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
Artigo
em Inglês
| 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)
Assunto principal:
Sulfatases
/
Mucopolissacaridose II
/
Glicosaminoglicanos
/
Leucócitos
Tipo de estudo:
Pesquisa qualitativa
Limite:
Criança
/
Criança, pré-escolar
/
Humanos
/
Masculino
Idioma:
Inglês
Revista:
Arch. med. res
Assunto da revista:
Medicina
Ano de publicação:
1997
Tipo de documento:
Artigo
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