Gallegos-Arreola M P, González-Noriega A, Zúñiga-González G M, Flores-Martínez S E, Morán Moguel M C, Figuera L E, Sánchez-Corona J
División de Medicina Molecular, Centro de Investigación Biomédica de Occidente, Instituto Mexicano del Seguro Social, Guadalajara, Jal., México.
Arch Med Res. 1997 Spring;28(1):91-4.
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 increased. Electrophoresis revealed the presence of heparan sulfate (HS) and dermatan sulfate (DS) in four cases (80%), 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 this syndrome.
对5例呈现亨特综合征的患者进行了生化研究。进行了尿糖胺聚糖（GAGs）定量、电泳特征分析以及与白细胞酶活性的相关性研究。在所有病例中，尿GAGs/肌酐比值均升高。电泳显示4例（80%）存在硫酸乙酰肝素（HS）和硫酸皮肤素（DS），但其余患者仅存在DS。所有患者均表现出酶活性不足。这些结果显示了该综合征存在生化差异的证据。
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