Abstract
Patients with severe Leukocyte adhesion deficiency type 1 (LAD-1) rarely live longer than two years of age without hematopoietic stem cell transplant (HSCT). Those who undergo a HSCT transplant have a 75% of survival. LAD-1 is a disease caused by a deficiency of expression, or function of CD18. CD18 is a common subunit of the beta 2-integrin coded by ITGF2 gene. Integrins mediate leukocyte adhesion, extravasation, and migration into tissues thus making the patients prone to severe cutaneous and mucosal infections.
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