Exploring the impact of paediatric localized scleroderma on health-related quality of life: focus groups with youth and caregivers.

BACKGROUND: Paediatric localized scleroderma (LS) can negatively impact health-related quality of life (HRQoL) by causing skin fibrosis, abnormal limb development, disfigurement, and side-effects from immunosuppressive treatment. Studies to date have rarely included qualitative data gathered directly from paediatric patients with LS. OBJECTIVES: To assess the impact of LS on HRQoL among affected youth and their caregivers using qualitative description. METHODS: Youth with all subtypes of LS and their caregivers were purposively sampled to participate in age-appropriate focus groups (younger children, early adolescents, adolescents). Each group started with a drawing exercise followed by in-depth discussion of topics including skin symptoms (e.g. itch, pain, tightness), functional impairment, physical appearance, family and peer relationships, and treatment burden. Focus groups were transcribed verbatim and co-coded, with adjudication of differentially applied codes. The study findings were triangulated via comparison with adult reports and published literature. RESULTS: Eleven youth aged 9-16 years and 16 caregivers participated in three focus groups each. Major identified areas of impact included uncomfortable skin symptoms, physical functioning limitations, extracutaneous manifestations, body image, bullying and teasing, unwanted questioning from others, and treatment side-effects and burden. CONCLUSIONS: This is the first qualitative study of HRQoL in LS to include all major LS subtypes. We identified domains of HRQoL impacted by LS, some of which replicate earlier findings and some of which were novel. As impact also changed with developmental stage, our findings support the need for ongoing, formal evaluation of HRQoL in children and adolescents with LS. What is already known about this topic? Paediatric localized scleroderma (LS) negatively impacts health-related quality of life (HRQoL) via skin fibrosis, musculoskeletal and other extracutaneous manifestations from the disease process, and side-effects of systemic immunosuppression. The full impact of LS and its treatment on HRQoL is incompletely understood, with only one published qualitative study of youth with LS, which was limited to facial involvement. There are no qualitative studies of HRQoL in other LS subtypes to date. What does this study add? This is the first qualitative evaluation of HRQoL in youth with LS inclusive of all disease subtypes. Our study confirms that LS affects HRQoL across multiple distinct domains, including uncomfortable skin sensations, impacts on body image, bullying and teasing from peers, unwanted intrusive questioning, physical limitations, extracutaneous manifestations and high treatment burden. These results indicate the need for ongoing clinical assessment of paediatric patients in these domains. What are the clinical implications of the work? These results support the need to care for patients with LS holistically by synthesizing cutaneous, musculoskeletal and extracutaneous disease assessments with multidimensional evaluation of psychosocial impact and adverse effects of treatments. The development of an LS-specific HRQoL measure would advance such efforts.

Experiences of Daily Weighing Among Successful Weight Loss Individuals During a 12-Month Weight Loss Study.

The purpose of the study was to describe participants' experience of daily weighing and to explore factors influencing adherence to daily weighing among individuals who were successful in losing weight during a behavioral weight loss intervention. Participants completed a 12-month weight loss intervention study that included daily self-weighing using a Wi-Fi scale. Individuals were eligible to participate regardless of their frequency of self-weighing. The sample ( N = 30) was predominantly female (83.3%) and White (83.3%) with a mean age of 52.9 ± 8.0 years and mean body mass index of 33.8 ± 4.7 kg/m2. Five main themes emerged: reasons for daily weighing (e.g., feel motivated, being in control), reasons for not weighing daily (e.g., interruption of routine), factors that facilitated weighing, recommendations for others about daily weighing, and suggestions for future weight loss programs. Our results identified several positive aspects to daily self-weighing, which can be used to promote adherence to this important weight loss strategy.

Acral Hemorrhagic Darier Disease. / Enfermedad de Darier acral hemorrágica.

Darier disease is an autosomal-dominant inherited condition caused by mutation of a gene, which produces a protein involved in calcium channel regulation. The disease has a variety of manifestations and lacks consistent genotype-phenotype correlations. Acral hemorrhagic Darier disease causes macules, papules, vesicles and/or hemorrhagic blisters on the extremities. Other classic signs of the disease may be present in the same patient or relatives. Histopathology reveals dyskeratosis and suprabasal acantholysis with hemorrhagic lacunae. We report 3 new cases of this type of Darier disease triggered by injuries. Response to retinoid therapy was good.

Patterns of self-weighing behavior and weight change in a weight loss trial.

BACKGROUND/OBJECTIVES: Regular self-weighing has been associated with weight loss and maintenance in adults enrolled in a behavioral weight loss intervention; however, few studies have examined the patterns of adherence to a self-weighing protocol. The study aims were to (1) identify patterns of self-weighing behavior; and (2) examine adherence to energy intake and step goals and weight change by self-weighing patterns. SUBJECTS/METHODS: This was a secondary analysis of self-monitoring and assessment weight data from a 12-month behavioral weight loss intervention study. Each participant was given a scale that was Wi-Fi-enabled and transmitted the date-stamped weight data to a central server. Group-based trajectory modeling was used to identify distinct classes of trajectories based on the number of days participants self-weighed over 51 weeks. RESULTS: The sample (N=148) was 90.5% female, 81.1% non-Hispanic white, with a mean (s.d.) age of 51.3 (10.1) years, had completed an average of 16.4 (2.8) years of education and had mean body mass index of 34.1 (4.6) kg m(-2). Three patterns of self-weighing were identified: high/consistent (n=111, 75.0% self-weighed over 6 days per week regularly); moderate/declined (n=24, 16.2% declined from 4-5 to 2 days per week gradually); and minimal/declined (n=13, 8.8% declined from 5-6 to 0 days per week after week 33). The high/consistent group achieved greater weight loss than either the moderate/declined and minimal/declined groups at 6 months (-10.19%±5.78%, -5.45%±4.73% and -2.00%±4.58%) and 12 months (-9.90%±8.16%, -5.62%±6.28% and 0.65%±3.58%), respectively (P<0.001). The high/consistent group had a greater mean number days per week of adherence to calorie intake goal or step goal but not higher than the moderate/declined group. CONCLUSIONS: This is the first study to reveal distinct temporal patterns of self-weighing behavior. The majority of participants were able to sustain a habit of daily self-weighing with regular self-weighing leading to weight loss and maintenance as well as adherence to energy intake and step goals.