scholarly journals In vitro molecular reconstitution of the respiratory burst in B lymphoblasts from p47-phox-deficient chronic granulomatous disease.

1993 ◽  
Vol 91 (1) ◽  
pp. 201-207 ◽  
Author(s):  
B D Volpp ◽  
Y Lin
Keyword(s):  
Chronic Granulomatous Disease ◽  
Respiratory Burst ◽  
Granulomatous Disease

scholarly journals Improvement of superoxide production in monocytes from patients with chronic granulomatous disease by recombinant cytokines

Blood ◽  
1993 ◽  
Vol 81 (8) ◽  
pp. 2131-2136
Author(s):  
V Jendrossek ◽  
AM Peters ◽  
S Buth ◽  
J Liese ◽  
U Wintergerst ◽  
...  
Keyword(s):  
Chronic Granulomatous Disease ◽  
Respiratory Burst ◽  
Interleukin 1 ◽  
Superoxide Production ◽  
Granulomatous Disease ◽  
Necrosis Factor Alpha ◽  
Ifn Gamma ◽  
Phorbolmyristate Acetate

Cytokines have been shown to modulate the respiratory burst of polymorphonuclear leukocytes and monocytes from normal controls. We have examined whether monocytes from children with chronic granulomatous disease (CGD) can be primed by cytokines other than interferon-gamma (IFN gamma), which has been demonstrated to improve the production of reactive oxygen species in vivo and in vitro. Monocytes isolated from peripheral blood were cultured without and with IFN gamma (500 U/mL), tumor necrosis factor-alpha (500 U/mL), interleukin-1 beta (IL-1 beta) (100 U/mL), and IL-3 (100 U/mL). After 3 days of culture, the phorbolmyristate acetate (2 ng/mL) and the formyl- methionyl-leucyl-phenylalanine (0.1 mumol/L)-stimulated superoxide- production was determined in a microtiter system. In nearly all of the 14 patients examined (5 autosomal, 5 X-chromosomal, and 4 of unknown inheritance), an improvement of superoxide production could be demonstrated. The most impressive effect with the cytokines newly tested was seen with monocytes from autosomal CGD patients treated with IL-3 and stimulated by phorbolmyristate acetate. In single patients cultivation of monocytes with IL-6 and granulocyte-macrophage colony- stimulating factor resulted in only slight improvement of superoxide production. Our findings indicate that cytokines other than IFN gamma can positively modulate the defective respiratory burst in CGD and that each patient reacts with an individual pattern to different cytokines.

scholarly journals Absence of Respiratory Burst in X-linked Chronic Granulomatous Disease Mice Leads to Abnormalities in Both Host Defense and Inflammatory Response to Aspergillus fumigatus

1997 ◽  
Vol 185 (2) ◽  
pp. 207-218 ◽  
Author(s):  
David E. Morgenstern ◽  
Mary A.C. Gifford ◽  
Ling Lin Li ◽  
Claire M. Doerschuk ◽  
Mary C. Dinauer
Keyword(s):  
Inflammatory Response ◽  
Aspergillus Fumigatus ◽  
Chronic Granulomatous Disease ◽  
Alveolar Macrophages ◽  
Respiratory Burst ◽  
Pulmonary Inflammation ◽  
Clinical Manifestations ◽  
Granulomatous Disease ◽  
Wild Type

Mice with X-linked chronic granulomatous disease (CGD) generated by targeted disruption of the gp91phox subunit of the NADPH–oxidase complex (X-CGD mice) were examined for their response to respiratory challenge with Aspergillus fumigatus. This opportunistic fungal pathogen causes infection in CGD patients due to the deficient generation of neutrophil respiratory burst oxidants important for damaging A. fumigatus hyphae. Alveolar macrophages from X-CGD mice were found to kill A. fumigatus conidia in vitro as effectively as alveolar macrophages from wild-type mice. Pulmonary disease in X-CGD mice was observed after administration of doses ranging from 105 to 48 spores, none of which produced disease in wild-type mice. Higher doses produced a rapidly fatal bronchopneumonia in X-CGD mice, whereas progression of disease was slower at lower doses, with development of chronic inflammatory lesions. Marked differences were also observed in the response of X-CGD mice to the administration of sterilized Aspergillus hyphae into the lung. Within 24 hours of administration, X-CGD mice had significantly higher numbers of alveolar neutrophils and increased expression of the proinflammatory cytokines IL-1β and TNF-α relative to the responses seen in wild-type mice. By one week after administration, pulmonary inflammation was resolving in wild-type mice, whereas X-CGD mice developed chronic granulomatous lesions that persisted for at least six weeks. This is the first experimental evidence that chronic inflammation in CGD does not always result from persistent infection, and suggests that the clinical manifestations of this disorder reflect both impaired microbial killing as well as other abnormalities in the inflammatory response in the absence of a respiratory burst.

scholarly journals Improvement of superoxide production in monocytes from patients with chronic granulomatous disease by recombinant cytokines

Blood ◽  
1993 ◽  
Vol 81 (8) ◽  
pp. 2131-2136 ◽  
Author(s):  
V Jendrossek ◽  
AM Peters ◽  
S Buth ◽  
J Liese ◽  
U Wintergerst ◽  
...  
Keyword(s):  
Chronic Granulomatous Disease ◽  
Respiratory Burst ◽  
Interleukin 1 ◽  
Superoxide Production ◽  
Granulomatous Disease ◽  
Necrosis Factor Alpha ◽  
Ifn Gamma ◽  
Phorbolmyristate Acetate

Abstract Cytokines have been shown to modulate the respiratory burst of polymorphonuclear leukocytes and monocytes from normal controls. We have examined whether monocytes from children with chronic granulomatous disease (CGD) can be primed by cytokines other than interferon-gamma (IFN gamma), which has been demonstrated to improve the production of reactive oxygen species in vivo and in vitro. Monocytes isolated from peripheral blood were cultured without and with IFN gamma (500 U/mL), tumor necrosis factor-alpha (500 U/mL), interleukin-1 beta (IL-1 beta) (100 U/mL), and IL-3 (100 U/mL). After 3 days of culture, the phorbolmyristate acetate (2 ng/mL) and the formyl- methionyl-leucyl-phenylalanine (0.1 mumol/L)-stimulated superoxide- production was determined in a microtiter system. In nearly all of the 14 patients examined (5 autosomal, 5 X-chromosomal, and 4 of unknown inheritance), an improvement of superoxide production could be demonstrated. The most impressive effect with the cytokines newly tested was seen with monocytes from autosomal CGD patients treated with IL-3 and stimulated by phorbolmyristate acetate. In single patients cultivation of monocytes with IL-6 and granulocyte-macrophage colony- stimulating factor resulted in only slight improvement of superoxide production. Our findings indicate that cytokines other than IFN gamma can positively modulate the defective respiratory burst in CGD and that each patient reacts with an individual pattern to different cytokines.

NORMAL GRANULOCYTE INFUSION THERAPY FOR ASPERGILLOSIS IN CHRONIC GRANULOMATOUS DISEASE

PEDIATRICS ◽  
1973 ◽  
Vol 51 (2) ◽  
pp. 230-233
Author(s):  
Andrew A. Raubitschek ◽  
Alan S. Levin ◽  
Daniel P. Stites ◽  
Edward B. Shaw ◽  
H. Hugh Fudenberg
Keyword(s):  
Adverse Effects ◽  
Chronic Granulomatous Disease ◽  
Blood Cells ◽  
White Blood Cells ◽  
Infusion Therapy ◽  
Granulomatous Disease ◽  
Transient Improvement ◽  
Life Threatening ◽  
Granulocyte Function

An 8-year-old boy with chronic granulomatous disease (CGD) was admitted in moribund condition with aspergillus pneumonia. Because of the gravity of the situation, normal granulocyte infusions were used as adjuncts to the more conventional antimicrobial therapy. White blood cells, derived from a total of 58 units of whole blood obtained by leukophoresis of the father, were given in two separate doses. The first dose, totaling 2.8 x 1010 granulocytes, was coincident with significant improvement, and the second, totaling 3.0 x 1010 granulocytes, was coincident with the onset of clinical improvement and interim recovery. Transient improvement in in vitro granulocyte function was noted in cells taken from the patient's blood immediately after infusion. No adverse effects of the infusions were noted in either the patient or the donor. Although it is impossible to divorce the therapeutic effect of the granulocyte infusions from the more conventional therapy, we conclude that normal granulocyte infusions can be considered a valid adjunct in children with CGD who are suffering from a life-threatening infection.

Decreased Nitroblue Tetrazolium Dye Reduction in the Phagocytes of Patients Receiving Prednisone

PEDIATRICS ◽  
1970 ◽  
Vol 45 (5) ◽  
pp. 861-865
Author(s):  
Denis R. Miller ◽  
Henry G. Kaplan
Keyword(s):  
Chronic Granulomatous Disease ◽  
Nitroblue Tetrazolium ◽  
Quantitative Assay ◽  
Granulomatous Disease ◽  
Host Defenses ◽  
Dye Reduction ◽  
Slide Test ◽  
Intracellular Metabolism

Nitroblue tetrazolium (NBT) dye reduction by leukocytes of 21 patients receiving prednisone was significantly decreased. Nineteen percent of the patients had values similar to those found in children with chronic granulomatous disease, and 57% had heterozygous-range NBT dye reduction. A qualitative NBT dye reduction "slide test" correlated well with the quantitative assay. The uptake of particles by the phagocytes of steroid-treated patients appeared normal. The exact mechanism of corticosteroid action remains unknown. The decreased dye reduction observed in vitro suggests an induced defect of intracellular metabolism which may be related to known alterations of host defenses which occur in patients receiving these hormones.

scholarly journals Chronic granulomatous disease. Expression of the metabolic defect by in vitro culture of bone marrow progenitors.

1980 ◽  
Vol 66 (3) ◽  
pp. 599-602 ◽  
Author(s):  
P E Newburger ◽  
M S Kruskall ◽  
J M Rappeport ◽  
S H Robinson ◽  
M E Chovaniec ◽  
...  
Keyword(s):  
Bone Marrow ◽  
In Vitro Culture ◽  
Chronic Granulomatous Disease ◽  
Granulomatous Disease ◽  
Disease Expression ◽  
Metabolic Defect

scholarly journals Detection of carriers of the autosomal form of chronic granulomatous disease

Blood ◽  
1988 ◽  
Vol 71 (2) ◽  
pp. 505-507 ◽  
Author(s):  
AJ Verhoeven ◽  
ML van Schaik ◽  
D Roos ◽  
RS Weening
Keyword(s):  
Chronic Granulomatous Disease ◽  
Respiratory Burst ◽  
Granulomatous Disease ◽  
Mosaic Pattern ◽  
Simple Method ◽  
Nbt Test ◽  
Activated Neutrophils ◽  
Slide Test ◽  
Microscopic Slide ◽  
Stimulation Of

The NADPH:O2 oxidoreductase catalyzing the respiratory burst in activated phagocytes from healthy individuals is not operative in phagocytes from patients with chronic granulomatous disease (CGD). In a microscopic slide test using the dye nitroblue tetrazolium (NBT), carriers of X-linked CGD can be recognized by a mosaic pattern of NBT- positive and NBT-negative cells, governed by the expression of an unaffected or an affected X chromosome, respectively. Until now, it has not been possible to detect carriers of the autosomal form of CGD (other than by family studies) because all cells of these carriers stain positive in the NBT test. We have investigated whether neutrophils from carriers of autosomal CGD can be recognized by measurement of the rate of oxygen uptake upon stimulation of the cells. It was found that with the phorbol ester PMA as a stimulus, the respiratory burst is significantly lower in autosomal CGD carriers. With serum-treated zymosan as a stimulus, no difference between controls and carriers was observed. The addition of f-Met-Leu-Phe (1 microM) to PMA-activated neutrophils of control donors caused a transient increase in oxygen consumption of about 40%. Under these conditions, an increase of more than 100% was observed in neutrophils from carriers of autosomal CGD. These findings provide a simple method for the detection of carriers of the autosomal form of CGD.

scholarly journals X-Linked Chronic Granulomatous Disease: Mutations in the CYBB Gene Encoding the gp91-phox Component of Respiratory-Burst Oxidase

1998 ◽  
Vol 62 (6) ◽  
pp. 1320-1331 ◽  
Author(s):  
Julie Rae ◽  
Peter E. Newburger ◽  
Mary C. Dinauer ◽  
Deborah Noack ◽  
Penelope J. Hopkins ◽  
...  
Keyword(s):  
Chronic Granulomatous Disease ◽  
Respiratory Burst ◽  
Granulomatous Disease ◽  
Gene Encoding ◽  
Disease Mutations ◽  
Cybb Gene ◽  
Respiratory Burst Oxidase

STUDIES OF POLYMORPHONUCLEAR LEUKOCYTES FROM PATIENTS WITH CHRONIC GRANULOMATOUS DISEASE OF CHILDHOOD: BACTERICIDAL CAPACITY FOR STREPTOCOCCI

PEDIATRICS ◽  
1968 ◽  
Vol 41 (3) ◽  
pp. 591-599
Author(s):  
Edward L. Kaplan ◽  
Throstur Laxdal ◽  
Paul G. Quie
Keyword(s):  
Chronic Granulomatous Disease ◽  
Polymorphonuclear Leukocytes ◽  
Gram Negative Bacteria ◽  
Granulomatous Disease ◽  
Gram Negative ◽  
Modified Method ◽  
Bactericidal Capacity ◽  
Normal Manner ◽  
Streptococcal Species

Polymorphonuclear leukocytes (PMNs) from children with chronic granulomatous disease of childhood have been shown to readily phagocytize staphylococci and certain gram-negative bacteria, but they demonstrate an impaired intracellular bactericidal capacity for these organisms. The in vitro phagocytic and bactericidal capacities of PMNs from three patients with this disease for four species of streptococci (Streptococcus faecalis, Streptococcus viridans, microaerophlic streptococci, and Streptococcus pyogenes) were tested by the modified method of Maaloe. The PMNs obtained from the patients phagocytized and killed the four species of streptococci in a normal manner while still showing the defect for Staph. aureus and S. marcescens. Morphologic examination of coverslip preparations of PMNs revealed minimal post-phagocytic degranulation and vacuole formation when either staphylococci and serratia or the streptococcal species were tested. This suggests that different intracellular mechanisms may be responsible for the streptococcal killing. These observations are in accord with the clinical courses of these patients, who rarely have serious streptococcal infections in contrast to the frequent and life-threatening infections caused by staphylococci and some gram-negative bacteria.

Leprosy (Hansen’s disease)

2010 ◽  
pp. 836-848
Author(s):  
Diana N.J. Lockwood
Keyword(s):  
Public Health ◽  
Chronic Granulomatous Disease ◽  
Health Problem ◽  
Disease Transmission ◽  
Public Health Problem ◽  
Mycobacterium Leprae ◽  
Granulomatous Disease ◽  
Important Public Health Problem ◽  
Important Public Health

Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae, an acid-fast intracellular organism not yet cultivated in vitro. It is an important public health problem worldwide, with an estimated 4 million people disabled by the disease. Transmission of M. leprae is only partially understood, but untreated lepromatous patients discharge abundant organisms from their nasal mucosa into the environment....

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