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Arch. med. res ; 28(1): 91-4, mar. 1997. tab, ilus
Artigo em Inglês | LILACS | ID: lil-225202

RESUMO

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


Assuntos
Humanos , Masculino , Pré-Escolar , Criança , Glicosaminoglicanos/urina , Leucócitos/enzimologia , Mucopolissacaridose II/metabolismo , Sulfatases/deficiência
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