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• W2022794095 abstract "Abstract Leukocyte adhesion deficiency type II (LADII) is a rare inherited disorder of fucose metabolism. Patients with LADII lack fucosylated glycoconjugates, including the carbohydrate ligands of the selectins, leading to an immunodeficiency caused by the lack of selectin-mediated leukocyte-endothelial interactions. A simple and effective therapy has recently been described for LADII, based on the administration of oral fucose. Parallel to this treatment the lack of E- and P-selectin ligands on neutrophils was corrected, and high peripheral neutrophil counts were reduced to normal levels. This study reports that discontinuation of this therapy leads to the complete loss of E-selectin ligands within 3 days and of P-selectin ligands within 7 days. Peripheral neutrophil counts increased parallel to the decrease of selectin ligands. Selectin ligands reappeared promptly after resumption of the fucose therapy, demonstrating a causal relationship between fucose treatment and selectin ligand expression and peripheral neutrophil counts." @default.
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• W2022794095 date "2001-01-01" @default.
• W2022794095 modified "2023-10-14" @default.
• W2022794095 title "Discontinuation of fucose therapy in LADII causes rapid loss of selectin ligands and rise of leukocyte counts" @default.
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• W2022794095 doi "https://doi.org/10.1182/blood.v97.1.330" @default.
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