Impaired Leucotactic Responsiveness in a Child With Recurrent Infections

Ward, P.A.; Schlegel, RobertJ.

doi:10.1016/s0140-6736(69)92699-3

Cited by 96 publications

(20 citation statements)

References 12 publications

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“…A few cases have been reported in which there were serum defects in generation of chemotactic factors (25,26) and, in a single case, a serum inhibitor of chemotaxis (27). In the present report, a defect in in vitro granulocyte chemotaxis associated with abnor-'Blume, R. S., and S. M. Wolff.…”

Section: Introductionsupporting

confidence: 48%

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Defective granulocyte chemotaxis in the Chediak-Higashi syndrome

Clark¹,

Kimball²

1971

J. Clin. Invest.

281

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A B S T R A C T In vivo and in vitro studies of granulocyte chemotaxis were performed in three patients with the Chediak-Higashi syndrome. Rebuck skin windows showed a decreased accumulation of leukocytes at an inflammatory site. Studies in Boyden chambers documented a cellular defect in granulocyte chemotaxis. The chemotactic response of Chediak-Higashi cells by this technique averaged approximately 40% of normal and was consistently reduced using several different chemotactic stimuli. This deficit was magnified by shortening the chamber incubation time or by decreasing the pore size of the micropore filter and was independent of granulocytopenia. No abnormalities of passive motility, adhesiveness, viability, or pH optimum for migration were found in these cells. Chediak-Higashi serum contained no inhibitors of chemotaxis and was capable of generating normal amounts of chemotactic factors with the exception of one patient with the accelerated phase of the disease. Heterozygotes for the Chediak-Higashi trait had normal chemotactic function. This cellular defect in chemotaxis may contribute to the marked susceptibility to pyogenic infections which is so characteristic of patients with the Chediak-Higashi syndrome.

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Section: Introductionsupporting

confidence: 48%

“…At time periods from 1 to 4 hr, the chemotactic responses of cells from heterozygotes were normal. (27). Apparent inhibition (approximately 80%) was seen with all sera so tested.…”

Section: Studies Of Chs Cell Chemotaxismentioning

confidence: 88%

Defective granulocyte chemotaxis in the Chediak-Higashi syndrome

Clark¹,

Kimball²

1971

J. Clin. Invest.

281

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“…Two males (S. E., aged 9, and B. P., 5), with typical sex-linked CGD, including histories of recurrent septic and granulomatous pulmonary lymph node and hepatic infections, and defective leukocyte bactericidal and respiratory capacities, were used for comparison. Some features of these patients have been reported (14,15).' Studies were performed on these patients both when they were infected and clinically well.…”

Section: Introductionmentioning

confidence: 99%

Abnormal Bactericidal, Metabolic, and Lysosomal Functions of Chediak-Higashi Syndrome Leukocytes

Root¹,

Rosenthal²,

Balestra³

1972

J. Clin. Invest.

334

109

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“…Lepow also collaborated with me as we described neutrophil chemotactic factors generated by bacteria during their growth phase (14). Along with Robert Schlegal of the Department of Pediatrics at Walter Reed Army Medical Center, we described the first human case of a chemotactic defect in neutrophils in a child with recurrent bacterial infection (15); the case probably represented what would later be described by others as a defect in CD18, which results in defective adhesion of neutrophils to the vascular endothelium.…”

Section: Wwwannualreviewsorg • the First Fifty Years In Researchmentioning

confidence: 78%