Leukocyte adhesion deficiency in a case presenting as septic arthritis


KILIÇ GÜLTEKİN S. Ş.

International Pediatrics, vol.17, no.2, pp.96-97, 2002 (Refereed Journals of Other Institutions) identifier

  • Publication Type: Article / Article
  • Volume: 17 Issue: 2
  • Publication Date: 2002
  • Title of Journal : International Pediatrics
  • Page Numbers: pp.96-97
  • Keywords: Adhesion molecules, Arthritis, Leukocytes

Abstract

Reported is the first description of septic arthritis in a patient with leukocyte adhesion deficiency (LAD-1). A three-month-old boy was referred due to an antibiotic resistant sepsis and a history of delayed umbilical cord separation. Leukocyte adhesion deficiency (LAD) is an autosomal recessive genetic disease involving deficient expression of three related leukocyte adhesion glycoproteins: LFA1, the C3b receptor (CR3, mac-1) and p150, 95. All three glycoproeins are expressed on monocytes and granulocytes, while LFA-1 is expressed on lymphocytes. These molecules are essential for many leukocyte adhesion related functions, including chemotaxis, aggregation, phagocytosis, and antibody directed cellular cytotoxicity. The diagnosis of LAD-1 relies on both clinical and in-vitro observation.