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Citations to this article

Defective granulocyte chemotaxis in the Chediak-Higashi syndrome
Robert A. Clark, Harry R. Kimball
Robert A. Clark, Harry R. Kimball
Published December 1, 1971
Citation Information: J Clin Invest. 1971;50(12):2645-2652. https://doi.org/10.1172/JCI106765.
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Research Article

Defective granulocyte chemotaxis in the Chediak-Higashi syndrome

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Abstract

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.

Authors

Robert A. Clark, Harry R. Kimball

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RB Zurier
Journal of Investigative Dermatology 1978
Lazy leucocyte syndrome--disorder of the granulocyte membrane?
PH Pinkerton, JB Robinson, JS Senn
Journal of clinical pathology 1978
The effect of acrylamide on human polymorphonuclear neutrophils in vitro
D Mangan, IS Snyder
British journal of industrial medicine 1978
Specific protease deficiency in polymorphonuclear leukocytes of Chédiak-Higashi syndrome and beige mice
JD Vassalli, A Granelli-Piperno, C Griscelli, E Reich
Journal of Experimental Medicine 1978
Defective neutrophil motility and recurrent infection. In vitro and in vivo effects of levamisole
AR Rabson, R Anderson, A Glover
Clinical & Experimental Immunology 1978
The Chédiak-Higashi syndrome. Evidence that defective leukotaxis is primarily due to an impediment by giant granules
CC Clawson, JG White, JE Repine
The American Journal of Pathology 1978
Immunocytochemical Identification of Azurophilic and Specific Granule Markers in the Giant Granules of Chediak-Higashi Neutrophils
PG Rausch, KB Pryzwansky, JK Spitznagel
New England Journal of Medicine 1978
Chronic Granulomatous Disease in Three Siblings
B Elgefors, S Olling, H Peterson
Scandinavian Journal of Infectious Diseases 1978
Microbial—Phagocytic Interactions
PG Quie, EL Mills
Clinics in rheumatic diseases 1978
Mechanism for the inflammatory response in primate lungs. Demonstration and partial characterization of an alveolar macrophage-derived chemotactic factor with preferential activity for polymorphonuclear leukocytes
JA Kazmierowski, JI Gallin, HY Reynolds
Journal of Clinical Investigation 1977
Effects of levamisole on normal and abnormal leukocyte locomotion
DG Wright, CH Kirkpatrick, JI Gallin
Journal of Clinical Investigation 1977
LYSOSOMES AND DERMATOLOGY
RB Zurier
International Journal of Dermatology 1977
Acute inflammation. A review
GB Ryan, G Majno
The American Journal of Pathology 1977
Effects of tobacco smoke on chemotaxis and glucose metabolism of polymorphonuclear leukocytes
RB Bridges, JH Kraal, LJ Huang, BM Chancellor
Infection and immunity 1977
Effects of cigarette smoke components on in vitro chemotaxis of human polymorphonuclear leukocytes
RB Bridges, JH Kraal, LJ Huang, MB Chancellor
Infection and immunity 1977
Defective neutrophil chemotaxis in juvenile periodontitis
RA Clark, RC Page, G Wilde
Infection and immunity 1977
Clinical conditions associated with defective polymorphonuclear leukocyte chemotaxis
PG Quie, KL Cates
The American Journal of Pathology 1977
Delayed hypersensitivity: indicator of acquired failure of host defenses in sepsis and trauma
JL Meakins, JB Pietsch, O Bubenick, R Kelly, H Rode, J Gordon, LD MacLean
Annals of Surgery 1977
Function of Neutrophils in Preleukaemia
P Ruutu, T Ruutu, P Vuopio, TU Kosunen, A la Chapelle
Scandinavian Journal of Haematology 1977
Cutaneous Manifestations of Defective Host Defenses
WL Weston
Pediatric Clinics of North America 1977
The Clinical and Laboratory Diagnosis of Chronic Granulomatous Disease of Childhood
BR Macpherson
Critical Reviews in Clinical Laboratory Sciences 1977
Disorders of Leukocyte Chemotaxis
R Snyderman, MC Pike
Pediatric Clinics of North America 1977
Biological Amplification Systems in Immunology
NK Day, RA Good
1977
Correction of characteristic abnormalities of microtubule function and granule morphology in Chediak-Higashi syndrome with cholinergic agonists
JM Oliver, RB Zurier
Journal of Clinical Investigation 1976
Defective neutrophil chemotaxis in bone marrow transplant patients
RA Clark, FL Johnson, SJ Klebanoff, ED Thomas
Journal of Clinical Investigation 1976
Impaired microtubule function correctable by cyclic GMP and cholinergic agonists in the Chediak-Higashi syndrome
JM Oliver
The American Journal of Pathology 1976
Effect of prophylactic colchicine therapy on leukocyte function in patients with familial mediterranean fever
CA Dinarello, MJ Chusid, AS Fauci, JI Gallin, DC Dale, SM Wolff
Arthritis & Rheumatism 1976
The steroid effect on the in vitro human neutrophil chemotactic response
JA Majeski, JW Alexander
Journal of Surgical Research 1976
A familial defect in cellular chemotaxis associated with redheadedness and recurrent infection
S Witemeyer, DE van Epps
The Journal of Pediatrics 1976
Defective neutrophil motility in children with measles
R Anderson, AR Rabson, R Sher, HJ Koornhof, D Bact
The Journal of Pediatrics 1976
Host Response in Chronic Periodontal Disease: V. The Dental and Periodontal Status of Mink and Mice Affected by Chediak-Higashi Syndrome*
WS Lavine, RC Page, GA Padgett
Journal of Periodontology 1976
Inflammation and Localization of Infection
GB Ryan
Surgical Clinics of North America 1976
Impaired Resistance to Infection
DM Whitten
Clinics in Perinatology 1976
Interaction of leukocyte chemotactic factors with the cell surface. I. Chemotactic factor-induced changes in human granulocyte surface charge
JI Gallin, JR Durocher, AP Kaplan
Journal of Clinical Investigation 1975
Growth characteristics of bone marrow cells from beige mutant, the mouse homologue of the Chediak-Highshi syndrome of man, propagated in semisolid agar cultures
HW Renshaw, WC Davis
In Vitro 1975
ABNORMALITIES OF LEUKOCYTE CHEMOTAXIS IN HUMAN DISEASE
R Snyderman, MC Pike, LC Altman
Annals of the New York Academy of Sciences 1975
Comparison of leukocyte count and function in smoking and nonsmoking young men
RC Noble, BB Penny
Infection and immunity 1975
Effects of serotonin, carbamylcholine, and ascorbic acid on leukocyte cyclic GMP and chemotaxis
JA Sandler, JI Gallin, M Vaughan
The Journal of Cell Biology 1975
Suppression of cellular immune responses following transfer factor: Report of a case
CH Kirkpatrick, JI Gallin
Cellular Immunology 1975
Opportunistic infection in children
RD Feigin, WT Shearer
The Journal of Pediatrics 1975
Evaluation of Infants and Children with Recurrent Infection
AJ Ammann, DW Wara
Current Problems in Pediatrics 1975
Kinetic analysis of chemotactic factor generation in human serum via activation of the classical and alternate complement pathways
JI Gallin, RA Clark, MM Frank
Clinical Immunology and Immunopathology 1975
Modulation of the inflammatory response by products released from human polymorphonuclear leukocytes during phagocytosis: Generation and inactivation of the chemotactic factor C5a
DG Wright, JI Gallin
Inflammation 1975
Leucocyte Chemotaxis: Physiological Considerations and Abnormalities
JI Gallin, SM Wolff
Clinics in Haematology 1975
Granulocytendysfunktion: II. Erworbene Störungen
D Niethammer, A Wildfeuer, E Kleihauer, O Haferkamp
Klinische Wochenschrift 1975
DEFECT IN NEUTROPHIL GRANULOCYTE CHEMOTAXIS IN JOB'S SYNDROME OF RECURRENT "COLD" STAPHYLOCOCCAL ABSCESSES
HR Hill, PG Quie, HF Pabst, HD Ochs, RA Clark, SJ Klebanoff, RJ Wedgwood
The Lancet 1974
RAISED SERUM-IgE LEVELS AND DEFECTIVE NEUTROPHIL CHEMOTAXIS IN THREE CHILDREN WITH ECZEMA AND RECURRENT BACTERIAL INFECTIONS
HR Hill, PG Quie
The Lancet 1974
Interactions of Antibodies, Complement Components and Various Cell Types in Immunity Against Viruses and Pyogenic Bacteria
AC Allison
Immunological Reviews 1974
Leukocyte dysfunction in the bovine homologue of the Chediak-Higashi syndrome of humans
HW Renshaw, WC Davis, HH Fudenberg, GA Padgett
Infection and immunity 1974
Leukotaxis and leukotactic disorders. A review
PA Ward
The American Journal of Pathology 1974
The regulatory role of divalent cations in human granulocyte chemotaxis. Evidence for an association between calcium exchanges and microtubule assembly
JI Gallin, AS Rosenthal
The Journal of Cell Biology 1974
Infection and immunoglobulin concentrations in Chediak-Higashi mice
RJ Elin, JB Edelin, SM Wolff
Infection and immunity 1974
Molecular basis for functional disorders of phagocytes
RL Baehner
The Journal of Pediatrics 1974
Phagocytosis: The Normal Process and Its Clinically Significant Abnormalities
JA Winkelstein, RH Drachman
Pediatric Clinics of North America 1974
Diseases Which Simulate Allergy
RF Beers
Medical Clinics of North America 1974
Human Leucocytes and Infection
JA Raeburn
Scottish Medical Journal 1974
Neutrophil chemotaxis in systemic lupus erythematosus.
Clark RA, Kimball HR, Decker JL
Annals of the rheumatic diseases 1974
Disorders of neutrophil function. Defects in the early stages of the phagocytic process
PJ Edelson, DP Stites, S Gold, HH Fudenberg
Clinical & Experimental Immunology 1973
The Chediak-Higashi syndrome. Fine structure of giant inclusions in freeze-fractured neutrophils
JG White
The American Journal of Pathology 1973
Leukocyte movement—in vitro and in vivo correlates
ME Miller
The Journal of Pediatrics 1973
Granulocytopathies
PG Quie, HR Hill
Disease-a-Month 1973
Generation of chemotactic factors in guinea pig serum via activation of the classical and alternate complement pathways
RA Clark, MM Frank, HR Kimball
Clinical Immunology and Immunopathology 1973
Disorders of Host Defense Against Infection: Pathophysiologic and Diagnostic Considerations
RB Johnston, AR Lawton, MD Cooper
Medical Clinics of North America 1973
Problematik und klinische Bedeutung der Leukocyten-Funktionstestung
PA Miescher
Klinische Wochenschrift 1973
Abnormal Bactericidal, Metabolic, and Lysosomal Functions of Chediak-Higashi Syndrome Leukocytes
RK Root, AS Rosenthal, DJ Balestra
Journal of Clinical Investigation 1972
Disorders of Phagocyte Function
PG Quie
Current Problems in Pediatrics 1972
Protides of the Biological Fluids
PA Small, RH Waldman
Protides of the Biological Fluids 1969
The Inflammatory Process
R Macfarlane
The Inflammatory Process 1965
Hematology
JJ Shea, JJ Shea
The Laryngoscope 1949

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