RESUMEN
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.
Asunto(s)
Calidad de Vida , Esclerodermia Localizada , Adolescente , Adulto , Imagen Corporal , Cuidadores , Niño , Grupos Focales , HumanosAsunto(s)
Fármacos Dermatológicos/uso terapéutico , Dermatosis Facial/tratamiento farmacológico , Isotretinoína/uso terapéutico , Cuero Cabelludo/patología , Enfermedades Cutáneas Papuloescamosas/tratamiento farmacológico , Adulto , Alopecia/complicaciones , Dermatosis Facial/complicaciones , Femenino , Fibrosis/complicaciones , Humanos , Persona de Mediana Edad , Inducción de Remisión , Enfermedades Cutáneas Papuloescamosas/complicacionesRESUMEN
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.
Asunto(s)
Pacientes/psicología , Evaluación de Programas y Proyectos de Salud/normas , Pérdida de Peso , Adulto , Anciano , Terapia Conductista/métodos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Evaluación de Programas y Proyectos de Salud/métodos , Automanejo/métodosAsunto(s)
Adenocarcinoma/secundario , Neoplasias de la Próstata/patología , Neoplasias Cutáneas/secundario , Adenocarcinoma/tratamiento farmacológico , Adenocarcinoma/patología , Adenocarcinoma/cirugía , Anciano de 80 o más Años , Anilidas/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Biopsia , Terapia Combinada , Resultado Fatal , Goserelina/administración & dosificación , Humanos , Metástasis Linfática , Masculino , Nitrilos/administración & dosificación , Neoplasias de la Próstata/tratamiento farmacológico , Neoplasias de la Próstata/cirugía , Neoplasias Cutáneas/patología , Úlcera Cutánea/etiología , Compuestos de Tosilo/administración & dosificación , Resección Transuretral de la PróstataAsunto(s)
Hiperpigmentación/complicaciones , Melanosis/complicaciones , Enfermedades Cutáneas Genéticas/complicaciones , Enfermedades Cutáneas Papuloescamosas/complicaciones , Femenino , Humanos , Hiperpigmentación/patología , Melanosis/patología , Persona de Mediana Edad , Enfermedades Cutáneas Genéticas/patología , Enfermedades Cutáneas Papuloescamosas/patologíaRESUMEN
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.