RESUMO
This study evaluates hand functioning in patients with mucopolysaccharidosis (MPS) and validates the Michigan Hand Outcomes Questionnaire (MHQ) as a measure of hand functioning evaluation. Patients with diagnosis of MPS between the ages of 8 and 21 years were eligible for enrolment in the study irrespective of whether they were or were not receiving treatment (enzyme replacement therapy). Individuals with mental disorders and those who had already undergone hand surgery were excluded. Clinical and demographic data were collected as well as hand functioning evaluation based on the Brazilian-Portuguese version of the MHQ. The global score and domains score of the entire group is presented. With regard to the validation of the MHQ, internal consistency of the domains were evaluated using Cronbach's α correlation coefficient. Ten individuals were women and six were men; two were type II MPS and 14 were type VI. Mean age was 11.7 ± 4.4 years and five patients had signs and symptoms suggestive of carpal tunnel syndrome. The MHQ global and domains mean scores were: global, 56.68 ± 16.17; hand function, 64.17 ± 21.62; activities of daily living, 46.87 ± 27.78; school performance, 56.01 ± 32.69; pain, 79.33 ± 30.87; aesthetics, 77.81 ± 11.16; and satisfaction, 74.57 ± 27.16. Global and domain internal consistency were: global, 0.888 (good); hand function, 0.815 (good); activities of daily living, 0.873 (good); school performance, 0.815 (good); pain, 0.468 (poor); aesthetics, -0.044 (unacceptable); and satisfaction.
