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Discontinuation of fucose therapy in LADII causes rapid loss of selectin ligands and rise of leukocyte counts.

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 neut...

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Publication status:
Published

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Publisher copy:
10.1182/blood.v97.1.330

Authors


Journal:
Blood
Volume:
97
Issue:
1
Pages:
330-332
Publication date:
2001-01-05
DOI:
EISSN:
1528-0020
ISSN:
0006-4971
URN:
uuid:eabff39f-3fdf-4a7e-afc4-01586795fc0b
Source identifiers:
31800
Local pid:
pubs:31800

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