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Phagocytosis By Human Neutrophils
Published in Hans H. Gadebusch, Phagocytes and Cellular Immunity, 2020
In vitro experiments certainly demonstrate that MP0-H2O2-halide may be bactericidal, but whether this system is, in fact, a major bactericidal mechanism in vivo is not clear. Although most inhibitors of MPO cause inhibition of clearance by intact cells,1*4 it is possible to completely inhibit myeloperoxidase-mediated reactions with ascorbic acid with no detectable effect on the bactericidal activity of the cell.297 This could be the result of conflicting phenomena, however, since the inhibited peroxidase system might be offset by the bactericidal activity of the ascorbate itself (discussed below). Even more significant is the fact that patients with a congenital deficiency of MPO have been reported. Such patients do not suffer from recurrent bacterial infection as do patients with chronic granulomatous disease. Further, only a relatively mild defect in clearance has been detected in the isolated cells of MPO-deficient patients.298*299 Taken together, these two syndromes suggest that, although oxidative mechanisms are crucial to the normal function of the cell, those mechanisms need not be mediated by myeloperoxidase. It is possible that the peroxidase-deficient cells have compensated for the defect by substituting other nonenzymatic bactericidal mechanisms that might normally be of lesser importance. This point has been emphasized by Klebanoff who demonstrated a significantly greater degree of oxidative metabolism in such deficient cells.300,301
Trimethoprim and Trimethoprim–Sulfamethoxazole (Cotrimoxazole)
Published in M. Lindsay Grayson, Sara E. Cosgrove, Suzanne M. Crowe, M. Lindsay Grayson, William Hope, James S. McCarthy, John Mills, Johan W. Mouton, David L. Paterson, Kucers’ The Use of Antibiotics, 2017
Jason A. Trubiano, M. Lindsay Grayson
Chronic granulomatous disease is characterized by recurrent, often life-threatening, bacterial and mycotic infections. It is caused by the inability of the patient’s phagocytes to kill catalase-positive organisms, particularly S. aureus. Long-term prophylaxis with CoT has resulted in a reduction in infectious episodes but had no effect on the occurrence of C. albicans and Aspergillus infections (Weening et al., 1983; Van der Meer and Van den Broek, 1984; Mouy et al., 1989; Margolis et al., 1990). This beneficial effect of CoT appears to be due to the uptake and concentration of its components in granulocytes (Gmünder and Seger, 1981). Margolis et al. (1990) reviewed the National Institutes of Health experience with long-term prophylaxis with CoT in patients with chronic granulomatous disease. Prophylaxis decreased the incidence of nonfungal infections from 7.1–2.4 per 100 patient-months in patients with autosomal chronic granulomatous disease and from 15.8–6.9 infections per 100 patient-months in X-linked chronic granulomatous disease patients (p = 0.06). However, there was no significant change in the incidence of fungal infections in these patients. Sulfonamides alone may also be effective in this disease. Nevertheless, CoT continues to be recommended for prophylaxis in this disease (Seger, 2008).
Gastrointestinal and hepatobiliary
Published in Dave Maudgil, Anthony Watkinson, The Essential Guide to the New FRCR Part 2A and Radiology Boards, 2017
Dave Maudgil, Anthony Watkinson
The differential diagnosis of hepatic calcification includes the following. True or false? Hydatid disease.Sarcoidosis.Portal vein thrombosis.Ovarian metastases.Chronic granulomatous disease of childhood.
Primary Immunodeficiency Diseases Presenting with Chalazia as the First Manifestation
Published in Ocular Immunology and Inflammation, 2021
María Nieves-Moreno, María Granados, Susana Noval
The chronic granulomatous disease is a rare inherited disease in which phagocytic cells are unable to generate the oxygen compounds necessary to kill catalase-positive organisms.1 The affected patients present severe and recurrent bacterial and fungal infections along with inflammatory complications that lead to granulomatous lesions.6,7 Ocular involvement of patients with CGD has been described in the literature; patients usually present chorioretinal lesions associated with atrophic scars. The histopathology of the chorioretinal lesions often shows granulomatous inflammation of the choroid.8 Blepharoconjunctivitis and punctate keratitis have also been widely reported in patients with CGD.1 CGD has also been associated with chronic uveitis,9,10 limbal granulomas, corneal ulcers,11 and retinal masses. To the author’s knowledge, this is the first case in the literature of CGD where a chalazion was the manifestation that led to the diagnosis of the disease. The prompt diagnosis and treatment of this child prevented the development of more severe ocular and systemic complications.
Pigmented paravenous chorioretinal atrophy revealing a chronic granulomatous disease
Published in Ophthalmic Genetics, 2019
Vasily M. Smirnov, Delphine Ley, Brigitte Nelken, Florence Petit, Sabine Defoort-Dhellemmes
At follow-up visits, the child complained of abdominal pain, diarrhea, unexplained fever, and oral ulcers, associated with weight loss and perianal abscess, and fistula. Abdominal ultrasonography and colonoscopy revealed severe pancolitis with mucosal aphthous ulcers. Microscopic analysis of colonic biopsies showed acute colitis with non-caseating granuloma. Crohn’s-like inflammatory bowel disease was suspected and the child was treated with infliximab and azathioprine. However, the intestinal disease was complicated by colonic perforation with parasigmoid abscess formation. MR enterography revealed a diffuse mesenteric infiltration and lymphadenopathy, and this was the reason that the patient was screened for immune dysfunction. Nitro blue tetrazolium test (NBT) result was 0%. Homozygous mutation was found in the NCF1 gene (c.75_76delGT in exon 2), confirming the diagnosis of recessive chronic granulomatous disease (CGD).
Gastrointestinal manifestations of primary immune deficiencies in children
Published in International Reviews of Immunology, 2018
Chronic granulomatous disease (CGD) is a rare inherited disorder of the innate immune system caused by mutations in any of the genes encoding subunits of the superoxide-generating phagocyte NADPH oxidase, which is essential for killing catalase-producing bacteria and fungi by phagocytes19 CGD is X-linked in 60% of cases and is due to a mutation in the CYBB gene responsible for encoding the gp961-phox protein. This protein is an essential subunit forming the NADPH oxidase complex responsible for phagocytosis. In the 30% of cases which are autosomal recessive the mutation is due to a lack of the p47phox protein which is also a subunit of the neutrophil NADPH oxidase.20 CGD also causes an inability in degradation of inflammatory mediators released during an infection, resulting in granuloma formation. This extremely rare condition affects 1 in every 200,000 livebirths.21 The gastrointestinal tract is one of the commonest systems affected in chronic granulomatous disease.