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Continuous mannose infusion in carbohydrate‐deficient glycoprotein syndrome type I
Author(s) -
Mayatepek E.,
Schröder M.,
Kohlmüller D.,
Bieger WP,
Nützenadel W.
Publication year - 1997
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1997.tb14825.x
Subject(s) - glycoprotein , medicine , mannose , pathological , endocrinology , toxicity , biochemistry , biology
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/1 and were well tolerated without signs of liver or renal toxicity. While most of the pathological glycoprotein patterns, including α 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.