SciELO - Scientific Electronic Library Online

vol.84 issue2The prevalence of symptoms of sleep-disordered breathing in Brazilian schoolchildrenDetermination of glycemia and insulinemia and the homeostasis model assessment (HOMA) in schoolchildren and adolescents with normal body mass index author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand




Related links


Jornal de Pediatria

Print version ISSN 0021-7557On-line version ISSN 1678-4782


CARDOSO-SANTOS, Antonio et al. Mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome): assessment of joint mobility and grip and pinch strength. J. Pediatr. (Rio J.) [online]. 2008, vol.84, n.2, pp.130-135. ISSN 0021-7557.

OBJECTIVE: To describe the profile of joint mobility and grip and pinch strength of MPS VI patients and to correlate this with urinary excretion of glycosaminoglycans (GAGs), ARSB activity, and the distance covered in a 6-minute walking test (6MWT). METHODS: This was an observational study of 28 patients with MPS VI, who had not undergone specific treatment. All patients were assessed for amplitude of joint mobility (shoulder, elbow, and knee), grip and pinch strength and urinary GAG excretion and also performed the 6MWT. RESULTS: Shoulder flexion exhibited the greatest limitation, with no correlation with age, followed by knee extension and elbow flexion, both of which were correlated inversely with age. Hand grip strength was compromised in all patients, and pinch strength exhibited a positive correlation with age. CONCLUSIONS: The fact that restricted shoulder flexion was not correlated with age suggests that this finding is present early on in MPS VI and that it constitutes an important clinical sign that should arouse diagnostic suspicion of this disease. The amplitude of knee extension and elbow flexion, in turn, are possible markers of disease progression since they have a negative correlation with age. Further studies are needed to confirm these hypotheses.

Keywords : Mucopolysaccharidosis type VI; joint mobility; muscle strength.

        · abstract in Portuguese     · text in English | Portuguese     · English ( pdf epdf ) | Portuguese ( pdf epdf )


Creative Commons License All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License