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Lookup NU author(s): Professor Hermann Josef Vormoor
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Leukocyte adhesion deficiency II has been described in only 2 patients; herein we report extensive investigation of another patient. The physical stigmata were detected during prenatal ultrasonographic investigation. Sialyl-Lewis X (sLex) was absent from the surface of polymorphonuclear neutrophils, and cell binding to E- and P-selectin was severely impaired, causing an immunodeficiency. The elevation of peripheral neutrophil counts occurred within several days after birth. A severe hypofucosylation of glycoconjugates bearing fucose in different glycosidic links was present in all cell types investigated, demonstrating that leukocyte adhesion deficiency II is not only a disorder of leukocytes but a generalized inherited metabolic disease affecting the metabolism of fucose.
Author(s): Marquardt T, Brune T, Lühn K, Zimmer KP, Körner C, Fabritz L, van der Werft N, Vormoor J, Freeze HH, Louwen F, Biermann B, Harms E, von Figura K, Vestweber D, Koch HG
Publication type: Article
Publication status: Published
Journal: Journal of Pediatrics
Year: 1999
Volume: 134
Issue: 6
Pages: 681-688
Print publication date: 01/06/1999
ISSN (print): 0022-3476
ISSN (electronic): 1097-6833
URL: http://dx.doi.org/10.1016/S0022-3476(99)70281-7
DOI: 10.1016/S0022-3476(99)70281-7
Notes: 0022-3476 (Print) Case Reports Journal Article
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