CD18 deficiency
MIM.600065 21q22.3
ITGB2 deficiency, LFA1 leukocyte adhesion deficiency
Autosomal recessive disorder - Leukocyte adhesion deficiency
Synopsis
recurrent bacterial infections
impaired pus formation
impaired wound healing
abnormal adhesion-dependent functions of granulocytes, monocytes, and lymphocytes
infection of umbilical cord stump
omphalitis
gingivitis (periodontosis)
Associations
systemic juvenile rheumatoid arthritis
Biology
leukocyte cell adhesion molecule defect
reduced neutrophil phagocytic and respiratory burst
response to bacteria and yeast
reduced adherance to various substances
reduced migration into infection sites
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