TY - JOUR
T1 - Defective neutrophil chemotaxis with variant ichthyosis, hyperimmunoglobulinemia E, and recurrent infections
AU - Pincus, Stephanie H.
AU - Thomas, I. Talfryn
AU - Clark, Robert A.
AU - Ochs, Hans D.
N1 - Funding Information:
From the Departments of Medicine and Pediatrics, University of Washingwn. Supported in part by Grants AM-05250, AI-07763, AI-07073, HD-02266, and RR-37 from the United States Public Health Service, and Grant 1-384 from the National Foundation-March of Dimes. *Reprint address: Department of Medicine (Dermatology), University of Washington, Seattle, Wash. 98195.
PY - 1975/12
Y1 - 1975/12
N2 - Host defense mechanisms were evaluated in a 41/2-year-old boy with recurrent pyogenic infections and a unique hyperkeratotic skin disorder. The patient's neutrophils were consistently defective in chemotactic responsiveness but had normal NBT reduction, glucose oxidation, and iodination. Serum concentrations of IgE were markedly elevated and the secondary antibody response was abnormal. No T-cell dysfunction was detected. These findings suggest a relationship between this patient, and patients with other syndromes associated with recurrent infections, cutaneous disease, defective chemotaxis, immunodeficiency, and hyperimmunoglobulinemia E.
AB - Host defense mechanisms were evaluated in a 41/2-year-old boy with recurrent pyogenic infections and a unique hyperkeratotic skin disorder. The patient's neutrophils were consistently defective in chemotactic responsiveness but had normal NBT reduction, glucose oxidation, and iodination. Serum concentrations of IgE were markedly elevated and the secondary antibody response was abnormal. No T-cell dysfunction was detected. These findings suggest a relationship between this patient, and patients with other syndromes associated with recurrent infections, cutaneous disease, defective chemotaxis, immunodeficiency, and hyperimmunoglobulinemia E.
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U2 - 10.1016/S0022-3476(75)80903-6
DO - 10.1016/S0022-3476(75)80903-6
M3 - Article
C2 - 1185392
AN - SCOPUS:0016703481
SN - 0022-3476
VL - 87
SP - 908
EP - 911
JO - The Journal of pediatrics
JF - The Journal of pediatrics
IS - 6 PART 1
ER -