MUCOPOLYSACCHARIDOSES TYPE-II - ENZYMATIC-ACTIVITY AND QUANTITATIVE AND QUALITATIVE STUDIES OF URINARY GLYCOSAMINOGLYCANS IN 5 PATIENTS

Authors
GALLEGOSARREOLA MP GONZALEZNORIEGA A ZUNIGAGONZALEZ GM FLORESMARTINEZ SE MOGUEL MCM FIGUERA LE SANCHEZCORONA J
Citation
Mp. Gallegosarreola et al., MUCOPOLYSACCHARIDOSES TYPE-II - ENZYMATIC-ACTIVITY AND QUANTITATIVE AND QUALITATIVE STUDIES OF URINARY GLYCOSAMINOGLYCANS IN 5 PATIENTS, Archives of medical research, 28(1), 1997, pp. 91-94
Citations number
11
Categorie Soggetti
Medicine, Research & Experimental
Journal title
Archives of medical research → ACNP
ISSN journal
01884409
Volume
28
Issue
1
Year of publication
1997
Pages
91 - 94
Database
ISI
SICI code
0188-4409(1997)28:1<91:MT-EAQ>2.0.ZU;2-U
Abstract
Five patients presenting Hunter's syndrome were biochemically studied. Quantification of urinary glycosaminoglycans (GAGs), electrophoretic characterization and correlation with enzymatic activity in leucocytes were carried out. In all cases, urinary GAGs/creatinine ratio was inc reased. Electrophoresis revealed the presence of heparan sulfate (HS) and dermatan sulfate (DS) in four cases (80%), but in the remaining pa tient, only DS was present. In all patients, deficient enzymatic activ ity was demonstrated. These results show evidences of biochemical diff erences in this syndrome.