[Bilateral ureteral obstruction by granuloma formation in chronic granulomatosis].

A 24-year-old patient with chronic granulomatous disease had bilateral hydronephrosis resulting from ureteral granuloma formation. He was treated with high-dose steroids, antibiotics and percutaneous nephrostomy. The etiology, incidence, clinical findings and treatment of chronic granulomatous disease are discussed.

[Long-term treatment of patients with itraconazole for the prevention of Aspergillus infections in patients with chronic granulomatous disease (CGD)].

Chronic granulomatous disease (CGD) represents an innate immunodeficiency: the reduced production of oxygen radicals in phagocytosing cells results in decreased ability to kill pathogenic microorganisms. The patients concerned suffer from severe recurrent infections due to bacteria and fungi. Prophylactic administration of trimethoprim-sulfamethoxazole, as usual in CGD-patients, has markedly reduced the incidence of bacterial infections.

Pyrrolidine dithiocarbamate inhibits NF-kappa B mobilization and TNF production in human monocytes.

The human TNF promoter contains four potential nuclear factor-kappa B (NF-kappa B)-binding sites, with the strongest binding seen for the -605 motif. Nuclear extracts from unstimulated cells of the human monocytic cell line, Mono Mac 6, contain one specific binding protein (complex II), consistent with a constitutive p50 homodimer. Stimulation of Mono Mac 6 cells with LPS will increase complex II and will strongly induce a second specific complex (complex I), which represents the p50/65 heterodimer.

Sulfite stimulates NADPH oxidase of human neutrophils to produce active oxygen radicals via protein kinase C and Ca2+/calmodulin pathways.

The effect of sulfite on the oxidative metabolism of human neutrophils was studied in vitro. Superoxide anion production of PMN was determined using superoxide dismutase-inhibitable lucigenin-dependent CL. The addition of sulfite in concentrations of 0.

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

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).

Is the IFN-gamma-induced enhancement of superoxide production in CGD-phagocytes caused by increased expression of the p47-phox cytosolic protein.

Interferon-gamma (IFN-gamma) had been shown to increase superoxide production of cultured monocytes from patients with chronic granulomatous disease (CGD). To elucidate the mechanism of the IFN-gamma-induced improvement of superoxide production of cultured monocytes from patients with CGD we examined the influence of IFN-gamma on the expression and the activity of the NADPH-oxidase in the monocytic cell-line Mono Mac 6. After cultivation of Mono Mac 6-cells in the presence of 500 U/ml IFN-gamma the superoxide production was found to be increased as well as the expression of the p47-phox cytosolic protein of the phagocytic NADPH-oxidase.

High- vs low-dose immunoglobulin therapy in the long-term treatment of X-linked agammaglobulinemia.

The data of 29 patients with X-linked agammaglobulinemia, who received immunoglobulin replacement therapy between 1965 and 1990, were analyzed for dose-dependent long-term results concerning infectious complications. Patients who received high-dose intravenous immunoglobulin replacement (greater than 400 mg/kg every 3 weeks) showed a significant increase in trough serum IgG levels and a significant decrease in the incidence of pneumonias and the number of days spent in the hospital compared with patients receiving intravenous immunoglobulin low-dose (less than 200 mg/kg every 3 weeks) or intramuscular immunoglobulin (less than 100 mg/kg every 3 weeks) treatment. Improvements in therapeutic outcome were particularly evident when high-dose intravenous immunoglobulin replacement therapy was started before the age of 5 years.