR-QOL and stress the need for treatments that favor the neuroplastic adaptation after amputation, for which ICF may be used as a possible marker.

[EQ-5D-3L based quality of life for patients of pneumoconiosis combined with tuberculosis and its influencing factors].

Objective: To understand the health-related quality of life for patients of pneumoconiosis combined with tuberculosis and its main influencing factors. Methods: This was a cross-sectional study, and 951 patients of pneumoconiosis combined with tuberculosis from the pneumoconiosis survey in 27 provinces and autonomous regions in China from December 2017 to December 2021 were selected for the study. The nonparametric Mann-Whitney test and the Kruskal-Wallis H test were used to compare the health utility values, and multiple linear regression was used for multifactor analysis. AMOS 24.0 was used to establish a structural equation modeling. Results: The mean age of 951 patients of pneumoconiosis combined with tuberculosis was (59.3±12.4) years. The main types were silicosis combined with tuberculosis (62.2%, 591/951) and coal-worker's pneumoconiosis combined with tuberculosis (34.9%, 332/951), and other type pneumoconiosis-combined tuberculosis was 2.9% (28/951). The proportion of patients with stage Ⅰ, Ⅱ, Ⅲ, and unstaged clinical diagnosis was 27.4% (261/951), 26.6% (253/951), 32.5% (309/951) and 13.5% (128/951), respectively. 63.3% (602/951) of study participants suffered from other chronic diseases, and the percentage of patients combined the number of chronic diseases with 1, 2, and more than 3 respectively were 24.1% (229/951), 16.3% (155/951) and 22.9% (218/951). The median and quartiles of health utility values and the mean±standard deviation of self-rating scores of patients of pneumoconiosis combined with tuberculosis were 0.562 (0.482, 0.766) and (53.7±18.4), respectively, which were lower than patients of pneumoconiosis without tuberculosis (Z=-11.29, P<0.001; t=8.97, P<0.01). The health utility values and self-rating scores for patients of pneumoconiosis combined with tuberculosis were significantly different between urban and rural areas (Z= -2.22, P=0.027; t=4.85, P<0.01). Pain/discomfort was the most frequently reported problem in the five-dimensional distribution of problems, followed by daily activities and anxiety/depression, and the difference in the percentage reported by anxiety/depression between urban and rural areas was significant (χ(2)=30.28, P<0.01). The results of multiple linear regression showed that the survey area, body mass index, education level, age, employment status, annual personal income, stage of pneumoconiosis, number of multi-morbidities, hemoptysis, acute exacerbation of symptoms in two-week, social support and minimum living standard were the main influences on the health utility values of the patients of pneumoconiosis combined with tuberculosis (P<0.05). The results of structural equation model showed that economic security and health status directly affected the health-related quality of life among patients of pneumoconiosis combined with tuberculosis and played a chain-mediating effect in the influence of socioeconomic status on the health-related quality of life among patients of pneumoconiosis combined with tuberculosis. Conclusion: Health-related quality of life was poorer in patients of pneumoconiosis with tuberculosis, with pain and discomfort and anxiety/depression problems being more pronounced, and economic status and health status played multiple mediating roles in the influence of general socio-demographic characteristics on quality of life in pneumoconiosis.

Demonstrating responsiveness of the pediatric cardiac quality of life inventory in children and adolescents undergoing arrhythmia ablation, heart transplantation, and valve surgery.

PURPOSE: Pediatric Cardiac Quality of Life Inventory (PCQLI) is a disease-specific pediatric cardiac health-related quality of life (HRQOL) instrument that is reliable, valid, and generalizable. We aim to demonstrate PCQLI responsiveness in children undergoing arrhythmia ablation, heart transplantation, and valve surgery before and after cardiac intervention. METHODS: Pediatric cardiac patients 8-18 years of age from 11 centers undergoing arrhythmia ablation, heart transplantation, or valve surgery were enrolled. Patient and parent-proxy PCQLI Total, Disease Impact and Psychosocial Impact subscale scores were assessed pre- and 3-12 months follow-up. Patient clinical status was assessed by a clinician post-procedure and dichotomized into markedly improved/improved and no change/worse/much worse. Paired t-tests examined change over time. RESULTS: We included 195 patient/parent-proxies: 12.6 ± 3.0 years of age; median follow-up time 6.7 (IQR = 5.3-8.2) months; procedural groups - 79 (41%) ablation, 28 (14%) heart transplantation, 88 (45%) valve surgery; clinical status - 164 (84%) markedly improved/improved, 31 (16%) no change/worse/much worse. PCQLI patient and parent-proxies Total scores increased (p ≤ 0.013) in each intervention group. All PCQLI scores were higher (p < 0.001) in the markedly improved/improved group and there were no clinically significant differences in the PCQLI scores in the no difference/worse/much worse group. CONCLUSION: The PCQLI is responsive in the pediatric cardiac population. Patients with improved clinical status and their parent-proxies reported increased HRQOL after the procedure. Patients with no improvement in clinical status and their parent-proxies reported no change in HRQOL. PCQLI may be used as a patient-reported outcome measure for longitudinal follow-up and interventional trials to assess HRQOL impact from patient and parent-proxy perspectives.

It is important to have quality of life (QOL) measures that are sensitive to change in QOL before and after procedures and to be sensitive to change over time. The Pediatric Cardiac Quality of Life Inventory (PCQLI) is a QOL measure specifically developed for children with cardiac disease. This study assessed the responsiveness of the PCQLI to detect change in QOL over time. QOL in Children and adolescents who were being treated for abnormal heart rhythms, heart transplantation, and aortic, pulmonary, or mitral valve surgery were assessed before and after their procedure. Children and adolescents with improved clinical status post-procedure, and their parents, reported better QOL after the procedure. Patients with no improvement from a cardiac standpoint and their parents reported no change in QOL after their procedure. The PCQLI may be used to assess QOL before and after cardiac procedures or medical treatment and follow QOL over time.

On the ability of the SF-6D to capture the consequences of chronic illnesses on subjective well-being: Evidence from France.

Using cross-sectional data from a representative sample of the French population (the 2008 Disability Health survey), this paper examines whether the SF-6D, a widely used preference-based measure of health-related quality of life in economic evaluations, fully captures the variation in subjective well-being (SWB) due to chronic illnesses. We conduct a mediation analysis to disentangle the direct and indirect, through the SF-6D, effects of various chronic conditions on SWB (happiness). Our results show that the SF-6D reflects changes in happiness due to most illnesses except mental illness. Changes in SWB mediated by the SF-6D account for 74% of the total effect. The variation unexplained by the SF-6D is significant and increases substantially in the presence of multimorbidity when a chronic illness is combined with anxiety or depression. Overall, our results suggest that the SF-6D incompletely captures the subjective experience of chronically ill patients, especially those with comorbid conditions.

A cross-sectional study on dental anxiety and state anxiety related to removal of fixed partial dentures.

OBJECTIVES: This study aimed to evaluate the effect of the clinical removal of fixed partial dentures (FPDs) on oral health-related quality of life (OHRQoL) and the anxiety values of individuals and to determine the clinical factors of high anxiety levels. METHOD AND MATERIALS: In total, 300 participants were included in this study. Six different reasons for the clinical removal of FPDs (oral examination, denture renewal, endodontic treatment, tooth extraction, periodontal treatment, and composite filling restoration) were defined. The United Kingdom Oral Health-Related Quality-of-Life Measure (OHRQoL-UK), the Modified Dental Anxiety Scale (MDAS), and the Spielberger State-Trait Anxiety Inventory- State (STAI-S) and Trait (STAI-T) were answered. The reason groups were compared using one-way analyses of variance (ANOVA). Binary logistic regression analyses were performed to evaluate the risk factors for high anxiety. RESULTS: There was no significant difference in OHRQoL-UK scores (P=.279) among the reason groups, but there were significant differences in MDAS, STAI-S, and STAI-T scores (P=.004, P<.001, P=.018 respectively) among the reason groups. Endodontic treatment, tooth extraction, and gender were determined to be risk factors, considering the anxiety scales. CONCLUSIONS: Females are 2.2 times more likely to have trait anxiety than men. Although the effect of the reason for the clinical removal of FPDs on OHRQoL was similar among the groups, it is concluded that endodontic treatment and tooth extraction reasons for the clinical removal of FPDs could be risk factors for high anxiety regardless of FPD usage time.

Creating an SF-6Dv2 social value set for New Zealand.

The SF-6D health descriptive system and its second version published in 2020, the SF-6Dv2, is used worldwide for valuing health-related quality of life (HRQoL) for economic evaluation and measuring patient-reported health outcomes. In this study, a valuation tool was developed and applied to create a social value set, comprising 18,750 health state values, for the SF-6Dv2 for New Zealand (NZ). This tool was adapted and extended from the one used to create a social value set for the EQ-5D-5L, a simpler health descriptive system with fewer dimensions and health states. The tool implements the PAPRIKA method, a type of adaptive discrete choice experiment, and a binary search algorithm to identify health states worse than dead and has extensive data quality controls to ensure the validity and reliability of the social value set derived from participants' personal value sets. The tool, accompanied by a short introductory video designed specifically for the SF-6Dv2, was distributed via an online survey to a large representative sample of adult New Zealanders in June-July 2022. The tool's data quality controls enabled participants who failed to understand or sincerely engage with the valuation tasks to be identified and excluded, resulting in the participants being pared down to a sub-sample of 2985 'high-quality' participants whose personal value sets were averaged for the social value set. These results, including participants' positive feedback, demonstrate the feasibility and acceptability of using the tool to value larger health descriptive systems such as the SF-6Dv2. Having successfully created an SF-6Dv2 social value set for NZ, the valuation tool can be readily applied to other countries, used to generate personal value sets for personalised medicine and adapted to create value sets for other health descriptive systems.

Virtual reality-based therapy for chronic low back and neck pain: a systematic review with meta-analysis.

Purpose: To compile all the scientific evidence available to date to evaluate the effect of virtual reality based therapy (VRBT) on reducing pain intensity, kinesiophobia, and associated disability, and on increasing the hr-QoL in patients with chronic neck pain (CNP) or chronic low back pain (CLBP). Methods: Studies published in PubMed Medline, SCOPUS, Web of Science, CINAHL Complete, and Physiotherapy Evidence Database (PEDro) up to June 2023 were searched. All searches followed the PICOS Framework. Two authors independently screened the studies found in the searches. Any differences of opinion regarding the selection of studies were settled by a third author. Results: Twenty-five RCTs, published between 2013 and 2022, providing data from 1261 patients (20 RCTs) with CLBP and 261 patients (five RCTs) with CNP, were included. In reducing pain intensity for patients with CLBP, meta-analyses showed that VRBT is effective in reducing pain just to the end of the intervention, and this effect could be maintained 1 and 6 months after the therapy. Conclusion: VRBT was found to be better than therapeutic exercise (TE), sham, and no intervention (NI), showing a major effect when VRBT was used as a complementary therapy to conventional physiotherapy (CPT). Further, VRBT showed an immediate effect and immersive VRBT was the most adequate VRBT modality in reducing pain in CNP patients. No differences were found between non-immersive VRBT and immersive VRBT in reducing pain, kinesiophobia, disability, and hr-QoL in patients with CLBP.

Health Related Quality of life Amongst Refugees: A meta Analysis of Studies Using the SF-36.

The health of refugees has been widely documented, as has the impact of a range of factors throughout the migration journey from being exposed to violence to the impacts of immigration detention. This study adds to our understanding of health-related quality of life amongst refugees and asylum seekers by evaluating health-related quality of life as measured by the Short-Form 36 (SF-36) Health Survey using meta-analysis. The aims of this study were to (1) provide a summary and overview of health-related quality of life (as measured by the SF-36), including the extent to which this varies and (2) explore the factors that influence health-related quality of life (as measured by the SF-36) amongst refugees and asylum seekers. A search was undertaken of MEDLINE, CINAHL, PSYCINFO and SCOPUS, returning 3965 results. Papers were included if they sampled refugees (or asylum seeker or those with refugee-like experiences) and used the SF-36 (or its variants) as an outcome measure. Mean scores and standard deviations were pooled using a random effects model. The pooled sample size was 18,418. The pooled mean scores for the SF-36 physical summary measures was 54.99 (95% CI 46.01-63.99), while the mental health summary measure was 52.39 (95% CI 43.35-61.43). The pooled mean scores for each of the sub-scales ranged from 49.6 (vitality) to 65.54 (physical functioning). High heterogeneity was found between both summary measures and all sub-scales. In comparison to SF-36 results from general populations in high and middle income countries, these results suggest that refugee quality of life is generally poorer. However, this varied substantially between studies. One issue that is not well clarified by this review are the factors that contributed to health-related quality of life.

Impact of oral care intervention on quality of life of patients with oral cancer undergoing radiotherapy in Sri Lanka: A quasi-experimental study.

BACKGROUND: Radiotherapy is used to treat oral cancer, yet it negatively affects patients' health-related quality of life (HRQOL). The aim was to evaluate the impact of an oral healthcare intervention on HRQOL of patients with oral cancer who receive radiotherapy with or without chemotherapy. METHODS: An oral healthcare intervention was provided to 41 patients with oral cancer before radiotherapy (fluoride varnish application, scaling, permanent restorations, adjustment of sharp teeth, and extraction of teeth with questionable prognosis, oral hygiene instructions), during, and 3 months after radiotherapy (baking soda mouthwash, artificial saliva spray). EORTC QLQ-H&N35 was used to compare the HRQOL of the intervention and control groups, with the latter having received routine oral healthcare. RESULTS: The intervention group showed lesser values for HRQOL domains and items indicating fewer side effects during the last week of radiotherapy and 3 months after, compared to the control group. Most of the changes in HRQOL were significantly less in the intervention group compared to the control group (p < 0.01). CONCLUSION: The oral healthcare intervention effectively reduced the effect of radiotherapy and positively impacted on HRQOL of patients with oral cancer.

Changes in health-related quality of life in common variable immunodeficiency: an eight-year journey, including the COVID-19 pandemic.

BACKGROUND: Personalized medicine requires the assessment of the impact of health care interventions on Health-Related Quality of Life. RESEARCH DESIGN AND METHODS: We run an observational study of HRQoL in 140 CVID patients with biannual assessments over 8 years using a disease-specific tool, the CVID_QoL, and the GHQ questionnaires. Factors influencing changes in HRQoL scores were identified using multiple linear regression models with a stepwise procedure. RESULTS: Infections frequency, female gender, and chronic enteropathy were associated with worse global CVID_QoL scores. The presence of permanent organ damage and older age contributed to the perception of being at risk of health deterioration, while chronic enteropathy was associated with fatigue. The presence of permanent organ damage was also associated with perceived difficulties in usual activities. The frequency of infections was the main risk factor for difficulties in long-term planning and perceptions of vulnerability. Before COVID-19, improved HRQoL scores were associated with reduced respiratory infections and changes in immunoglobulin replacement route and setting. The COVID-19 pandemic caused a sudden deterioration in all HRQoL dimensions, and a further deterioration in the emotional dimension was observed during the pandemic period. Patients who died during the study had worse CVID_QoL scores at all time points, confirming that HRQoL performance is strongly related to patient outcome. CONCLUSIONS: Periodic HRQoL assessments are needed to capture relevant issues that change over time in patients affected by long-term chronic conditions such CVID, possibly identifying areas of intervention.

Emotional intelligence: a novel predictor of quality of life in patients with systemic sclerosis.

Systemic sclerosis (SSc), a chronic systemic autoimmune disease, affects skin and internal organs compromising organ function and leading to significant morbidity and poor health-related quality of life (HrQoL). This cross-sectional study investigated whether HrQoL is influenced by trait emotional intelligence (TEI). Sixty patients with SSc (Female: 86.67%) completed the socio-demographic characteristics form, TEI Questionnaire Short-Form (TEIQue-SF), and Short-Form Health Survey (SF-36). Sixty healthy controls were also completed the TEIQue-SF. A series of multiple linear regression analyses with correlation matrix was used to analyze SF-36 domains as dependent variables with TEI domains (well-being, self-control, emotionality, sociability) as independent variables. The average age of participants was 57.3 ± 12.9 years with a mean disease duration of 7.7 ± 6.7 years. Patients differed from controls in the sociability domain of TEI. TEI global was found to affect the physical and mental component summaries (p < .001), and all 8 dimensions of the HrQoL (p < .001). Age, disease duration, and gastrointestinal manifestations were negatively associated with various components of SF-36. TEI was positively associated with all dimensions of HrQoL. Understanding the relationship between TEI and HrQoL dimensions is important for the support and empowerment of SSc patients, as well as the establishment and implementation of appropriate psychotherapeutic interventions.

Health-related quality of life with comprehensive geriatric assessment guided care versus usual care in older adults with cancer: A systematic review and meta-analysis of randomized trials.

BACKGROUND: To evaluate if comprehensive geriatric assessment (CGA)-guided care improves health-related quality of life (HRQL) in older adults with cancer compared to usual care. METHODS: Relevant randomized controlled trials (RCTs) were identified through biomedical databases. Meta-analyses using DerSimonian-Laird model summarized the difference in the mean change of HRQL scores from baseline across various time points, with evidence certainty assessed by the GRADE tool. Logistic regression via generalized estimating equations analyzed predictors of HRQL improvement. RESULTS: Potential improvement in the global HRQL score by CGA-guided care at 3 months (Cohen's d 0.27, 95 % CI -0.03-0.58, moderate certainty), could not be excluded. Larger RCTs or those mandating CGA before initiating anti-cancer treatment were predictors of improved HRQL. CONCLUSION: The effects of CGA-guided care on HRQL were variable. Larger RCTs and those mandating pre-treatment CGA tended to report improved HRQL.

Appropriateness of the EQ-5D-5L in capturing health-related quality of life in individuals with transfusion-dependent ß-thalassemia: a mixed methods study.

BACKGROUND: Individuals with transfusion-dependent ß-thalassemia (TDT) experience symptoms and functional impacts that reduce their health-related quality of life. However, EQ-5D-derived health utility index scores in TDT often indicate good HRQoL, suggesting the EQ-5D may not adequately capture the impact of TDT. This study explored the disease and treatment burden of TDT and examined the appropriateness of the EQ-5D-5L descriptive system (DS) in measuring HRQoL in TDT. METHODS: Adults with TDT in the United Kingdom, United States, and France completed a background questionnaire and EQ-5D-5L DS, followed by 60-minute semi-structured interviews on symptoms and HRQoL impacts of TDT (concept elicitation) and appropriateness of EQ-5D-5L DS (cognitive debrief). Transcribed interviews were analyzed using thematic and content analyses. The relationship between TDT symptoms and impacts were summarized in a conceptual model. EQ-5D-5L DS was mapped to concepts identified in the qualitative data to assess its capture of HRQoL concepts. Participants' EQ-5D-5L DS scores were compared to their qualitative descriptions for each dimension to assess their concordance. RESULTS: Thirty participants in the United States (n = 14 [46.7%]), United Kingdom. (n = 12 [40.0%]), and France (n = 4 [13.3%]) completed the study (73.3% female; mean age = 28.4 years [standard deviation (SD) = 5.1]; mean annual red blood cell transfusion [RBCT] frequency = 18.4 [SD = 7.6]). Participants reported TDT symptoms and impacts on HRQoL, all fluctuating across the RBCT cycle. EQ-5D-5L DS did not fully capture 11 of 16 (68.8%) HRQoL concepts reported. Most participants (n = 20/27 [74.1%]) reported that EQ-5D-5L DS did not capture important aspects of living with TDT, and 42.9% (n = 12/28) reported negative/neutral overall impressions of EQ-5D-5L DS. The highest degree of discordance between participants' qualitative data and EQ-5D-5L DS dimension scores was observed with mobility (42.3%) and self-care (34.6%), where the qualitative descriptions relating to these dimensions were worse than their quantitative scores. CONCLUSION: Current findings suggest that EQ-5D-5L DS lacks content validity and the derived health utility index score may not fully represent the burden of disease in TDT.

Changes in the Quality of Life and Nutrition Markers in Transition From End-Stage Kidney Disease to Kidney Transplantation: Insights From a Vietnamese Cohort.

Background Malnutrition is strongly associated with lower quality of life (QoL) and lower survival rates in patients with end-stage kidney disease. However, the impact of renal transplantation on nutrition factors and QoL is unclear. Therefore, this study aims to assess changes in QoL and investigate the relationships with nutrition factors among kidney transplant recipients (KTRs). Materials and methods A longitudinal study included 86 dialysis patients aged 18-65 years who underwent primary kidney transplantation (KTx) and were followed up for one year. Body weight, biochemical parameters, and QoL data were collected before transplantation (T0) and at six months (T6) and 12 months (T12) post-transplantation. Effect size (ES) was used to measure the impact of KTx on QoL and nutritional status from T0 to T12. The predictors of QoL were calculated with ß-coefficients and p<0.05 in linear regression. Results The ES of transplantation on the QoL of KTRs was large, at 1.1 for health change, 0.9 for physical health, and moderate (0.7) for mental health (MH) over one year. Hemoglobin and malnourished were affected by KTx, with ES being 2.4 and 0.6, respectively. Linear regression showed that physical health was predicted by hemoglobin (ß=0.12, p<0.01), phosphorus (ß=7.82, p<0.05), and dose of mycophenolate mofetil (MMF) (ß=-0.01, p<0.05). Mental health was predicted by obesity (ß=-7.63, p<0.05), hemoglobin (ß=0.11, p<0.05), and phosphorus (ß=8.49, p<0.01). Health change was indicated by nutritional risk index (NRI) score (ß=0.47, p<0.05), total cholesterol (ß=3.39, p<0.01), and kidney function (ß=0.15, p<0.05). Conclusions The transition from end-stage kidney disease to transplantation has positive impacts on QoL and nutrition markers. Nutritional status, kidney function, and the dose of mycophenolate mofetil are significant determinants of QoL in KTRs.

The association between body mass index, exercise capacity, and health-related quality of life in heart transplant recipients.

Introduction: Pre-transplant obesity and weight gain after heart transplantation are both associated with increased risk of poor clinical outcomes. We aimed to assess the association between overweight or obesity, exercise capacity, and health-related quality of life in heart transplant recipients. Methods: This study is based on baseline data from the IronIC trial, in which we randomized 102 heart transplant recipients with iron deficiency to ferric derisomaltose or placebo. We performed cardio pulmonary exercise testing in all participants. To assess quality of life, we used the SF-36v2 questionnaire, using two sum scores: the physical component summary and the mental component summary. A minimal clinically important difference was defined as ≥2 and ≥3 for the physical and the mental component summary, respectively. Results: 24/102 heart transplant recipients (24%) had a body mass index (BMI) ≥30 kg/m2. Peak oxygen consumption was 17.3 ± 4.6 ml/kg/min in the obese group vs. 24.7 ± 6.4 ml/kg/min in the group with a BMI <30 for a between-group difference of 7.4 (95% confidence interval 4.7-10.2) ml/kg/min: p < 0.001. The physical component summary score was on average 5.2 points lower in the patients with a body mass index ≥30 than in the lower weight group (p = 0.04). Conclusion: Almost a quarter of our heart transplant recipients in long-term follow-up had a BMI ≥30 kg/m2. These patients had substantially lower exercise capacity and lower quality of life in the physical domain.

Benefits of a family-based care transition program for older adults after hip fracture surgery.

BACKGROUND: Hip fracture (HF) in older adults is strongly associated with a greater decline in their activities of daily living (ADLs) and health-related (HRQoL). The present study aimed to evaluate the effects of a family-based care transition program (FBCTP) on ADLs, HRQoL and social support in this age group after HF surgery. METHODS: A quasi-experimental design was conducted on 100 older adults who had undergone HFS and were selected by convenience sampling and allocated to the IG (n = 50) and the CG (n = 50). Data were collected utilizing the Barthel Index, the 12-item Short Form Health Survey (SF-12), and the Multidimensional Scale of Perceived Social Support. The FBCTP was delivered in-hospital education sessions, home visit, and a follow-up and telephone counselling session. The data were collected at three stages, including the baseline, four weeks after discharge, and eight weeks later. The level of statistical significance was set at 0.05. RESULTS: The results of the study indicated that the effects of time and group on the increase in ADLs were 15.2 and 36.69 (p < 0.000), respectively, following the completion of the FBCTP. Furthermore, time and group were found to have a positive effect on HRQoL, with an increase of 2.82 and 5.60 units, respectively (p < 0.000). In this context, time and group also interacted in the IG compared to the CG, with scores increasing by 1.86 units over time (p < 0.000). Although the study results indicated that social support improved by 1.98 units over time (p < 0.000), the effects of group alone and the time × group interaction were not statistically significant. This indicates that the program was not effective in accelerating social support. CONCLUSION: Consequently, nurses, policymakers, and planners engaged in geriatric healthcare may utilize these results to enhance the health status of this age group following HFS.

Hereditary angioedema prevalence and satisfaction with prophylaxis in South Australia.

Background: Hereditary angioedema (HAE) due to deficiency of C1 Inhibitor (C1INH-HAE) is a rare, unpredictable and potentially fatal genetic disorder. There are relatively few systematic population prevalence studies, with reports from various countries of between 1 in 20,000 and 1 in 150,000. and no Australian data. The therapeutic landscape for HAE has changed dramatically in recent years with a focus on highly effective prophylaxis, with the aim of total suppression of angioedema and achievement of a normal life. Objectives: Epidemiological survey of HAE in South Australia, with description of patient characteristics, quality of life and treatment, with a focus on prophylaxis. Methods: Case ascertainment was conducted over 18 months from January 2021 to July 2022, using a range of approaches with the aim of identifying all people with C1INH-HAE in South Australia. Questionnaires were administered to consenting patients utilising established HAE-specific and general survey instruments. Results: We identified 35 people with HAE in South Australia, yielding a population prevalence of 1 in 52,400, in line with average established international prevalence. HAE was identified in 4 patients of Indigenous Australian heritage. Seventeen of 31 adult patients completed an additional multi-questionnaire survey, revealing overall satisfactory disease control. Most common prophylactic therapies were danazol, lanadelumab, and subcutaneous C1 inhibitor. Many patients (mostly male) with milder disease had responded well to low-dose danazol with good tolerance and have continued to use it, whereas patients with higher disease burden are now using newer therapies, and overall satisfaction with current prophylaxis is high. Conclusions: Prevalence of HAE in South Australia aligns with international reports. Our population survey indicates that current long-term prophylaxis therapies including danazol, lanadelumab and C1-inhibitor, applied to appropriate patients taking into account disease activity and drug risks and tolerance, are effective for HAE attack prevention and produce high levels of satisfaction.

The effectiveness of health-related quality of life (HRQOL) model in people with HIV: A cross sectional study.

Background and Aims: Acquired immune deficiency syndrome (AIDS) are a chronic and disabling disease that has a significant impact on quality of life due to weakening of physical health, financial problems and social stigma. This study aims to validate the Health-related quality of life (HRQOL) model in people with human immunodeficiency viruses (HIV) in Iran. Methods: Four hundred and fifty-two people with HIV from Imam Khomeini Hospital between the age of 18 and 65 years (men: 308, women: 144) participated in the research. anonymously completed a battery of questionnaires, namely the Persian basic psychological need satisfaction and frustration scale, SF-36, PSQI and mindful attention awareness scale. The method of the present study was the structural equation model. Results: Current findings indicated there is a significant positive between mindfulness and need satisfaction, physical and mental health and, significant negative between mindfulness and quality and quantity of sleep. Mindfulness and need satisfaction are significant positive predictors of quality of life in people with HIV. Quality and quantity of sleep are significant negative predictors of quality of life in people with HIV. There is a positive significance between need satisfaction and physical and mental health but there is a negative significance between need satisfaction and quality and quantity. there is a negative significant between the quality and quantity of sleep with physical and mental health. Mindfulness facilitates the satisfaction of more psychological needs and improves the quality of sleep. The quality of sleep is a negative significant predictor for physical and mental health but the quantity of sleep is a negative significant predictor for physical health. Conclusion: The HRQOL model can explain 18% of physical health and 16% of mental health in people with HIV in Iran. The elements of this model can be useful in evaluating and treating people with HIV in the Iranian Population. They also can use the models to plan for better services.

Do Measures of Real-World Physical Behavior Provide Insights Into the Well-Being and Physical Function of Cancer Survivors? Cross-Sectional Analysis.

BACKGROUND: As the number of cancer survivors increases, maintaining health-related quality of life in cancer survivorship is a priority. This necessitates accurate and reliable methods to assess how cancer survivors are feeling and functioning. Real-world digital measures derived from wearable sensors offer potential for monitoring well-being and physical function in cancer survivorship, but questions surrounding the clinical utility of these measures remain to be answered. OBJECTIVE: In this secondary analysis, we used 2 existing data sets to examine how measures of real-world physical behavior, captured with a wearable accelerometer, were related to aerobic fitness and self-reported well-being and physical function in a sample of individuals who had completed cancer treatment. METHODS: Overall, 86 disease-free cancer survivors aged 21-85 years completed self-report assessments of well-being and physical function, as well as a submaximal exercise test that was used to estimate their aerobic fitness, quantified as predicted submaximal oxygen uptake (VO2). A thigh-worn accelerometer was used to monitor participants' real-world physical behavior for 7 days. Accelerometry data were used to calculate average values of the following measures of physical behavior: sedentary time, step counts, time in light and moderate to vigorous physical activity, time and weighted median cadence in stepping bouts over 1 minute, and peak 30-second cadence. RESULTS: Spearman correlation analyses indicated that 6 (86%) of the 7 accelerometry-derived measures of real-world physical behavior were not significantly correlated with Functional Assessment of Cancer Therapy-General total well-being or linked Patient-Reported Outcomes Measurement Information System-Physical Function scores (Ps≥.08). In contrast, all but one of the physical behavior measures were significantly correlated with submaximal VO2 (Ps≤.03). Comparing these associations using likelihood ratio tests, we found that step counts, time in stepping bouts over 1 minute, and time in moderate to vigorous activity were more strongly associated with submaximal VO2 than with self-reported well-being or physical function (Ps≤.03). In contrast, cadence in stepping bouts over 1 minute and peak 30-second cadence were not more associated with submaximal VO2 than with the self-reported measures (Ps≥.08). CONCLUSIONS: In a sample of disease-free cancer survivors, we found that several measures of real-world physical behavior were more associated with aerobic fitness than with self-reported well-being and physical function. These results highlight the possibility that in individuals who have completed cancer treatment, measures of real-world physical behavior may provide additional information compared with self-reported and performance measures. To advance the appropriate use of digital measures in oncology clinical research, further research evaluating the clinical utility of real-world physical behavior over time in large, representative samples of cancer survivors is warranted. TRIAL REGISTRATION: ClinicalTrials.gov NCT03781154; https://clinicaltrials.gov/ct2/show/NCT03781154.