Leukocyte adhesion deficiency in a child with severe oral involvement.
Leukocyte adhesion deficiency is a rare inherited defect of phagocytic function resulting from a lack of leukocyte cell surface expression of beta2 integrin molecules (CD11 and CD18) that are essential for leukocyte adhesion to endothelial cells and chemotaxis. A small number of patients with leukocyte adhesion deficiency-1 have a milder defect, with residual expression of CD18. These patients tend to survive beyond infancy; they manifest progressive severe periodontitis, alveolar bone loss, periodontal pocket formation, and partial or total premature loss of the primary and permanent dentitions. We report on a 13-year-old boy with moderate leukocyte adhesion deficiency-1 and severe prepubertal periodontitis. This case illustrates the need for the dentist to work closely with the pediatrician in the prevention of premature tooth loss and control of oral infection in these patients.
Majorana
A
A
Department of Oral Medicine and Pediatric Dentistry, School of Dentistry, University Of Brescia, Italy.
Notarangelo
L D
LD
Savoldi
E
E
Gastaldi
G
G
Lozada-Nur
F
F
D000293:Adolescent; D016301:Alveolar Bone Loss / Q000000981:diagnostic imaging / Q000209:etiology; D019980:Amoxicillin-Potassium Clavulanate Combination / Q000627:therapeutic use; D017742:Dental Care for Chronically Ill; D005885:Gingival Hyperplasia / Q000209:etiology; D006801:Humans; D018370:Leukocyte-Adhesion Deficiency Syndrome / Q000150:complications*; D008297:Male; D019226:Oral Ulcer / Q000188:drug therapy / Q000382:microbiology; D010518:Periodontitis / Q000209:etiology*; D011859:Radiography; D016388:Tooth Loss / Q000209:etiology
D019980:Amoxicillin-Potassium Clavulanate Combination