scholarly journals Leukocyte adhesion deficiency type II

2020 ◽  
Author(s):  
Keyword(s):  
Leukocyte Adhesion ◽  
Type Ii ◽  
Leukocyte Adhesion Deficiency ◽  
Deficiency Type

Fucose supplementation in leukocyte adhesion deficiency type II

Blood ◽  
2000 ◽  
Vol 95 (11) ◽  
pp. 3641-3643 ◽  
Author(s):  
Amos Etzioni ◽  
Michela Tonetti
Keyword(s):  
Leukocyte Adhesion ◽  
Type Ii ◽  
Leukocyte Adhesion Deficiency ◽  
Deficiency Type

Correction of Leukocyte Adhesion Deficiency Type II With Oral Fucose

Blood ◽  
1999 ◽  
Vol 94 (12) ◽  
pp. 3976-3985 ◽  
Author(s):  
Thorsten Marquardt ◽  
Kerstin Lühn ◽  
Geetha Srikrishna ◽  
Hudson H. Freeze ◽  
Erik Harms ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Culture Medium ◽  
Leukocyte Adhesion ◽  
Effective Therapy ◽  
Type Ii ◽  
Leukocyte Adhesion Deficiency ◽  
Oral Supplementation ◽  
Selectin Ligands ◽  
Deficiency Type ◽  
Core Fucosylation

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.

scholarly journals Defective intracellular activity of GDP-d-mannose-4,6-dehydratase in leukocyte adhesion deficiency type II syndrome

FEBS Letters ◽  
1998 ◽  
Vol 429 (3) ◽  
pp. 274-278 ◽  
Author(s):  
Laura Sturla ◽  
Amos Etzioni ◽  
Angela Bisso ◽  
Davide Zanardi ◽  
Giovanni De Flora ◽  
...  
Keyword(s):  
Leukocyte Adhesion ◽  
Type Ii ◽  
Leukocyte Adhesion Deficiency ◽  
Intracellular Activity ◽  
Deficiency Type

scholarly journals Exploratory laparoscopy for rotational abnormality of the intestine in a child with leukocyte adhesion deficiency type II

2013 ◽  
Vol 1 (8) ◽  
pp. 244-246
Author(s):  
Thomas Hays ◽  
Elizabeth Feuille ◽  
Edith Schussler ◽  
Charlotte Cunningham-Rundles ◽  
Peter Midulla
Keyword(s):  
Leukocyte Adhesion ◽  
Type Ii ◽  
Leukocyte Adhesion Deficiency ◽  
Deficiency Type ◽  
Exploratory Laparoscopy

scholarly journals Late diagnosis of leukocyte adhesion deficiency type II and Bombay blood type in a child: a rare case report

2019 ◽  
Vol 44 (2) ◽  
pp. 206-209
Author(s):  
Yöntem Yaman ◽  
Sultan Aydin Köker ◽  
Fahri Yüce Ayhan ◽  
Ferah Genel ◽  
Can Acıpayam ◽  
...  
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Leukocyte Adhesion ◽  
Late Diagnosis ◽  
Blood Type ◽  
Type Ii ◽  
Leukocyte Adhesion Deficiency ◽  
Rare Case Report ◽  
Deficiency Type

Secretory Cell Hyperplasia and Defects in Notch Activity in a Mouse Model of Leukocyte Adhesion Deficiency Type II

2010 ◽  
Vol 138 (3) ◽  
pp. 1079-1090.e5 ◽  
Author(s):  
Christopher C.M. Waterhouse ◽  
Steven Johnson ◽  
Mia Phillipson ◽  
Lori Zbytnuik ◽  
Björn Petri ◽  
...  
Keyword(s):  
Mouse Model ◽  
Secretory Cell ◽  
Leukocyte Adhesion ◽  
Type Ii ◽  
Leukocyte Adhesion Deficiency ◽  
Cell Hyperplasia ◽  
Notch Activity ◽  
Deficiency Type
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