Further evaluation of luminol-enhanced luminescence in the diagnosis of disorders of leukocyte oxidative metabolism: role of myeloperoxidase.

1983 ◽  
Vol 29 (3) ◽  
pp. 513-515 ◽  
Author(s):  
M S Cohen ◽  
P S Shirley ◽  
L R DeChatelet
Keyword(s):  
Chronic Granulomatous Disease ◽  
Oxidative Metabolism ◽  
Clinical Consequence ◽  
Granulomatous Disease ◽  
Myeloperoxidase Deficiency ◽  
Luminescence Assay ◽  
Diagnostic Confusion ◽  
Enhanced Luminescence ◽  
Hereditary Nature

Abstract Chemiluminescence can be used to identify defects in the oxidative metabolism of granulocytes. This procedure has recently been adopted for use with microliter quantities of whole blood, appropriate for prenatal or neonatal study. Although the contribution of myeloperoxidase to the chemiluminescence assay has been noted, the possible diagnostic confusion between chronic granulomatous disease of childhood (which is rare and severe) and myeloperoxidase deficiency (which is common and of little clinical consequence) has not been stressed. We report a father and his infant daughter whose cells emitted no light in the luminol-enhanced luminescence assay; both patients are totally peroxidase deficient. These results emphasize the hereditary nature of myeloperoxidase deficiency, and the possibility for erroneous diagnosis of chronic granulomatous disease of childhood based on the luminol-enhanced luminescence test.

scholarly journals Estrogen binding by leukocytes during phagocytosis,.

1977 ◽  
Vol 145 (4) ◽  
pp. 983-998 ◽  
Author(s):  
S J Klebanoff
Keyword(s):  
Chronic Granulomatous Disease ◽  
Reaction Mechanisms ◽  
Dehydrogenase Deficiency ◽  
Cell Types ◽  
Neutrophil Function ◽  
Granulomatous Disease ◽  
Glucose 6 Phosphate Dehydrogenase ◽  
Estrogen Binding ◽  
Two Populations

Estradiol binds covalently to normal leukocytes during phagocytosis. The binding involves three cell types, neutrophils, eosinophils, and monocytes and at least two reaction mechanisms, one involving the peroxidase of neutrophils and monocytes (myeloperoxidase [MPO]) and possibly the eosinophil peroxidase, and the second involving catalase. Binding is markedly reduced when leukocytes from patients with chronic granulomatous disease (CGD), severe leukocytic glucose 6-phosphate dehydrogenase deficiency, and familial lipochrome histiocytosis are employed and two populations of neutrophils, one which binds estradiol and one which does not, can be demonstrated in the blood of a CGD carrier. Leukocytes from patients with hereditary MPO deficiency also bind estradiol poorly although the defect is not as severe as in CGD. These findings are discussed in relation to the inactivation of estrogens during infection and the possible role of estrogens in neutrophil function.

Corticosteroids in the Management of Cystitis Secondary to Chronic Granulomatous Disease

PEDIATRICS ◽  
1990 ◽  
Vol 85 (2) ◽  
pp. 219-221
Author(s):  
REBECCA J. COLLMAN ◽  
JOSEPH D. DICKERMAN
Keyword(s):  
Chronic Granulomatous Disease ◽  
Oxidative Metabolism ◽  
Phagocytic Cell ◽  
Granulomatous Disease ◽  
Intracellular Killing ◽  
Inherited Disorders ◽  
Recurrent Pyogenic Infections

Chronic granulomatous disease refers to a group of inherited disorders characterized by a deficit in phagocytic-cell oxidative metabolism, resulting in recurrent pyogenic infections due to defective intracellular killing of microorganisms.1 Granulomas form in various organs or tissues, presumably in an attempt to "wall off" the infection, and these can be a major cause of morbidity when vital structures are obstructed or compromised. Cultures of the granulomas are usually negative, although antibiotics have been advocated in the treatment of this complication. Recently, three patients with chronic granulomatous disease-two with gastrointestinal and genitourinary involvement2 and one with pulmonary involvement3—were treated with corticosteroids, which resulted in an amelioration of symptoms.

Role of oxygen intermediates in cytotoxicity: Studies in chronic granulomatous disease

Inflammation ◽  
1993 ◽  
Vol 17 (1) ◽  
pp. 77-92 ◽  
Author(s):  
Robert L. Roberts ◽  
Bonnie J. Ank ◽  
Michael W. Fanger ◽  
Li Shen ◽  
E. Richard Stiehm
Keyword(s):  
Chronic Granulomatous Disease ◽  
Granulomatous Disease ◽  
Oxygen Intermediates ◽  
Cytotoxicity Studies

A Variant of Chronic Granulomatous Disease: Deficient Oxidative Metabolism Due to a Low-Affinity NADPH Oxidase

1981 ◽  
Vol 305 (22) ◽  
pp. 1329-1333 ◽  
Author(s):  
P. Daniel Lew ◽  
Frederick S. Southwick ◽  
Thomas P. Stossel ◽  
John C. Whitin ◽  
Elizabeth Simons ◽  
...  
Keyword(s):  
Nadph Oxidase ◽  
Chronic Granulomatous Disease ◽  
Oxidative Metabolism ◽  
Granulomatous Disease

Liver involvement in chronic granulomatous disease: the role of ultrasound in diagnosis and treatment.

Radiology ◽  
1984 ◽  
Vol 153 (1) ◽  
pp. 117-121 ◽  
Author(s):  
L A Garel ◽  
D M Pariente ◽  
C Nezelof ◽  
V J Barral ◽  
C Aboulker ◽  
...  
Keyword(s):  
Chronic Granulomatous Disease ◽  
Diagnosis And Treatment ◽  
Liver Involvement ◽  
Granulomatous Disease

scholarly journals Monocyte function in patients with chronic granulomatous disease of childhood

Blood ◽  
1982 ◽  
Vol 60 (5) ◽  
pp. 1151-1158 ◽  
Author(s):  
GR Donowitz ◽  
GL Mandell
Keyword(s):  
Chronic Granulomatous Disease ◽  
Bactericidal Activity ◽  
Oxidative Metabolism ◽  
Neutrophil Function ◽  
Granulomatous Disease ◽  
Metabolic Defects ◽  
Monocyte Function ◽  
The Difference ◽  
Normal Controls

Abstract Adults with chronic granulomatous disease of childhood (CGD) have been described who remain relatively free of infection despite markedly abnormal neutrophil function. Monocyte function in four adults with this mild or atypical CGD syndrome was examined and compared to that of normal controls and to that of two patients with the more severe or classic CGD syndrome. Monocytes from patients with atypical CGD killed 75.7% +/- 2.6% (mean +/- SEM) of ingested organisms at 30 min, while monocytes from the patients with classic CGD killed only 50.3% +/- 4.2% of bacteria (p less than 0.001). The difference in bactericidal activity between atypical CGD monocytes and normal monocytes was relatively small (75.7% +/- 2.6% versus 88.1% +/- 3.7%, respectively) but was statistically significant (p = 0.007). Monocytes from both atypical and classic CGD patients showed markedly impaired oxidative metabolism. Differences in monocyte bactericidal activity may explain why atypical CGD patients have fewer infections than classic CGD patients. The presence of nonoxidative bactericidal mechanisms in atypical CGD monocytes is suggested by the demonstration of bactericidal activity despite severe oxidative metabolic defects.

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