FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2079334811> ?p ?o ?g. }

• W2079334811 endingPage "1413" @default.
• W2079334811 startingPage "1411" @default.
• W2079334811 abstract "To the Editor: Chronic granulomatous disease (CGD) results from mutations in either X-linked (gp91phox) or autosomal (p47phox, p67phox, and p22phox) genes encoding the phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase. Impaired generation of reactive oxygen species predisposes to recurrent life-threatening bacterial and fungal infections. Septated hyaline molds (particularly Aspergillus species) are the primary fungal pathogens in CGD. Fungi of the order Mucorales (pauciseptated molds) are environmentally ubiquitous and cause mucormycosis in select immunocompromised patient populations, such as those with diabetic ketoacidosis and hematologic malignancy and recipients of transplants or deferoxamine. We investigated the prevalence of mucormycosis in patients with CGD. Medical and microbiology records of all patients with CGD evaluated at the National Institutes of Health were reviewed (n = 278). Of these, 12 patients had specimens with pathologic/microbiological evidence of Mucorales. By using international consensus criteria,1De Pauw B. Walsh T.J. Donnelly J.P. Stevens D.A. Edwards J.E. Calandra T. et al.Revised Definitions of Invasive Fungal Disease from the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group (EORTC/MSG) Consensus Group.Clin Infect Dis. 2008; 46: 1813-1821Crossref PubMed Scopus (3950) Google Scholar only 4 had proved invasive disease, 4 had colonization, and 4 had contaminants. A fifth invasive case was referred (Table I).2Siddiqui S. Anderson V.L. Hilligoss D.M. Abinun M. Kuijpers T.W. Masur H. et al.Fulminant mulch pneumonitis: an emergency presentation of chronic granulomatous disease.Clin Infect Dis. 2007; 45: 673-681Crossref PubMed Scopus (113) Google Scholar, 3Bruun J.N. Solberg C.O. Hamre E. Janssen C.J.J. Thunold S. Eide J. Acute disseminated phycomycosis in a patient with impaired neutrophil granulocyte function.Acta Pathol Microbiol Scand. 1976; 84: 93-99Google Scholar, 4Dekkers R. Verweij P.E. Weemaes C.M.R. Severijnen R.S.V.M. Van Krieken J.H.J.M. Warris A. Gastrointestinal zygomycosis due to Rhizopus microsporus var. rhizopodiformis as a manifestation of chronic granulomatous disease.Med Mycol. 2008; 46: 491-494Crossref PubMed Scopus (15) Google ScholarTable ISummary of cases of mucormycosis in patients with CGDPatient featuresType of CGDImmunosuppressionClinical diseaseCausative agentOutcome14-year-old boy∗Current report.2Siddiqui S. Anderson V.L. Hilligoss D.M. Abinun M. Kuijpers T.W. Masur H. et al.Fulminant mulch pneumonitis: an emergency presentation of chronic granulomatous disease.Clin Infect Dis. 2007; 45: 673-681Crossref PubMed Scopus (113) Google Scholarp47phoxMethlyprednisolone, 10 mg/kg/d, for 3 d; then prednisone, 1 mg/kg for 7 d; then prednisone, 0.5 mg/kg“Mulch pneumonitis” with multiorgan failureAspergillus fumigatus (lungs, brain); Absidia corymbifera (lungs, liver, spleen, kidneys)Deceased19-year-old man∗Current report.p47phoxPrednisone, 10 mg daily; methotrexate, 25 mg daily (with leucovorin ; hydroxychloroquine, 400 mg dailyPulmonary massRhizopus oryzaeSurvived20-year-old man∗Current report.X-linkedPrednisone, 30 mg dailyPulmonary nodulesRhizopus speciesSurvived24-year-old man∗Current report.X-linkedSteroids equivalent to prednisone, 50–80 mg daily, for 3 wkS aureus–induced septic shock with multiorgan failure and diffuse bilateral pulmonary infiltratesRhizopus microsporus groupDeceased12-year-old boy∗Current report.X-linkedMethylprednisolone, 1.33-2 mg/kg daily, and azathioprine, 50 mg dailyPulmonary and splenic nodules, brain abscess, renal infiltratesRhizopus speciesDeceased13-year-old girl8Kitabchi A.E. Umpierrez G.E. Fisher J.N. Murphy M.B. Stentz F.B. Thirty years of personal experience in hyperglycemic crises: diabetic ketoacidosis and hyperglycemic hyperosmolar state.J Clin Endocrinol Metab. 2008; 93: 1541-1552Crossref PubMed Scopus (120) Google ScholarNSHigh-dose corticosteroids; cyclophosphamideThoracic wall mass; bronchopleural fistula; quadriceps myonecrosis; subarachnoid hemorrhageMucoracious fungi on histopathology; culture not reportedDeceased10-month-old boy9Morena M. Delbosc S. Dupuy A.M. Canaud B. Cristol J.P. Overproduction of reactive oxygen species in end-stage renal disease patients: a potential component of hemodialysis-associated inflammation.Hemodial Int. 2005; 9: 37-46Crossref PubMed Scopus (148) Google ScholarNSPreceding RSV, unclear if corticosteroids administeredMass of small intestineR microsporus var. rhizopodiformisSurvivedNS, Not specified; RSV, respiratory syncytial virus.∗ Current report. Open table in a new tab NS, Not specified; RSV, respiratory syncytial virus. Patient 1 was a 14-year-old boy with fulminant mulch pneumonitis who had Aspergillus fumigatus on lung biopsy.2Siddiqui S. Anderson V.L. Hilligoss D.M. Abinun M. Kuijpers T.W. Masur H. et al.Fulminant mulch pneumonitis: an emergency presentation of chronic granulomatous disease.Clin Infect Dis. 2007; 45: 673-681Crossref PubMed Scopus (113) Google Scholar Adjunctive high-dose steroids were administered for hypoxia, but he died after approximately 1 month. Autopsy found aspergillosis and disseminated Absidia corymbifera. Patient 2 was a 19-year old man who was receiving prednisone, methotrexate, and hydroxychloroquine for autoinflammatory manifestations. Biopsy of an enlarging pulmonary mass revealed Rhizopus oryzae, which was successfully treated with liposomal amphotericin B and posaconazole. Patient 3 was a 20-year-old man with recurrent gastrointestinal obstructions in whom pulmonary nodules developed during high-dose prednisone therapy. Biopsy yielded Rhizopus species. Amphotericin B and tapering of steroids resolved the infection. Patient 4 was a 24-year-old man who presented with Staphylococcus aureus cervical and hepatic abscesses. Radiofrequency ablation of the liver abscesses led to septic shock with multiorgan failure. After 3 weeks of stress-dose steroids, the Rhizopus microsporus group was isolated from lung biopsy. New lesions in the liver, spleen, and brain caused death. Autopsy showed disseminated mucormycosis. Patient 5 was a 12-year-old boy in whom refractory CGD colitis developed, requiring high-dose steroids, azathioprine, and steroid enemas. After 1 month, nodules in the lungs, spleen, and brain grew Rhizopus species. Despite liposomal amphotericin B and posaconazole, he died. Of 279 patients, 5 (1.8%) had definite invasive mucormycosis. All cases were immediately preceded by a steroid-based immunosuppressive regimen for 3 or more weeks. None of the patients had neutropenia, metabolic acidosis, or iron overload. Review of the literature identified 2 other cases of mucormycosis in patients with CGD (Table I). Furthermore, spontaneous mucormycosis was reported only once out of 16 fungal pneumonias in 72 mice with CGD.5Bingel S.A. Pathology of a mouse model of x-linked chronic granulomatous disease.Contemp Top Lab Anim Sci. 2002; 41: 33-38PubMed Google Scholar Molds infect a broad range of immunocompromised hosts. Although invasive aspergillosis is most common in patients with hematologic malignancy or transplants, mucormycosis has the same demographic niche but also affects those with diabetic ketoacidosis and those receiving iron chelation. Despite the environmental ubiquity of both these molds, the host factors relating to differential susceptibility are ill defined. CGD provides an opportunity to examine susceptibility to these 2 mycoses within the framework of a defined immune defect. Although patients with CGD are uniquely susceptible to spontaneous invasive aspergillosis, spontaneous mucormycosis in patients with CGD is rare. In our cohort mucormycosis only occurred in patients receiving significant immunosuppression for several weeks. Differences in environmental exposure do not seem to account for this discrepancy because both Aspergillus species and Mucorales were identified from 4 patients with CGD with mulch pneumonitis.2Siddiqui S. Anderson V.L. Hilligoss D.M. Abinun M. Kuijpers T.W. Masur H. et al.Fulminant mulch pneumonitis: an emergency presentation of chronic granulomatous disease.Clin Infect Dis. 2007; 45: 673-681Crossref PubMed Scopus (113) Google Scholar None of these latter 4 patients had been receiving immunosuppression at the time of exposure; none had mucormycosis. Although those 4 patients were initiated on steroids for their pneumonitis, duration was typically less than 2 weeks. In addition to susceptibility to infections, patients with CGD have dysregulated hyperinflammatory responses with granuloma formation and visceral obstruction. Although intense immunosuppression (eg, 1 mg/kg/d prednisone) typically relieves these complications, prolonged high-dose treatment might be permissive for infections not typical of CGD, such as mucormycosis. However, lower-dose steroid therapy (<0.5 mg/kg/d) is well tolerated without any apparent increased risk for invasive mold infection. Predisposition of patients with CGD to invasive aspergillosis, particularly with the weakly virulent Aspergillus nidulans, suggests that NADPH oxidase–related pathways are central to the control of Aspergillus species. It is therefore notable that Mucorales, agents that occupy a similar environmental niche and are clearly capable of causing fatal invasive infection in the proper setting, do not cause disease in patients with CGD without superimposed immune impairment. These observations suggest that NADPH oxidase–independent pathways are chiefly responsible for resistance to Mucorales and that these pathways must be precisely those that are being lesioned among the patients with CGD with mucormycosis. However, CGD appears to be a prerequisite for this permissiveness, likely interacting with iatrogenic macrophage/T-cell immunosuppression to decrease some critical threshold of resistance because these modest immunosuppressive maneuvers do not by themselves lead to mucormycosis in otherwise immunocompetent individuals. Based on these observations and review of the literature, a model of immunity to these mycoses is proposed. To cause disease, spores must germinate to produce hyphae, activating neutrophils, macrophages, and T cells. When both the NADPH oxidase (in neutrophils and macrophages) and oxidase-independent pathways (in neutrophils, macrophages, and T cells) are intact, no mycosis develops. In patients with CGD, the anti–Aspergillus species activity is fragile but apparently sufficient to permit clearance of the low levels of spores typically encountered (approximately 200-2000/d). High-level exposure (eg, mulching and composting) overwhelms this fragile defense, and spores germinate to hyphae. On the other hand, NADPH oxidase–independent mechanisms effectively clear the sporangiospores of Mucorales, preventing disease. In patients with CGD receiving high-dose immunosuppression that affects macrophages and T cells, susceptibility to mucormycosis is apparently enhanced. In patients with diabetes mellitus, NADPH oxidase activity is not impaired or is actually enhanced. In diabetic ketoacidosis acidosis might favor iron acquisition by Mucorales,6Kontoyiannis D.P. Lewis R.E. Invasive Zygomycosis: Update on Pathogenesis, Clinical Manifestations, and Management.Infect Dis Clin North Am. 2006; 20: 581-607Abstract Full Text Full Text PDF PubMed Scopus (194) Google Scholar and there is alteration of monocyte and T-cell functions.7Stewart J. Collier A. Patrick A.W. Clarke B.F. Weir D.M. Alterations in monocyte receptor function in type 1 diabetic patients with ketoacidosis.Diabet Med. 1991; 8: 213-216Crossref PubMed Scopus (8) Google Scholar, 8Kitabchi A.E. Umpierrez G.E. Fisher J.N. Murphy M.B. Stentz F.B. Thirty years of personal experience in hyperglycemic crises: diabetic ketoacidosis and hyperglycemic hyperosmolar state.J Clin Endocrinol Metab. 2008; 93: 1541-1552Crossref PubMed Scopus (120) Google Scholar Patients undergoing dialysis frequently have enhanced NADPH oxidase activity,9Morena M. Delbosc S. Dupuy A.M. Canaud B. Cristol J.P. Overproduction of reactive oxygen species in end-stage renal disease patients: a potential component of hemodialysis-associated inflammation.Hemodial Int. 2005; 9: 37-46Crossref PubMed Scopus (148) Google Scholar and mucormycosis typically occurs only when deferoxamine is used to chelate iron. The iron-deferoxamine complex might actually favor iron uptake by Rhizopus species, thereby enhancing growth.6Kontoyiannis D.P. Lewis R.E. Invasive Zygomycosis: Update on Pathogenesis, Clinical Manifestations, and Management.Infect Dis Clin North Am. 2006; 20: 581-607Abstract Full Text Full Text PDF PubMed Scopus (194) Google Scholar In these latter patient groups, enhanced NADPH oxidase activity might explain the relative paucity of aspergillosis, indicating that it is the impairment of oxidase–independent pathways leading to mucormycosis. Fungal infections in patients with CGD, both with and without additional immunosuppression, provide insight into human immunity to Aspergillus species and Mucorales. NADPH oxidase–dependent pathways are critical for immunity to Aspergillus species, whereas oxidase–independent pathways, including those involving T lymphocytes, appear to be key for defense against Mucorales." @default.
• W2079334811 created "2016-06-24" @default.
• W2079334811 creator A5013149726 @default.
• W2079334811 creator A5025311094 @default.
• W2079334811 creator A5044250397 @default.
• W2079334811 creator A5056552919 @default.
• W2079334811 creator A5063867007 @default.
• W2079334811 creator A5089792719 @default.
• W2079334811 creator A5090229750 @default.
• W2079334811 date "2009-06-01" @default.
• W2079334811 modified "2023-10-17" @default.
• W2079334811 title "Mucormycosis in chronic granulomatous disease: Association with iatrogenic immunosuppression" @default.
• W2079334811 cites W1980613033 @default.
• W2079334811 cites W2008366252 @default.
• W2079334811 cites W2022539296 @default.
• W2079334811 cites W2032671153 @default.
• W2079334811 cites W2127335431 @default.
• W2079334811 cites W2166734004 @default.
• W2079334811 cites W2169518694 @default.
• W2079334811 doi "https://doi.org/10.1016/j.jaci.2009.02.020" @default.
• W2079334811 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/4103906" @default.
• W2079334811 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/19368967" @default.
• W2079334811 hasPublicationYear "2009" @default.
• W2079334811 type Work @default.
• W2079334811 sameAs 2079334811 @default.
• W2079334811 citedByCount "40" @default.
• W2079334811 countsByYear W20793348112012 @default.
• W2079334811 countsByYear W20793348112013 @default.
• W2079334811 countsByYear W20793348112014 @default.
• W2079334811 countsByYear W20793348112015 @default.
• W2079334811 countsByYear W20793348112016 @default.
• W2079334811 countsByYear W20793348112017 @default.
• W2079334811 countsByYear W20793348112018 @default.
• W2079334811 countsByYear W20793348112019 @default.
• W2079334811 countsByYear W20793348112020 @default.
• W2079334811 countsByYear W20793348112021 @default.
• W2079334811 countsByYear W20793348112022 @default.
• W2079334811 countsByYear W20793348112023 @default.
• W2079334811 crossrefType "journal-article" @default.
• W2079334811 hasAuthorship W2079334811A5013149726 @default.
• W2079334811 hasAuthorship W2079334811A5025311094 @default.
• W2079334811 hasAuthorship W2079334811A5044250397 @default.
• W2079334811 hasAuthorship W2079334811A5056552919 @default.
• W2079334811 hasAuthorship W2079334811A5063867007 @default.
• W2079334811 hasAuthorship W2079334811A5089792719 @default.
• W2079334811 hasAuthorship W2079334811A5090229750 @default.
• W2079334811 hasBestOaLocation W20793348111 @default.
• W2079334811 hasConcept C142724271 @default.
• W2079334811 hasConcept C16005928 @default.
• W2079334811 hasConcept C203014093 @default.
• W2079334811 hasConcept C2777292849 @default.
• W2079334811 hasConcept C2778175462 @default.
• W2079334811 hasConcept C2779134260 @default.
• W2079334811 hasConcept C2780252810 @default.
• W2079334811 hasConcept C2991700627 @default.
• W2079334811 hasConcept C71924100 @default.
• W2079334811 hasConceptScore W2079334811C142724271 @default.
• W2079334811 hasConceptScore W2079334811C16005928 @default.
• W2079334811 hasConceptScore W2079334811C203014093 @default.
• W2079334811 hasConceptScore W2079334811C2777292849 @default.
• W2079334811 hasConceptScore W2079334811C2778175462 @default.
• W2079334811 hasConceptScore W2079334811C2779134260 @default.
• W2079334811 hasConceptScore W2079334811C2780252810 @default.
• W2079334811 hasConceptScore W2079334811C2991700627 @default.
• W2079334811 hasConceptScore W2079334811C71924100 @default.
• W2079334811 hasIssue "6" @default.
• W2079334811 hasLocation W20793348111 @default.
• W2079334811 hasLocation W20793348112 @default.
• W2079334811 hasLocation W20793348113 @default.
• W2079334811 hasLocation W20793348114 @default.
• W2079334811 hasOpenAccess W2079334811 @default.
• W2079334811 hasPrimaryLocation W20793348111 @default.
• W2079334811 hasRelatedWork W2079334811 @default.
• W2079334811 hasRelatedWork W2127335431 @default.
• W2079334811 hasRelatedWork W2345591107 @default.
• W2079334811 hasRelatedWork W2918969274 @default.
• W2079334811 hasRelatedWork W3118897809 @default.
• W2079334811 hasRelatedWork W3164560068 @default.
• W2079334811 hasRelatedWork W3213512676 @default.
• W2079334811 hasRelatedWork W4297184974 @default.
• W2079334811 hasRelatedWork W4376106206 @default.
• W2079334811 hasRelatedWork W67032640 @default.
• W2079334811 hasVolume "123" @default.
• W2079334811 isParatext "false" @default.
• W2079334811 isRetracted "false" @default.
• W2079334811 magId "2079334811" @default.
• W2079334811 workType "article" @default.