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Acta Paediatr. 1997 Oct;86(10):1138-40.

Continuous mannose infusion in carbohydrate-deficient glycoprotein syndrome type I.

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Division of Metabolic Diseases, University Children's Hospital, Heidelberg, Germany.


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 alpha1-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.

[Indexed for MEDLINE]

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