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PLoS One ; 12(6): e0178639, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28644838

RESUMO

The neurofibromatoses (neurofibromatosis type 1, neurofibromatosis type 2 and schwannomatosis) are rare disorders having clinical manifestations that vary greatly from patient to patient. The rarity and variability of these disorders has made it challenging for investigators to identify sufficient numbers of patients with particular clinical characteristics or specific germline mutations for participation in interventional studies. Similarly, because the natural history of all types of neurofibromatosis (NF) is variable and unique for each individual, it is difficult to identify meaningful clinical outcome measures for potential therapeutic interventions. In 2012, the Children's Tumor Foundation created a web-based patient-entered database, the NF Registry, to inform patients of research opportunities for which they fit general eligibility criteria and enable patients to contact investigators who are seeking to enroll patients in approved trials. Registrants were recruited through CTF-affiliated NF clinics and conferences, through its website, and by word-of-mouth and social media. Following online consent, demographic information and details regarding manifestations of NF were solicited on the Registry website. Statistical analyses were performed on data from a cohort of 4680 registrants (the number of registrants as of October 9, 2015) who met diagnostic criteria for one of the 3 NF conditions. The analyses support our hypothesis that patient-reported symptom incidences in the NF Registry are congruent with published clinician-sourced data. Between April 26, 2013 and July 8, 2016, the registry has been useful to investigators in recruitment, particularly for observational trials, especially those for development of patient-reported outcomes.


Assuntos
Internet , Neurofibromatoses , Sistema de Registros , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Pesquisa Biomédica/métodos , Criança , Pré-Escolar , Ensaios Clínicos como Assunto , Confidencialidade , Curadoria de Dados , Feminino , Humanos , Lactente , Internacionalidade , Masculino , Pessoa de Meia-Idade , Neurofibromatoses/epidemiologia , Participação do Paciente , Controle de Qualidade , Inquéritos e Questionários , Adulto Jovem
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