LEUKOCYTE ADHESION DEFICIENCY TYPE I: CASE REPORT

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Journal of the Korean Academy of Pediatric Dentistry 2009;36(4):586-590.
Published online November 30, 2009.

LEUKOCYTE ADHESION DEFICIENCY TYPE I: CASE REPORT

조선대학교 치과대학 소아치과학교실

Leukocyte adhesion deficiency type I 환자의 치험례

윤은영, 이난영, 이상호

조선대학교 치과대학 소아치과학교실

Correspondence: ,

Received: 4 June 2009 • Accepted: 27 October 2009

Abstract

Leukocyte adhesion deficiency is rare inherited defect on phagocytic function resulting lack of leukocyte cell surface expression of β2 integrin molecule that are essential for leukocyte adhesion to endothelial cells and chemotaxis. Clinical features of patients with leukocyte adhesion deficiency type I include recurrent necrotic infection of the skin mucous membranes, and intestinal tract with septicemia, and omphalitis arising from delayed umbilical cord separation. Oral manifestations are severe progressive periodontitis with alveolar bone loss, periodontal pockets, and partial and total premature loss of the deciduous and permanent dentitions. We report a case of leukocyte adhesion deficiency type I in a 5-year-old child with severe periodontitis. In order to prevent local and systemic infection, we controlled periodontal disease with periodic oral prophylaxis. Oral swabs and blood cultures were perfomed for suspected infection, so that optimal measures were taken through the use of appropriate antibiotics.

Key Words: Leukocyte adhesion deficiency type I, Leukocyte, Periodontitis, Oral hygiene