RESUMEN
OBJECTIVE: To assess cognitive, behavioral, and adaptive functions in children and young adults with hemophilia treated according to contemporary standards of care. STUDY DESIGN: Evolving Treatment of Hemophilia's Impact on Neurodevelopment, Intelligence, and Other Cognitive Functions (eTHINK) is a US-based, prospective, cross-sectional, observational study (September 2018 through October 2019). Males (aged 1-21 years) with hemophilia A or B of any severity, with or without inhibitors, were eligible. Participants underwent neurologic examinations and age-appropriate neuropsychological assessments, including standardized tests/ratings scales of early development, cognition, emotional/behavioral adjustment, and adaptive skills. RESULTS: Five hundred and fifty-one males with hemophilia A (n = 433) or B (n = 101) were enrolled. Performance on cognitive tests was largely comparable with that of age-matched US population norms, although participants in certain age groups (4-5 and 10-21 years) performed worse on measures of attention and processing speed. Furthermore, adolescents and young adults and those with comorbid attention-deficit/hyperactivity disorder (ADHD; n = 64) reported more adaptive and executive function problems in daily life. Incidence of ADHD in adolescents (21%) was higher than expected in the general population. CONCLUSIONS: In general, males with hemophilia demonstrated age-appropriate intellectual, behavioral, and adaptive development. However, specific patient/age groups showed poorer attention performance and concerns for executive and adaptive development. This study established a normative data set for monitoring neurodevelopment in individuals with hemophilia and highlight the importance of screening and intervention for challenges with cognitive and adaptive skills in this population. CLINICAL TRIAL REGISTRATION: Evolving Treatment of Hemophilia's Impact on Neurodevelopment, Intelligence, and Other Cognitive Functions (eTHINK); NCT03660774; https://clinicaltrials.gov/ct2/show/NCT03660774.
Asunto(s)
Hemofilia A , Humanos , Hemofilia A/complicaciones , Masculino , Adolescente , Niño , Estudios Transversales , Estudios Prospectivos , Adulto Joven , Preescolar , Lactante , Cognición , Pruebas Neuropsicológicas , Hemofilia B/complicaciones , Trastorno por Déficit de Atención con Hiperactividad , Función Ejecutiva , Adaptación PsicológicaRESUMEN
The purpose of this study was to develop and test two interventions designed to improve provider compliance with diabetes management guidelines: the use of a diabetes management flowsheet inserted into patient charts and the use of a diabetes management flowsheet plus quarterly provider feedback about compliance levels. Diabetic patient charts from six family practice clinics were randomly selected and audited at baseline and at 12 months. The analysis indicated that the use of the flowsheet was associated with improved provider compliance in the completion of foot examinations only. Providers involved in the study believed that the process of the flowsheet plus feedback contributed to their greater awareness of diabetes management guidelines.