QUALITY OF LIFE ASSESSMENT INSTRUMENTS IN CHILDREN AND ADOLESCENTS WITH NEUROMUSCULAR DISEASES: A SYSTEMATIC SCOPE REVIEW
Creators: name: Cruz, Karoliny Lisandra Teixeira
name: Santos, Isadora
Publisher: name: figshare
Year: 2022
Subjects: Neuroscience

description: <strong>INTRODUCTION:</strong> Neuromuscular diseases (NMD) comprise a set of hereditary or acquired and progressive diseases that affect anterior horn cells, peripheral nerves, neuromuscular junction and skeletal muscle (KENNEDY et al., 2020). In general, they manifest in the first years of life, linked to functional disability and dependence on specialized therapies (MERCURI et al., 2012; WANG et al., 2012). Due to the use of different instruments to assess the quality of life of children with NMD, it is necessary to develop a scope review in order to explore and identify the evidence and characteristics of each instrument available in the literature on this issue beyond the possibility of the present study be a precursor to a future systematic review. <strong>OBJECTIVE:</strong> (1) To identify the main instruments used to assess the quality of life in children and adolescents with neuromuscular disease; (2) Check the validation of instruments for the pediatric population.<strong> METHODS: </strong>This is a scoping review, which consists of mapping key concepts, with relevance to location, time, origin and source, regardless of the quality of the articles, conducted according to the methodology proposed by Joanna Briggs Institute Reviewers Manual (JBI, 2015). The searches took place between March and April 2022, and were carried out in 6 electronic databases: Embase, Scielo, Scopus, Pubmed and Lilacs. The gray literature being searched on Google, ending in “file:pdf” and expert reference. Experimental, descriptive and observational articles, published in English, Portuguese and Spanish, in the last 10 years (2012 to 2022), addressing the quality of life of children and adolescents (age 0 to 18 years) with a clinical diagnosis of disease were included. neuromuscular. The excluded studies were: those made up of different age groups (adults and children), those that investigated the caregiver's and/or family's quality of life, those that provided limited information regarding the QOL instrument, those related to the diagnosis of cancer, as well as , unpublished dissertations, clinical trials of drugs/therapies and surgeries (pre and post operative). <strong>RESULTS: </strong>Seven hundred and eighty-six (786) articles were identified in the databases and 72 articles were selected, following the eligibility criteria. Among the 72 articles that were fully read, 15 were included in this review. The number of participants included in the studies ranged from 35 (Kocova, et al., 2014) to 221 (Zamani, et al., 2016), totaling 941. Regarding ages, the sample consisted of minimum ages of 2 and maximum of 18 years. Five different instruments were found to assess the quality of life in children and adolescents with neuromuscular diseases. The most used instrument was the “Pediatric Quality of Life (PedsQLTM) Inventory 3.0 Neuromuscular Module” (n=6). Only 5 studies provided figures on reliability. All studies that used the showed good reliability showed good internal reliability (Cronbach's alpha).