Continuous mannose infusion in carbohydrate-deficient glycoprotein syndrome type I
Continuous mannose infusion in carbohydrate-deficient glycoprotein syndrome type I
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DOI：
10.1111/j.1651-2227.1997.tb14825.x
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发表时间：
1997-10-01
期刊：
影响因子：
3.8
通讯作者：
Nutzenadel, W
中科院分区：
文献类型：
--
作者：
Mayatepek, E;Schroder, M;Nutzenadel, W
对血清糖蛋白的等电聚焦模式的影响进行了研究，在CDG综合征I型和磷酸甘露变位酶缺乏症的患者在3周的连续静脉甘露糖输注。5.7 g/kg/天剂量导致血清甘露糖水平稳定，高达2.0 mmol/l，耐受性良好，无肝或肾毒性体征。虽然大多数病理性糖蛋白模式，包括α（1）-抗胰蛋白酶，典型的CDG综合征I型保持不变，甘露糖输注导致血清唾液转铁蛋白的等电聚焦模式的独特变化，治疗3周后出现两个额外的条带。
The effects on isoelectrofocusing patterns of serum glycoproteins were studied in a patient with CDG syndrome type I and phosphomannomutase deficiency during 3 weeks of continuous intravenous mannose infusion. Doses of 5.7 g/kg/day led to stable serum mannose levels up to 2.0 mmol/l and were well tolerated without signs of liver or renal toxicity. While most of the pathological glycoprotein patterns, including alpha(1)-antitrypsin, typical for CDG syndrome type I remained unchanged, mannose infusion led to a unique change of the isoelectrofocusing pattern of serum sialotransferrins with appearance of two extra bands after 3 weeks of treatment.