• Definition: A genetic condition characterized by a defect in the leukocytic chemotaxis that results in decreased phagocyte activity
• Etiology
  • Autosomal recessive inheritance
  • Absence of the β2-integrin leukocyte adhesion surface molecule LFA-1 (CD18) prevents leukocytes from migrating to tissues during infection or inflammation.
• Clinical features
  • Recurrent nonsuppurative bacterial infections (e.g., skin and mucosal infections) with minimal inflammation due to dysfunctional neutrophils
  • Impaired wound healing
  • Omphalitis
  • Delayed separation of the umbilical cord (> 30 days postpartum)
    • The normal separation of the umbilical cord is a process that involves inflammation and subsequent healing.
• Diagnosis
  • Leukocytosis; however, neutrophils are absent at the site of infection
  • Flow cytometry: absent CD18, CD11a, CD11b, and CD11c