Neutrophil dysfunction

Clardy CW, Edwards KM, Gay JC. Increased susceptibility to infection in h3fpothermic children possible role of acquired neutrophils dysfunction. Pediatr Infect Dis 1985 4(4) 379-382. 

Thus, the role of cytokines in inflammation, immune-cell function and tissue repair is varied and complex. Later sections of this book explore the cytokines generated by neutrophils during inflammatory challenge ( 7.3.4), the regulation of neutrophil function by cytokines ( 7.2.1) and human diseases associated with neutrophil dysfunction in which cytokines may play important roles ( 8.2.5, 8.8). 

Disorders similar to CHS in humans have been reported in beige mice, Aleutian mink, Hereford cattle and killer whales, and neutrophil dysfunctions similar to those reported in humans are apparent in these animals. Therapy of CHS in humans usually consists of careful management of infections with appropriate antibiotics, although improvements have been reported following administration of high doses of ascorbic acid. Bone-marrow transplantation offers some hope to these patients, especially those entering the accelerated phase of the disease. 

Type Ib equivalent to 10% of total type I similar to type la, with additional findings of neutropenia and neutrophil dysfunction... 

In patients who are severely G6PD deficient, on rare occasions an associated abnormality occurs in which there is neutrophil dysfunction secondary to G6PD deficiency This defect leads to impaired neutrophil bactericidal activity, resulting in recurrent infections with catalasepositive organisms. 

The first quantitative NBT test was developed by Baehner and Natham in 1968 to detect neutrophil dysfunction in patients with chronic granulomatous disease (CGD). The neutrophil membranes of CGD patients lack the specific cytochrome >245 which is a key component in generating superoxide by the cell, following bacterial stimulation of the cell. As a result the patients neutrophils are incapable of responding with a respiratory burst, failing to generate the free radicals necessary for the antimicrobial function of this cell. 

Leuzzi R, Banhegyi G, Kardon T, Marcolongo P, Capecchi PL, 42. Burger, HJ, Benedetti A, Fulceri R. Inhibition of microsomal glucose-6-phosphate transport in human neutrophils results in apoptosis a potential explanation for neutrophil dysfunction in 43. glycogen storage disease type lb. Blood 2003 101 2381-2387. 

Of 13 patients with glycogen storage disease type lb and neutropenia or neutrophil dysfunction treated with G-CSF, all developed splenomegaly, usually after 3 months of treatment (49). Hypersplenism, as defined by moderate thrombocytopenia on at least two consecutive blood counts, was found in five patients, but none required specific interventions. In one carefully... 

Calderwood S, Kilpatrick L, Douglas SD, Freedman M, Smith-Whitley K, Rolland M, Kurtzberg J. Recombinant human granulocyte colony-stimulating factor therapy for patients with neutropenia and/or neutrophil dysfunction secondary to glycogen storage disease type lb. Blood 2001 97(2) 376-82. 

Septic complications associated with total parental nutrition may be partly due to neutrophil dysfunction, though this has not been demonstrated (79). 

Neutrophil dysfunction in a case of glucose-6-phosphate dehydrogenase deficiency. 

Jun HS, et al. Molecular mechanisms of neutrophil dysfunction in glycogen storage disease type Ib. Blood. 2014 123(18) 2843-53. 

Olsen, G.E., and Polk, H.C. (1S77). In vitro effect of ascorbic acid on corticosteroid-caused neutrophil dysfunction. J. Surgical Res. 22 109-112. 

Rebora, A., Dallegri, F., and Patrone, F., 1980, Neutrophil dysfunction and repeated infections Influence of levamisole and ascorbic acid, Br. J. Dermatol. 102 49-56. 

Baehner, R. L., 1980, Neutrophil dysfunction associated with states of chronic and recurrent infections, Pediatr. Clin. North Am. 27 377. 

Maderazo, E. C., Woronick, C. L., and Albano, S. D., 1986, Neutrophil dysfunction in trauma Inappropriate activation, deactivation, and probable autooxidative damage as a mechanism of neutrophil locomotory defect, J. Infect. Dis. 154 471-477. 

Visser G, Rake JP, Fernandes J, Labrune P, Leonard JV, Moses S, Ullrich K, Smit GPA (2000) Neutropenia, neutrophil dysfunction, and inflammatory bowel disease in glycogen storage disease type Ib results of the European study on Glycogen Storage Disease Type I. J Ped 137 187-191. 

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