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
Artículo
en Inglés
| 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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Índice:
LILACS (Américas)
Asunto principal:
Sulfatasas
/
Mucopolisacaridosis II
/
Glicosaminoglicanos
/
Leucocitos
Tipo de estudio:
Investigación cualitativa
Límite:
Niño
/
Child, preschool
/
Humanos
/
Masculino
Idioma:
Inglés
Revista:
Arch. med. res
Asunto de la revista:
Medicina
Año:
1997
Tipo del documento:
Artículo
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