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. 1999 Dec 15;94(12):3976-85.

Correction of leukocyte adhesion deficiency type II with oral fucose

T Marquardt  1 , K Lühn, G Srikrishna, H H Freeze, E Harms, D Vestweber
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  • PMID: 10590041
Free article

Correction of leukocyte adhesion deficiency type II with oral fucose

T Marquardt et al. Blood. .
Free article

Abstract

We describe a simple, noninvasive, and effective therapy for leukocyte adhesion deficiency type II (LAD II), a rare inherited disorder of fucose metabolism. This disorder leads to an immunodeficiency caused by the absence of carbohydrate-based selectin ligands on the surface of neutrophils as well as to severe psychomotor and mental retardation. The fucosylation defect in LAD II fibroblasts can be corrected by addition of L-fucose to the culture medium. This prompted us to initiate dietary fucose therapy on a patient with LAD II. Oral supplementation of fucose in this patient induced the expression of fucosylated selectin ligands on neutrophils and core fucosylation of serum glycoproteins. During 9 months of treatment, infections and fever disappeared, elevated neutrophil counts returned to normal, and psychomotor capabilities improved.

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