Leukocyte adhesion deficiency.
Leukocyte adhesion deficiency is a rare syndrome with autosomal recessive pattern of inheritance. An eleven-month-old boy, whose parents were first degree relatives, was referred to clinic with recurrent episodes of pneumonia, otitis and extensive necrotic wounds of perianal area since neonatal period. His umbilical cord had separated 30 days after birth. Laboratory findings included marked leukocytosis, chemotaxis abnormality, and very low levels of CD 11 (0.5%) and CD 18 (2%). Leukocyte Adhesion Defect (LAD) is rare genetic defect of a group of leukocyte membrane glycoproteins. LAD affects nearly one out of every million individuals and is characterized by recurrent bacterial and fungal infections of skin and mucous membranes, diminished pus formation, delayed umbilical cord separation, granulocytosis, poor wound healing and progressive periodontitis. This is the first report of a case of LAD in Isfahan of Iran.
Akbari
H
H
Division of Pediatric Allergy and Immunology, Faculty of Medicine, Isfahan University of Medical Sciences, Iran.
Zadeh
M M
MM
D001004:Anus Diseases / Q000209:etiology / Q000473:pathology; D006801:Humans; D007223:Infant; D007492:Iran; D018370:Leukocyte-Adhesion Deficiency Syndrome / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D008297:Male; D009336:Necrosis; D014472:Umbilicus; D014946:Wound Infection / Q000209:etiology*