FUNCTIONAL INDEPENDENCE OF PEDIATRIC PATIENTS WITH MUCOPOLYSACCHARIDOSES

Author:

Lopes Paloma Silva1ORCID,Serra Filho Diógenes Pires1,Matos Marcos Antônio Almeida2ORCID

Affiliation:

1. Escola Bahiana de Medicina e Saúde Pública, Brazil

2. Escola Bahiana de Medicina e Saúde Pública, Brazil; Santa Casa de Misericórdia da Bahia, Brazil

Abstract

ABSTRACT Objective: To measure the functional independence to perform activities of daily living of pediatric patients diagnosed with mucopolysaccharidoses. Methods: A descriptive cross-sectional study was carried out with the population of pediatric patients with a confirmed enzymatic diagnosis of mucopolysaccharidoses, enrolled in the Orthopedics outpatient clinic of a hospital in the State of Bahia. The data were collected between October 2016 and March 2017, based on the documentary analysis of the assessment forms used in the department. The variables of this study comprised sex, age, type of MPS and level of functional independence, measured by the Functional Independence Measure scale. Results: Twenty-six patients participated in the study. These were predominantly male (61.5%), with a mean age of 10 ± 4.5 years, affected by MPS VI (73.1%). In the motor domain, the mean score was 65 (± 19.9 points); the cognitive domain obtained a mean score equal to 28 (± 8.2 points); and the total FIM score was 93 (± 26.5). Conclusion: Impaired functional independence was observed among children and adolescents with mucopolysaccharidoses. Tasks related to dressing, toileting, bathing, problem solving and social interaction were those that required the most assistance and/or supervision. Level of Evidence IV, Case Series.

Publisher

FapUNIFESP (SciELO)

Subject

Rehabilitation,Physical Therapy, Sports Therapy and Rehabilitation,Orthopedics and Sports Medicine

Reference19 articles.

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3. Diagnosis, quality of life, and treatment of patients with Hunter syndrome in the French healthcare system: a retrospective observational study;Guffon N;Orphanet J Rare Dis,2015

4. Assessment of musculoskeletal abnormalities in children with mucopolysaccharidoses using pGALS;Chan MO;Pediatr Rheumatol Online J,2014

5. Diagnosis of the mucopolysaccharidoses;Lehman TJ;Rheumatology (Oxford),2011

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