Saethre-Chotzen syndrome associated with defective neutrophil chemotaxis.
An 11-month-old male infant with Saethre-Chotzen syndrome and recurrent respiratory infections is described. Persistent extremely high leukocytosis warranted evaluation of neutrophil functions. It was found that the opsonophagocytic activity was normal, but neutrophil chemotaxis was markedly decreased. Further studies pointed to an intracellular neutrophil defect causing this motility dysfunction.
Etzioni
A
A
Department of Pediatrics A, Rambam Medical Center, Haifa, Israel.
Obedeanu
N
N
Benderly
A
A
Gershoni-Baruch
R
R
D000168:Acrocephalosyndactylia / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D001848:Bone Diseases, Developmental / Q000503:physiopathology*; D002634:Chemotaxis, Leukocyte; D006801:Humans; D006972:Hypertelorism / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D007223:Infant; D008297:Male; D009504:Neutrophils; D012008:Recurrence; D012141:Respiratory Tract Infections / Q000209:etiology