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
en En
| LILACS
| ID: lil-225202
Biblioteca responsable:
MX1.1
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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Índice:
LILACS
Asunto principal:
Sulfatasas
/
Mucopolisacaridosis II
/
Glicosaminoglicanos
/
Leucocitos
Tipo de estudio:
Qualitative_research
Límite:
Child
/
Child, preschool
/
Humans
/
Male
Idioma:
En
Revista:
Arch. med. res
Asunto de la revista:
MEDICINA
Año:
1997
Tipo del documento:
Article