Mucopolysaccharidoses type II: Enzymatic activity and quantitative and qualitative studies of urinary glycosaminoglycans in five patients
Arch. med. res
;
28(1): 91-4, mar. 1997. tab, ilus
Article
in English
| LILACS
| ID: lil-225202
ABSTRACT
Five patients presenting Hunter's syndrome were biochemically studied. Quantification of urinary glycosaminoglycans (GAGs), electrophoretic characterizatio and correlation with ensymatic activity in leucocytes were carried out. In all cases, urinary GAGs/creatinine ratio was increased. Electrophoresis revealed the presence of heparan sulfate (HS) and dermatan sulfate (DS) in four cases (80 perecent), but in the remaining patient, only DS was present. In all patients, deficient enzymatic activity was demonstrated. These results show evidences of biochemical differences in thys syndrome
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Index:
LILACS (Americas)
Main subject:
Sulfatases
/
Mucopolysaccharidosis II
/
Glycosaminoglycans
/
Leukocytes
Type of study:
Qualitative research
Limits:
Child
/
Child, preschool
/
Humans
/
Male
Language:
English
Journal:
Arch. med. res
Journal subject:
Medicine
Year:
1997
Type:
Article
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