Document Detail

Mucopolysaccharidosis type VI (Maroteaux-Lamy syndrome): assessment of joint mobility and grip and pinch strength.
MedLine Citation:
PMID:  18338089     Owner:  NLM     Status:  MEDLINE    
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.
Antonio Cardoso-Santos; Ana C M M Azevedo; Simone Fagondes; Maira G Burin; Roberto Giugliani; Ida V D Schwartz
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Publication Detail:
Type:  Journal Article     Date:  2008-03-18
Journal Detail:
Title:  Jornal de pediatria     Volume:  84     ISSN:  0021-7557     ISO Abbreviation:  J Pediatr (Rio J)     Publication Date:    2008 Mar-Apr
Date Detail:
Created Date:  2008-03-31     Completed Date:  2008-09-04     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  2985188R     Medline TA:  J Pediatr (Rio J)     Country:  Brazil    
Other Details:
Languages:  eng     Pagination:  130-5     Citation Subset:  IM    
Faculdade de Medicina, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.
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MeSH Terms
Elbow Joint / physiopathology
Glycosaminoglycans / urine
Hand Strength / physiology*
Joint Instability / diagnosis,  etiology,  physiopathology*
Knee Joint / physiopathology
Mucopolysaccharidosis VI / complications,  metabolism,  physiopathology*
N-Acetylgalactosamine-4-Sulfatase / blood
Reference Values
Shoulder Joint / physiopathology
Reg. No./Substance:
0/Glycosaminoglycans; EC
Comment In:
J Pediatr (Rio J). 2008 Mar-Apr;84(2):103-6   [PMID:  18372931 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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