Correction of leukocyte adhesion deficiency type II with oral fucose.

Journal article

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: Marquardt, T; Lühn, K; Srikrishna, G; Freeze, H; Harms, E

• Publication status: Published
• Journal: Blood
• Volume: 94
• Issue: 12
• Pages: 3976-3985
• Publication date: 1999-12-01
• EISSN: 1528-0020
• ISSN: 0006-4971
• Language: English
• Keywords: Humans; Selectins; Leukocyte-Adhesion Deficiency Syndrome; Administration, Oral; Fucose; Ligands