Leukocyte adhesion deficiency disorder: report of one case.
In this paper, a case of leukocyte adhesion deficiency disorder confirmed by flow cytometry and leukocyte chemotaxis assays is reported. The 32-day-old female infant was admitted to our ward on account of delayed umbilical cord detachment and omphalitis. After admission, hemogram revealed severe leukocytosis (white blood cell (WBC) counts: 86,800/cumm), and there was poor clinical response to several kinds of antibiotics. The above history indicated that the patient might be a case of leukocyte adhesion deficiency disorder. A definite absence of CD11b/CD18 adhesion molecule on the patient's granulocytes and severe defects of leukocyte function demonstrated by chemotaxis, chemokinesis and zymosan-induced respiratory burst assays confirmed this diagnosis.
Department of Pediatrics, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.
C H
CH
Hung
S N
SN
Cheng
Y M
YM
Hua
C L
CL
Wang
Y H
YH
Chen
K D
KD
Yang
D016026:Bone Marrow Transplantation; D002634:Chemotaxis, Leukocyte; D017809:Fatal Outcome; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007231:Infant, Newborn; D018370:Leukocyte-Adhesion Deficiency Syndrome / Q000175:diagnosis* / Q000628:therapy; D009504:Neutrophils