Correction of leukocyte adhesion deficiency type II with oral fucose.

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.

• Authors: T Marquardt, K Lühn, G Srikrishna, H H Freeze, E Harms, D Vestweber
• Journal: Blood (Blood) Vol. 94 Issue 12 Pg. 3976-85 (Dec 15 1999) ISSN: 0006-4971 [Print] United States
• PMID: 10590041 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Ligands
• Selectins
• Fucose

Topics

• Administration, Oral
• Fucose (administration & dosage, therapeutic use)
• Humans
• Leukocyte-Adhesion Deficiency Syndrome (drug therapy, metabolism)
• Ligands
• Selectins (metabolism)