Defective Granulocyte Regulation in the Chediak–Higashi Syndrome

Blume, R; Bennett, John M.; Yankee, Ronald A.; Wolff, Sheldon M.

doi:10.1056/nejm196811072791901

Cited by 76 publications

(26 citation statements)

References 22 publications

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“…Considering the current results in relation to previous studies of CHS, it seems likely that multiple factors including granulocytopenia (8), poor bone marrow granulocyte mobilization (8), decreased leukocyte bactericidal capacity (13,15,16), and defective granulocyte chemotaxis are involved in the increased susceptibility to infections characteristic of the Chediak-Higashi syndrome.…”

supporting

confidence: 63%

“…Granulocytopenia is a consistent finding in humans with CHS, and studies of granulocyte mobilization have documented decreased marrow granulocyte reserves in spite of adequate numbers of precursors in the marrow (8). Granulocyte responses to infection are likewise diminished.2 Leukocyte ingestion of bacteria and inert particles is normal in humans (9-13) as well as mink and cattle with CHS (14).…”

Section: Introductionmentioning

confidence: 99%

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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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supporting

confidence: 63%

Section: Introductionmentioning

confidence: 99%

Defective granulocyte chemotaxis in the Chediak-Higashi syndrome

Clark¹,

Kimball²

1971

J. Clin. Invest.

281

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“…Previous investigations (9) have confirmed normal immunoglobulin levels (save for patients in the advanced stage of illness), normal antibody formation, delayed hypersensitivity, and reticuloendothelial clearance functions previously reported for other similarly affected patients (6)(7)(8). Studies of leukocyte functions have documented the occurrence of neutropenia and decreased marrow granulocyte responses (10), diminished granulocyte responses to infection (9), impaired skin window migration and chemotactic responsiveness of their granulocytes (11), all of which might be incriminated in the pathogenesis of decreased host resistance. The present report deals with the phagocytic, bactericidal, and fungicidal properties of leukocytes obtained from three of these patients.…”

Section: Introductionsupporting

confidence: 54%

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

Root¹,

Rosenthal²,

Balestra³

1972

J. Clin. Invest.

336

109

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“…Abnormalities include poor mobilization of neutrophils from the marrow [31], defective chemotactic response of neutrophils and monocytes [32,33] and decreased bactericidal activity [34]. In vivo skin window studies also demonstrated defective neutrophil migration [32].…”

Section: Discussionmentioning

confidence: 99%