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Leukocyte-Adhesion Deficiency Syndrome

Rare, autosomal recessive disorder caused by deficiency of the beta 2 integrin receptors (RECEPTORS, LEUKOCYTE-ADHESION) comprising the CD11/CD18 family of glycoproteins. The syndrome is characterized by abnormal adhesion-dependent functions, especially defective tissue emigration of neutrophils, leading to recurrent infection.
Also Known As:
Deficiency Syndrome, Leukocyte-Adhesion; Deficiency Syndrome, Leukocyte Adhesion; Deficiency Syndromes, Leukocyte-Adhesion; Leukocyte Adhesion Deficiency Syndrome; Leukocyte-Adhesion Deficiency Syndromes; Syndrome, Leukocyte-Adhesion Deficiency; Syndromes, Leukocyte-Adhesion Deficiency
Networked: 16 relevant articles (0 outcomes, 4 trials/studies)

Disease Context: Research Results

Related Diseases

1. Alveolar Bone Loss
2. Wiskott-Aldrich Syndrome (Syndrome, Wiskott-Aldrich)
3. Suppuration
4. Mental Retardation (Idiocy)
5. Infection

Experts

1. Badolato, Raffaele: 1 article (06/2013)
2. Xu, Yu-Xin: 1 article (01/2013)
3. Ma, Anna: 1 article (01/2013)
4. Liu, Li: 1 article (01/2013)
5. Sperandio, Markus: 1 article (03/2009)
6. Moser, Markus: 1 article (03/2009)
7. Schmidt, Sarah: 1 article (03/2009)
8. Schmid, Stephan: 1 article (03/2009)
9. Sixt, Michael: 1 article (03/2009)
10. Bauer, Martina: 1 article (03/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Leukocyte-Adhesion Deficiency Syndrome:
1. IntegrinsIBA
2. LigandsIBA
3. 5- acetylneuraminyl- (2- 3)- galactosyl- (1- 4)- (fucopyranosyl- (1- 3))- N- acetylglucosamine (sialyl Lewis x)IBA
4. SelectinsIBA
5. E-SelectinIBA
6. DaptomycinFDA Link
7. tebufenozide (Mimic)IBA
8. NADPH Oxidase (NAD(P)H oxidase)IBA
9. L-SelectinIBA
10. ChemokinesIBA

Therapies and Procedures

1. Transplants (Transplant)
2. Bone Marrow Transplantation (Transplantation, Bone Marrow)
3. Transplantation (Transplant Recipients)
4. Ligation