Neural sensitivity to facial identity and facial expression discrimination in adults with autism.

The fluent processing of faces can be challenging for autistic individuals. Here, we assessed the neural sensitivity to rapid changes in subtle facial cues in 23 autistic men and 23 age and IQ matched non-autistic (NA) controls using frequency-tagging electroencephalography (EEG). In oddball paradigms examining the automatic and implicit discrimination of facial identity and facial expression, base rate images were presented at 6 Hz, periodically interleaved every fifth image with an oddball image (i.e. 1.2 Hz oddball frequency). These distinctive frequency tags for base rate and oddball stimuli allowed direct and objective quantification of the neural discrimination responses. We found no large differences in the neural sensitivity of participants in both groups, not for facial identity discrimination, nor for facial expression discrimination. Both groups also showed a clear face-inversion effect, with reduced brain responses for inverted versus upright faces. Furthermore, sad faces generally elicited significantly lower neural amplitudes than angry, fearful and happy faces. The only minor group difference is the larger involvement of high-level right-hemisphere visual areas in NA men for facial expression processing. These findings are discussed from a developmental perspective, as they strikingly contrast with robust face processing deficits observed in autistic children using identical EEG paradigms.

Oestrogen enforces the integrity of blood vessels in the bone during pregnancy and menopause.

The mammalian skeletal system shows sex differences in structure, functions, ageing and disease incidences. The role of blood vessels in physiological, regenerative and pathological bone functions indicates the requisite to understanding their sex specificity. Here, we find oestrogen regulates blood vessel physiology during pregnancy and menopause through oestrogen receptor alpha (ERα) and G-protein coupled oestrogen receptor-1 (Gper1) but not ERß-dependent signalling in mice. Oestrogen regulates BECs' lipid use and promotes lipolysis of adipocytes and FA uptake from the microenvironment. Low oestrogen conditions skew endothelial FA metabolism to accumulate lipid peroxides (LPO), leading to vascular ageing. High ferrous ion levels in female BECs intensify LPO accumulation and accelerate the ageing process. Importantly, inhibiting LPO generation using liproxstatin-1 in aged mice significantly improved bone heath. Thus, our findings illustrate oestrogen's effects on BECs and suggest LPO targeting could be an efficient strategy to manage blood and bone health in females.

Evaluation of a Collaborative Care Program for Patients With Treatment-Resistant Schizophrenia: Protocol for a Multiple Case Study.

BACKGROUND: Approximately one-third of all patients with schizophrenia are treatment resistant. Worldwide, undertreatment with clozapine and other effective treatment options exist for people with treatment-resistant schizophrenia (TRS). In this respect, it appears that regular health care models do not optimally fit this patient group. The Collaborative Care (CC) model has proven to be effective for patients with severe mental illness, both in primary care and in specialized mental health care facilities. The key principles of the CC model are that both patients and informal caregivers are part of the treatment team, that a structured treatment plan is put in place with planned evaluations by the team, and that the treatment approach is multidisciplinary in nature and uses evidence-based interventions. We developed a tailored CC program for patients with TRS. OBJECTIVE: In this paper, we provide an overview of the research design for a potential study that seeks to gain insight into both the process of implementation and the preliminary effects of the CC program for patients with TRS. Moreover, we aim to gain insight into the experiences of professionals, patients, and informal caregivers with the program. METHODS: This study will be underpinned by a multiple case study design (N=20) that uses a mixed methods approach. These case studies will focus on an Early Psychosis Intervention Team and 2 Flexible Assertive Community treatment teams in the Netherlands. Data will be collected from patient records as well as through questionnaires, individual interviews, and focus groups. Patient recruitment commenced from October 2020. RESULTS: Recruitment of participants commenced from October 2020, with the aim of enrolling 20 patients over 2 years. Data collection will be completed by the end of 2023, and the results will be published once all data are available for reporting. CONCLUSIONS: The research design, framed within the process of developing and testing innovative interventions, is discussed in line with the aims of the study. The limitations in clinical practice and specific consequences of this study are explained. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/35336.

Crying does not alleviate acute pain perception: Evidence from an experimental study.

BACKGROUND: Whereas previous studies revealed positive effects of emotional expressions such as swearing and laughing on acute pain, systematic research on the effects of crying on pain is missing. The rationale for the current study is that either a mere emotional distraction or changes in oxytocin and opioid levels represent a mechanism through which crying modulates pain, with the timing of mood changes as crucial information for distinguishing between potential mechanisms. METHODS: In two studies, we exposed participants (Study 1: n = 57; Study 2: n = 70) to a sad movie and measured their mood, and exposed them to pain induction procedures (electric shock and cold-pressor test, respectively) before and after the film. Dependent variables were pain threshold, tolerance, and intensity. In addition to baseline and one immediate post-crying mood and pain response measurement in both studies, in Study 2, we repeated these procedures 20 and 50 min later to discern between the potential role of neurobiological substances and distraction. RESULTS: Crying was elicited in 28 participants in Study 1 (49.1%) and 49 (70%) in Study 2. We found no systematic differences in pain and mood changes between criers and non-criers and no systematic dose-response relationship between crying and pain responses and mood. The only significant effects ran contrary to our hypotheses, showing detrimental effects of the occurrence (Study 1) and frequency of crying (both studies) on pain threshold. CONCLUSIONS: Results do not support the idea that crying has pain-alleviating effects, either via distraction or direct biological mechanisms. SIGNIFICANCE: Despite previous findings on pain alleviating effects of emotional expression and the widespread idea about the generally beneficial consequences of emotional crying, research on the possible pain alleviating effects of crying is largely missing. Two quasi-experimental studies demonstrated that crying induced in laboratory conditions does not alleviate acute pain responses, suggesting that role of crying in pain interventions is doubtful. Less directly, results cast light on the role of emotional distraction from acute pain and possible crying-related neurochemical changes.

Neural processing of facial identity and expression in adults with and without autism: A multi-method approach.

The ability to recognize faces and facial expressions is a common human talent. It has, however, been suggested to be impaired in individuals with autism spectrum disorder (ASD). The goal of this study was to compare the processing of facial identity and emotion between individuals with ASD and neurotypicals (NTs). Behavioural and functional magnetic resonance imaging (fMRI) data from 46 young adults (aged 17-23 years, NASD = 22, NNT = 24) was analysed. During fMRI data acquisition, participants discriminated between short clips of a face transitioning from a neutral to an emotional expression. Stimuli included four identities and six emotions. We performed behavioural, univariate, multi-voxel, adaptation and functional connectivity analyses to investigate potential group differences. The ASD-group did not differ from the NT-group on behavioural identity and expression processing tasks. At the neural level, we found no differences in average neural activation, neural activation patterns and neural adaptation to faces in face-related brain regions. In terms of functional connectivity, we found that amygdala seems to be more strongly connected to inferior occipital cortex and V1 in individuals with ASD. Overall, the findings indicate that neural representations of facial identity and expression have a similar quality in individuals with and without ASD, but some regions containing these representations are connected differently in the extended face processing network.

Blood Vessels and Vascular Niches in Bone Development and Physiological Remodeling.

Recent advances in our understanding of blood vessels and vascular niches in bone convey their critical importance in regulating bone development and physiology. The contribution of blood vessels in bone functions and remodeling has recently gained enormous interest because of their therapeutic potential. The mammalian skeletal system performs multiple functions in the body to regulate growth, homeostasis and metabolism. Blood vessels provide support to various cell types in bone and maintain functional niches in the bone marrow microenvironment. Heterogeneity within blood vessels and niches indicate the importance of specialized vascular niches in regulating skeletal functions. In this review, we discuss physiology of bone vasculature and their specialized niches for hematopoietic stem cells and mesenchymal progenitor cells. We provide clinical and experimental information available on blood vessels during physiological bone remodeling.

Bone Vasculature and Bone Marrow Vascular Niches in Health and Disease.

Bone vasculature and bone marrow vascular niches supply oxygen, nutrients, and secrete angiocrine factors required for the survival, maintenance, and self-renewal of stem and progenitor cells. In the skeletal system, vasculature creates nurturing niches for bone and blood-forming stem cells. Blood vessels regulate hematopoiesis and drive bone formation during development, repair, and regeneration. Dysfunctional vascular niches induce skeletal aging, bone diseases, and hematological disorders. Recent cellular and molecular characterization of the bone marrow microenvironment has provided unprecedented insights into the complexity, heterogeneity, and functions of the bone vasculature and vascular niches. The bone vasculature is composed of distinct vessel subtypes that differentially regulate osteogenesis, hematopoiesis, and disease conditions in bones. Further, bone marrow vascular niches supporting stem cells are often complex microenvironments involving multiple different cell populations and vessel subtypes. This review provides an overview of the emerging vascular cell heterogeneity in bone and the new roles of the bone vasculature and associated vascular niches in health and disease. © 2020 The Authors. Journal of Bone and Mineral Research published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research (ASBMR).

Adults with high functioning autism display idiosyncratic behavioral patterns, neural representations and connectivity of the 'Voice Area' while judging the appropriateness of emotional vocal reactions.

Understanding others in everyday situations requires multiple types of information processing (visual, auditory, higher order…) which implicates the use of multiple neural circuits of the human brain. Here, using a multisensory paradigm we investigate one aspect of social understanding less explored in the literature: instead of focusing on the capacity to infer what a specific person is thinking, we explore here how people with high functioning autism (HFA) and matched controls with typical development (TD) infer the "population thinking". For this we created an audio-visual 'social norm inference' task. Participants were required to imagine how most people would judge the appropriateness of vocal utterances in relation to different emotional visual contexts. Behavioral findings demonstrated that HFA individuals show more interindividual variability in these judgments despite equal within-participant reliability relative to TD. This was also the case for judgements of the valence of these vocalizations when presented in isolation. At the neural level, multivoxel pattern analysis of functional magnetic resonance imaging data revealed strikingly similar neural representations between HFA and TD participants at the group level across different hierarchical levels and neural systems. However, analyses at the individual-participant level revealed that the "Temporal Voice Area" (TVA) shows more interindividual variability in the HFA group, both for neural representations and functional connectivity. Thus, this larger neural idiosyncrasy in a high-level auditory area matches with the larger behavioral idiosyncrasy in HFA individuals, when judging auditory valence and its adequacy in different social scenarios. These results suggest that idiosyncrasy in task-relevant sensory areas in HFA participants could underlie their greater difficulties to estimate how others can think.

Intact neural representations of affective meaning of touch but lack of embodied resonance in autism: a multi-voxel pattern analysis study.

Background: Humans can easily grasp the affective meaning of touch when observing social interactions. Several neural systems support this ability, including the theory of mind (ToM) network and the somatosensory system linked to embodied resonance, but it is unclear how these systems are affected in autism spectrum disorder (ASD). Individuals with ASD exhibit impairments in the use of nonverbal communication such as social and reciprocal touch. Despite the importance of touch in social communication and the reported touch aversion in ASD, surprisingly little is known about the neural systems underlying impairments in touch communication in ASD. Methods: The present study applies a dynamic and socially meaningful stimulus set combined with functional magnetic resonance imaging (fMRI) to pinpoint atypicalities in the neural circuitry underlying socio-affective touch observation in adults with ASD. Twenty-one adults with ASD and 21 matched neurotypical adults evaluated the valence and arousal of 75 video fragments displaying touch interactions. Subsequently, they underwent fMRI while watching the same videos. Using multi-voxel pattern analysis (MVPA) and multiple regression analysis, we examined which brain regions represent the socio-affective meaning of observed touch. To further understand the brain-behavior relationship, we correlated the strength of affective representations in the somatosensory cortex with individuals' attitude towards social touch in general and with a quantitative index of autism traits as measured by the Social Responsiveness Scale. Results: Results revealed that the affective meaning of touch was well represented in the temporoparietal junction, a core mentalizing area, in both groups. Conversely, only the neurotypical group represented affective touch in the somatosensory cortex, a region involved in self-experienced touch. Lastly, irrespective of the group, individuals with a more positive attitude towards receiving, witnessing, and providing social touch and with a higher score on social responsivity showed more differentiated representations of the affective meaning of touch in these somatosensory areas. Conclusions: Together, our findings imply that male adults with ASD show intact cognitive understanding (i.e., "knowing") of observed socio-affective touch interactions, but lack of spontaneous embodied resonance (i.e., "feeling").

Correction to: Determinants of the quality of care relationships in long-term care - a systematic review.

In the original publication of this article [1], there is a layout mistake in the column "McCloughen et al. (2011)" of Table 2.

Users' Experiences With Web-Based Health Care Information: Qualitative Study About Diabetes and Dementia Information Presented on a Governmental Website.

BACKGROUND: Information on health and health care is abundant on the internet. To make informed choices, patients need reliable and easy-to-understand information about quality and availability of care providers and treatment options. However, the reliability of such Web-based information is difficult to assess. OBJECTIVE: This study aimed to test Web-based information about diabetes and dementia and specifically a new presentation format of care routes to see if people are able to understand and use the information. METHODS: Overall, 38 cognitive interviews were held; 20 people viewed the information about diabetes and 18 viewed the dementia information. Participants were asked what they would want to know about either diabetes or dementia, what choices they would want to make concerning their preferred care provider and treatment, and what information they would like to find to make these choices. They were then asked to view the relevant pages and comment on them. The interview was focused on general information about the condition, the care route, and the quality information for choosing a hospital. The interviews were transcribed verbatim and then systematically coded and ordered into themes. RESULTS: The themes that were developed for both Web pages during the analysis were information needs, findability, usability, comprehension and readability, recognizability, care route, quality information, and usefulness. Information needs were found to be very diverse and dependent on the personal situation and condition of the participant. Several participants were unable to find specific items because they were not where they expected them to be. Most participants were positive about the layout, font, and color scheme of the test pages. However, options of clicking through to another website and indications where information can be expanded and collapsed could be made clearer. Participants generally found the information easy to understand but felt a need for a more detailed explanation of the medical terms. Recognition of the information played an important role: participants assessed whether the information they found matched their experiences. The term care route meant little to most of the participants, but the layout of the care route itself was found to be clear. Not many respondents spontaneously went to the quality information, and a number of participants had difficulty understanding it. Overall, the participants thought the information on the website was useful and helpful. CONCLUSIONS: The cognitive interviews gave numerous insights into how Web-based information is processed and understood. The care route offers a clear overview of the various stages as the condition progresses, but the name care route is not clear to everyone. We gained insight into differences between subgroups of people in terms of information needs, comprehension, and use of the information because the diversity within the group of participants was lower than expected.

Determinants of the quality of care relationships in long-term care - a participatory study.

BACKGROUND: The quality of the care relationship between a client and a professional is important in long-term care, as most clients depend on support for a lengthy period. The three largest client groups who receive long-term care in the Netherlands are older adults who are physically or mentally frail, people with mental health problems and people with intellectual disabilities. There is little clarity about how generic and variable the determinants of the quality of care relationships are across these client groups. The aim of this study is to explore and compare the determinants of the quality of care relationships in these three client groups in long-term care. METHODS: This participatory study involving clients as co-researchers was held in three healthcare organizations, each providing long-term care to one client group. The research was conducted by three teams consisting of researchers and co-researchers. We interviewed clients individually and professionals in focus groups. The focus was on care relationships with professionals where there is weekly recurring contact for at least 3 months. Clients and professionals were selected using a convenience sample. The interviews were coded in open, axial and selective coding. The outcomes were compared between the client groups. RESULTS: The study sample consisted of 30 clients and 29 professionals. Determinants were categorized into four levels: client, professional, between client and professional, and context. The findings show that the majority of the determinants apply to the care relationships within all three client groups. At the professional level, eleven generic determinants were found. Eight determinants emerged at the client level of which two were found in two client groups only. At the level between a client and a professional, six determinants were found of which one applied to mental healthcare and disability care only. Five determinants were found at the contextual level of which two were specific for two client groups. CONCLUSIONS: The study yielded a variety of determinants that came to the fore in all three client groups in long-term care. This suggests that including a homogenous client group from a single care setting is not necessary when studying the quality of long-term care relationships.

How do doctors in the Netherlands perceive the impact of disciplinary procedures and disclosure of disciplinary measures on their professional practice, health and career opportunities? A questionnaire among medical doctors who received a disciplinary measure.

INTRODUCTION: Disciplinary procedures can have serious consequences for the health, personal life and professional functioning of doctors. Until recently, specific disciplinary measures (reprimands) were publicly disclosed in the Netherlands. The perceived additional impact of disclosing reprimands on the professional and personal life of doctors is unclear. METHODS: All doctors who received a disciplinary measure from the Dutch Disciplinary Board between July 2012 and August 2016 were invited to partake in a 60-item questionnaire concerning the respondents' characteristics, the complaint, experience with the procedure and perceived impact of the procedure on health and professional functioning as reported by doctors themselves. The response rate was 43% (n=210). 21.4% received a reprimand (disclosed); the remainder received a warning (not disclosed). Differences between the two groups were calculated. RESULTS: Respondents with a reprimand reported significantly more negative experiences and impact on health and work than respondents with a warning. 37.8% of the doctors said their health was very good. A small percentage reported moderate-to-severe depressive complaints (3.6%), moderate-to-severe anxiety disorder (2%) or indications of burnout (10.8%). The majority reported changes in their professional practices associated with 'defensive medicine', such as doing more supplementary research (41%) and complying more with patients' wishes (35%). CONCLUSION: The Dutch disciplinary procedure has strong negative side effects, that disclosing measures seems to increase. Dutch disciplinary law aims to contribute to the quality of professional practice. A safe environment is a basic condition for quality improvement and therefore, disclosure of disciplinary measures should be carefully considered. Disclosure of disciplinary measures has always been controversial and the results of this study has rekindled this debate. Recently, a majority in the Dutch House of Representatives has voted against disclosure of reprimands, leaving disclosure of reprimands a discretion of the disciplinary board when deemed appropriate or necessary.

Protocol for a participatory study for developing qualitative instruments measuring the quality of long-term care relationships.

INTRODUCTION: In long-term care (LTC), it is unclear which qualitative instruments are most effective and useful for monitoring the quality of the care relationship from the client's perspective. In this paper, we describe the research design for a study aimed at finding and optimising the most suitable and useful qualitative instruments for monitoring the care relationship in LTC. METHODS AND ANALYSIS: The study will be performed in three organisations providing care to the following client groups: physically or mentally frail elderly, people with mental health problems and people with intellectual disabilities. Using a participatory research method, we will determine which determinants influence the quality of a care relationship and we will evaluate up to six instruments in cooperation with client-researchers. We will also determine whether the instruments (or parts thereof) can be applied across different LTC settings. ETHICS AND DISSEMINATION: This study protocol describes a participatory research design for evaluating the quality of the care relationship in LTC. The Medical Ethics Committee of the Radboud University Nijmegen Medical Centre decided that formal approval was not needed under the Dutch Medical Research Involving Human Subjects Act. This research project will result in a toolbox and implementation plan, which can be used by clients and care professionals to measure and improve the care relationship from the client's perspective. The results will also be published in international peer-reviewed journals.

Determinants of the quality of care relationships in long-term care - a systematic review.

BACKGROUND: The quality of a care relationship between a client and a care professional is seen as fundamental if high-quality care is to be delivered. This study reviews studies about the determinants of the quality of the client-professional relationship in long-term care. METHODS: A systematic review was performed using the electronic databases of Medline, Psycinfo, CINAHL and Embase. The review focused on three client groups receiving long-term care: physically or mentally frail elderly, people with mental health problems and people with physical or intellectual disabilities. Included studies concern clients receiving inpatient or outpatient care and care professionals who provided recurring physical and supporting care for a long period of time. The studies we included contained primary empirical data, were written in English and were published in peer-reviewed journals. Data extraction was carried out by two researchers independently. RESULTS: Thirty-two studies out of 11,339 initial hits met the inclusion criteria. In total, 27 determinants were revealed, six at the client level, twelve at the professional level, six between the client and care professional levels and three at the contextual level. The data analysis showed that most determinants were relevant in more than one client group. CONCLUSIONS: This is the first review that looked at determinants of the quality of the care relationship for three large client groups receiving long-term care. It suggests that the current client group-specific focus in research and quality improvement initiatives for care relationships might not be needed. Care organisations can use the findings of this review as guidance on determinants to look for when mapping the quality of a care relationship in order to get a picture of specific points of attention for quality improvement.

Neural Representations Behind 'Social Norm' Inferences In Humans.

Humans are highly skilled in social reasoning, e.g., inferring thoughts of others. This mentalizing ability systematically recruits brain regions such as Temporo-Parietal Junction (TPJ), Precuneus (PC) and medial Prefrontal Cortex (mPFC). Further, posterior mPFC is associated with allocentric mentalizing and conflict monitoring while anterior mPFC is associated with self-reference (egocentric) processing. Here we extend this work to how we reason not just about what one person thinks but about the abstract shared social norm. We apply functional magnetic resonance imaging to investigate neural representations while participants judge the social congruency between emotional auditory utterances in relation to visual scenes according to how 'most people' would perceive it. Behaviorally, judging according to a social norm increased the similarity of response patterns among participants. Multivoxel pattern analysis revealed that social congruency information was not represented in visual and auditory areas, but was clear in most parts of the mentalizing network: TPJ, PC and posterior (but not anterior) mPFC. Furthermore, interindividual variability in anterior mPFC representations was inversely related to the behavioral ability to adjust to the social norm. Our results suggest that social norm inferencing is associated with a distributed and partially individually specific representation of social congruency in the mentalizing network.

Feeling happy and carefree: a qualitative study on the experiences of parents, medical clowns and healthcare professionals with medical clowns.

PURPOSE: To explore the effect of medical clowns and its relevant actors and conditions. METHOD: Semi-structured interviews were conducted with fourteen parents who had experiences with medical clowns in the Netherlands. Four focus groups were held with seven medical clowns and 25 healthcare professionals. The interviews and focus groups were audio-recorded and transcribed verbatim. Content analysis was used to analyse the data. RESULTS: Concerning the clown effect, we distinguished the following themes: happiness, distraction, carefree feeling and activation. This effect depended upon clown characteristics (appearance, tailoring, low-key play, making an effort); child characteristics (age, autonomy, fear, living cut off from society, communication); parent characteristics (autonomy, attitude); healthcare professional characteristics (attitude, communication); and organizational conditions (timing and planning, collaboration, accessibility, awareness). CONCLUSION: This study shows that medical clowns are of value for children with serious illnesses or mental disabilities in several care settings. An asset of medical clowns is that they tailor their play to the child and situation.Support of and communication with parents and healthcare professionals is critical. The proposed model of the clown effect can help shape future research. The results can help medical clown organizations to enhance their services and optimize clown encounters.

A Multitude of Neural Representations Behind Multisensory "Social Norm" Processing.

Humans show a unique capacity to process complex information from multiple sources. Social perception in natural environment provides a good example of such capacity as it typically requires the integration of information from different sensory systems, and also from different levels of sensory processing. Here, instead of studying one isolate system and level of representation, we focused upon a neuroimaging paradigm which allows to capture multiple brain representations simultaneously, i.e., low and high-level processing in two different sensory systems, as well as abstract cognitive processing of congruency. Subjects performed social decisions based on the congruency between auditory and visual processing. Using multivoxel pattern analysis (MVPA) of functional magnetic resonance imaging (fMRI) data, we probed a wide variety of representations. Our results confirmed the expected representations at each level and system according to the literature. Further, beyond the hierarchical organization of the visual, auditory and higher order neural systems, we provide a more nuanced picture of the brain functional architecture. Indeed, brain regions of the same neural system show similarity in their representations, but they also share information with regions from other systems. Further, the strength of neural information varied considerably across domains in a way that was not obviously related to task relevance. For instance, selectivity for task-irrelevant animacy of visual input was very strong. The present approach represents a new way to explore the richness of co-activated brain representations underlying the natural complexity in human cognition.

Measuring patient outcomes in chronic heart failure: psychometric properties of the Care-Related Quality of Life survey for Chronic Heart Failure (CaReQoL CHF).

BACKGROUND: The Care-Related Quality of Life survey for Chronic Heart Failure (CaReQoL CHF) is a newly developed patient-reported outcome measure (PROM) that measures care-related quality of life in patients diagnosed with chronic heart failure. This study describes the psychometric properties of the questionnaire and its relationship with disease severity and global rating of quality of care. METHOD: Insurance companies selected patients with a recorded diagnosis of chronic heart failure and for whom the hospital submitted a billing statement in the last year. Exploratory factor analysis, Cronbach's alpha and item-rest correlation were used to construct the CaReQoL CHF. Construct validity was assessed by examining the mean values of the CaReQoL CHF scales for the categories of the widely-used New York Heart Association (NYHA) functional classification and by correlating the global rating of quality of care with the CaReQoL CHF scales. RESULTS: One thousand eighteen patients with chronic heart failure filled out the CaReQoL CHF (RR: 35.7%). The CaReQoL CHF consists of 20 items and three scales: social and emotional problems, physical limitations, and being in safe hands. The mean scores of the three scales differed significantly for the NYHA categories, particularly for the social-emotional problems and physical limitation scales. The 'being in safe hands' scale showed a moderate positive correlation with the global rating of quality of care. CONCLUSIONS: The CaReQoL CHF is a concise and valid PROM that matches patients' priorities in healthcare. It adds a new element to existing quality of life questionnaires for patients with chronic heart failure, that is 'being in safe hands' scale. This scale is relevant for patients because they experience anxiety and tension about their condition. Future research should determine whether the CaReQoL CHF can help healthcare providers in daily practice to focus treatment on outcomes of care that are relevant to individual patients.

Development of Refractive Errors-What Can We Learn From Inherited Retinal Dystrophies?

PURPOSE: It is unknown which retinal cells are involved in the retina-to-sclera signaling cascade causing myopia. As inherited retinal dystrophies (IRD) are characterized by dysfunction of a single retinal cell type and have a high risk of refractive errors, a study investigating the affected cell type, causal gene, and refractive error in IRDs may provide insight herein. DESIGN: Case-control study. METHODS: Study Population: Total of 302 patients with IRD from 2 ophthalmogenetic centers in the Netherlands. Reference Population: Population-based Rotterdam Study-III and Erasmus Rucphen Family Study (N = 5550). Distributions and mean spherical equivalent (SE) were calculated for main affected cell type and causal gene; and risks of myopia and hyperopia were evaluated using logistic regression. RESULTS: Bipolar cell-related dystrophies were associated with the highest risk of SE high myopia 239.7; odds ratio (OR) mild hyperopia 263.2, both P < .0001; SE -6.86 diopters (D) (standard deviation [SD] 6.38), followed by cone-dominated dystrophies (OR high myopia 19.5, P < .0001; OR high hyperopia 10.7, P = .033; SE -3.10 D [SD 4.49]); rod dominated dystrophies (OR high myopia 10.1, P < .0001; OR high hyperopia 9.7, P = .001; SE -2.27 D [SD 4.65]), and retinal pigment epithelium (RPE)-related dystrophies (OR low myopia 2.7; P = .001; OR high hyperopia 5.8; P = .025; SE -0.10 D [SD 3.09]). Mutations in RPGR (SE -7.63 D [SD 3.31]) and CACNA1F (SE -5.33 D [SD 3.10]) coincided with the highest degree of myopia and in CABP4 (SE 4.81 D [SD 0.35]) with the highest degree of hyperopia. CONCLUSIONS: Refractive errors, in particular myopia, are common in IRD. The bipolar synapse and the inner and outer segments of the photoreceptor may serve as critical sites for myopia development.