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Chronic granulomatous disease

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https://www.readbyqxmd.com/read/29782813/actinomycotic-osteomyelitis-of-the-mandible-diagnosed-using-matrix-assisted-laser-desorption-ionization-time-of-flight-mass-spectrometry-a-case-report
#1
Shinsuke Yamamoto, Hiroshi Takegawa, Naoki Taniike, Toshihiko Takenobu
Actinomycosis is a rare, chronic, slowly progressive granulomatous disease caused by filamentous gram-positive anaerobic bacteria from the Actinomycetaceae family (genus Actinomyces). It has become a rare condition because of the widespread use of antibiotics. When clinical symptoms are not typical, diagnosis of this condition becomes difficult. This report describes a case involving an 82-year-old woman who was diagnosed with actinomycotic osteomyelitis of the mandible using matrix assisted laser desorption ionization-time-of-flight mass spectrometry (MALDI-TOF MS)...
April 26, 2018: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/29774526/retrotransposable-genetic-elements-causing-neutrophil-defects
#2
REVIEW
Dirk Roos, Martin de Boer
Retrotransposable elements are stretches of DNA that encode proteins with the inherent ability to insert their own RNA or another RNA by reverse transcriptase as DNA into a new genomic location. In humans, the only autonomous retrotransposable elements are members of the Long INterspersed Element-1 (LINE-1) family. LINE-1s may cause gene inactivation and human disease. We present a brief summary of the published knowledge about LINE-1s in humans and the RNAs that these elements can transpose, and we focus on the effect of LINE-1-mediated retrotransposition on human neutrophil function...
May 17, 2018: European Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29769775/primary-gingival-tuberculosis-in-pregnancy-a-rare-combination
#3
Shweta Sharma, Abdul Ahad, Narinder Dev Gupta, Vivek Kumar Sharma
Tuberculosis (TB), a common chronic-specific granulomatous disease, has become rare in the developed countries. However, it is still a common cause of morbidity and mortality in India. Although it commonly involves the lungs, its presentation in the oral cavity is quite uncommon. The very rare incidence of primary gingival TB, particularly in the absence of active pulmonary involvement often leads to misdiagnosis. Clinical features of oral lesions may include ulceration, nodules, granulomas, and fissures. TB in pregnant women is a major cause of obstetric complications and increased risk of maternal and child mortality...
March 2018: Journal of Indian Society of Periodontology
https://www.readbyqxmd.com/read/29766816/a-systematic-review-of-the-literature-of-the-three-related-disease-entities-cheilitis-granulomatosa-orofacial-granulomatosis-and-melkersson-rosenthal-syndrome
#4
Goetz Wehl, Markus Rauchenzauner
BACKGROUND AND OBJECTIVE: Melkersson Rosenthal syndrome (MRS) is a rare disorder of unknown etiology and comprises the triad: orofacial edema, recurrent facial paralysis and lingua plicata. In the current literature confusing heterogeneity exists, mixing together the historically grown terms cheilitis granulomatosa or granulomatous cheilitis, Melkersson Rosenthal syndrome and the umbrella term orofacial granulomatosis (OFG). METHODS: We provide a systematic review comprising all three disease entities of orofacial granulomatosis using the computerized database "Pubmed Medline" entering the key words "orofacial granulomatosis" (141 references), "Melkersson-Rosenthal syndrome" (207 references), "granulomatous cheilitis" or "cheilitis granulomatosa" (102 references) back to 1956...
May 14, 2018: Current Pediatric Reviews
https://www.readbyqxmd.com/read/29755533/new-onset-colitis-in-an-adult-patient-with-chronic-granulomatous-disease-treated-with-hematopoietic-stem-cell-transplantation-a-diagnostic-dilemma
#5
Kara Robertson, Stephen Couban, Desmond Leddin, Imran Ahmad, Lori Connors
Background: Chronic granulomatous disease (CGD) is a rare primary immunodeficiency characterized by recurrent life-threatening bacterial and fungal infections, granuloma formation and intestinal disease. This disease is caused by defects in NADPH oxidase, which result in the inability of phagocytes (neutrophils, monocytes and macrophages) to destroy certain microbes. The only established curative therapy for CGD is hematopoietic stem cell transplantation. Case presentation: A 23-year-old Caucasian male with X-linked chronic granulomatous disease underwent a reduced-intensity conditioning, matched unrelated donor peripheral blood stem cell transplant, after which he was started on tacrolimus and mycophenolate for graft-versus-host disease prophylaxis...
2018: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/29754190/allergic-and-immunologic-perspectives-of-inflammatory-bowel-disease
#6
REVIEW
Kofi Clarke, Jayakrishna Chintanaboina
Inflammatory bowel disease (IBD) is a chronic immune-mediated inflammatory condition primarily involving the gastrointestinal tract. It includes Crohn's disease (CD), ulcerative colitis (UC), and a less common phenotype-indeterminate colitis. It is thought to result from a complex interplay of environmental, microbial, and host factors including genetic factors, although the exact mechanism is not known. Dietary factors have been shown to play a role in the pathogenesis of IBD and can potentially alter the intestinal microbiota as well as disrupt the immune function in the gut...
May 12, 2018: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/29753791/obstructive-lymphangitis-precedes-colitis-in-murine-norovirus-infected-stat1-deficient-mice
#7
Audrey Seamons, Piper M Treuting, Stacey Meeker, Charlie Hsu, Jisun Paik, Thea Brabb, Sabine S Escobar, Jonathan S Alexander, Aaron Ericsson, Jason G Smith, Lillian Maggio-Price
Murine norovirus (MNV) is an RNA virus that can prove lethal in mice with impaired innate immunity. We found that MNV-4 infection of Stat1-/- mice was not lethal, but produced a 100% penetrant, previously undescribed lymphatic phenotype characterized by chronic-active lymphangitis with hepatitis, splenitis, and chronic cecal and colonic inflammation. Lesion pathogenesis progressed from early ileal enteritis and regional dilated lymphatics to lymphangitis, granulomatous changes in the liver and spleen, and ultimately, typhlocolitis...
May 10, 2018: American Journal of Pathology
https://www.readbyqxmd.com/read/29746681/prevention-of-infectious-complications-in-patients-with-chronic-granulomatous-disease
#8
Maria A Slack, Isaac P Thomsen
Chronic granulomatous disease (CGD) is a primary immunodeficiency that confers a markedly increased risk of bacterial and fungal infections caused by certain opportunistic pathogens. Current evidence supports the use of prophylactic antibacterial, antifungal, and immunomodulatory therapies designed to prevent serious or life-threatening infections in patients with CGD. In this review, we discuss current strategies for the prevention of infections in children and adults with CGD and the evidence that supports those strategies...
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29746680/allogeneic-hematopoietic-cell-transplantation-for-chronic-granulomatous-disease-controversies-and-state-of-the-art
#9
James A Connelly, Rebecca Marsh, Suhag Parikh, Julie-An Talano
Chronic granulomatous disease (CGD) is a congenital disorder characterized by recurrent life-threatening bacterial and fungal infections and development of severe inflammation secondary to a congenital defect in 1 of the 5 phagocyte oxidase (phox) subunits of the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. Hematopoietic cell transplant (HCT) is a curative treatment for patients with CGD that provides donor neutrophils with functional NADPH and superoxide anion production. Many characteristics of CGD, including preexisting infection and inflammation and the potential for cure with mixed-donor chimerism, influence the transplant approach and patient outcome...
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29746679/noninfectious-manifestations-and-complications-of-chronic-granulomatous-disease
#10
Sarah E Henrickson, Artemio M Jongco, Kelly F Thomsen, Elizabeth K Garabedian, Isaac P Thomsen
Chronic granulomatous disease (CGD), a primary immunodeficiency characterized by a deficient neutrophil oxidative burst and the inadequate killing of microbes, is well known to cause a significantly increased risk of invasive infection. However, infectious complications are not the sole manifestations of CGD; substantial additional morbidity is driven by noninfectious complications also. These complications can include, for example, a wide range of inflammatory diseases that affect the gastrointestinal tract, lung, skin, and genitourinary tract and overt autoimmune disease...
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29746678/infectious-complications-in-patients-with-chronic-granulomatous-disease
#11
Nicholas Bennett, Paul J Maglione, Benjamin L Wright, Christa Zerbe
No abstract text is available yet for this article.
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29746677/from-fatal-to-chronic-granulomatous-diseases-of-childhood
#12
Paul G Quie
No abstract text is available yet for this article.
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29746676/future-of-care-for-patients-with-chronic-granulomatous-disease-gene-therapy-and-targeted-molecular-medicine
#13
Michael D Keller, Luigi D Notarangelo, Harry L Malech
Chronic granulomatous disease is a rare and potentially fatal disorder of neutrophil function. Beyond current medical management and hematopoietic stem cell transplantation, new methods of gene therapy that use lentiviral vectors or gene editing might extend curative therapies to patients who lack a suitable transplantation donor while eliminating the risk of graft-versus-host disease. Furthermore, new therapies focused on altering the biology of phagolysosomes might offer novel targeted treatments for inflammatory complications in patients with chronic granulomatous disease...
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29746675/chronic-granulomatous-disease-epidemiology-pathophysiology-and-genetic-basis-of-disease
#14
N L Rider, M B Jameson, C B Creech
Chronic Granulomatous Disease is one of the classic primary immunodeficiencies of childhood. While the incidence and severity of bacterial and fungal infections have been greatly reduced in this patient population, much remains to be learned about the pathophysiology of the disease, particularly for autoinflammatory manifestations. In this review, we examine the epidemiology, pathophysiology, and genetic basis for CGD.
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29746674/considerations-in-the-diagnosis-of-chronic-granulomatous-disease
#15
Joyce E Yu, Antoine E Azar, Hey J Chong, Artemio M Jongco, Benjamin T Prince
Chronic granulomatous disease (CGD) is a rare primary immunodeficiency that is caused by defects in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase complex. The disease presents in most patients initially with infection, especially of the lymph nodes, lung, liver, bone, and skin. Patients with CGD are susceptible to a narrow spectrum of pathogens, and Staphylococcus aureus, Burkholderia cepacia complex, Serratia marcescens, Nocardia species, and Aspergillus species are the most common organisms implicated in North America...
May 9, 2018: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/29746460/a-holistic-approach-to-the-mycetoma-management
#16
Sahar Mubarak Bakhiet, Ahmed Hassan Fahal, Ahmed Mudawi Musa, El Samani Wadaa Mohamed, Rowa Fathelrahman Omer, Eiman Siddig Ahmed, Mustafa El Nour, El Rayah Mohamed Mustafa, Manar El Sheikh A Rahman, Suliman Hussein Suliman, Mohamed A Gadir El Mamoun, Hajo Mohamed El Amin
Mycetoma, one of the badly neglected tropical diseases, it is a localised chronic granulomatous inflammatory disease characterised by painless subcutaneous mass and formation of multiple sinuses that produce purulent discharge and grains. If untreated early and appropriately, it usually spread to affect the deep structures and bone resulting in massive damage, deformities and disabilities. It can also spread via the lymphatics and blood leading to distant secondary satellites associated with high morbidity and mortality...
May 2018: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/29744786/correction-to-raised-serum-il-8-levels-are-associated-with-excessive-fatigue-in-female-carriers-of-x-linked-chronic-granulomatous-disease-in-the-uk
#17
Alexandra C Battersby, Alexander J Martin, Jessica Tarn, W F Ng, Catherine M Cale, David Goldblatt, Andrew R Gennery
The original version of the article, "Raised Serum IL-8 Levels Are Associated with Excessive Fatigue in Female Carriers of X-Linked Chronic Granulomatous Disease in the UK" incorrectly listed the name of the fourth author as Fai W. Ng. The correct spelling of the author's name is WF Ng.
April 23, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29743881/host-defense-versus-immunosuppression-unisexual-infection-with-male-or-female-schistosoma-mansoni-differentially-impacts-the-immune-response-against-invading-cercariae
#18
Martina Sombetzki, Nicole Koslowski, Anne Rabes, Sonja Seneberg, Franziska Winkelmann, Carlos Fritzsche, Micha Loebermann, Emil C Reisinger
Infection with the intravascular diecious trematode Schistosoma spp . remains a serious tropical disease and public health problem in the developing world, affecting over 258 million people worldwide. During chronic Schistosoma mansoni infection, complex immune responses to tissue-entrapped parasite eggs provoke granulomatous inflammation which leads to serious damage of the liver and intestine. The suppression of protective host immune mechanisms by helminths promotes parasite survival and benefits the host by reducing tissue damage...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29741690/severe-multisystem-involvement-of-chronic-granulomatous-disease-in-a-pediatric-patient
#19
Zuhal Bayramoglu, Ibrahim Adaletli, Emine Caliskan, Manolya Acar, Selda Hancerli Torun, Ayper Somer
Chronic granulomatous disease (CGD) is a rare primary immunodeficiency disorder identified by recurrent pyogenic and fungal infections infections secondary to defective nicotinamide adenine dinucleotide phosphate oxidase enzyme. In the present study, we demonstrated a case with a history of multiple segmental lung resections because of invasive bronchopulmonary aspergillosis, multifocal hepatic and splenic granulomas, bilateral adnexal calcific foci presumed to be related with old granulomatous infection and finally gastric outlet obstruction secondary to the involvement of the stomach wall thickening with granulomatous tissue...
May 7, 2018: Journal of Tropical Pediatrics
https://www.readbyqxmd.com/read/29725938/gamma-delta-t-cell-expansion-in-whipple-s-disease-with-muscular-granulomatous-vasculitis
#20
Didier Brönnimann, Marie-Anne Vandenhende, Jean-François Viallard
Whipple's disease usually presents as chronic joint pain followed by digestive manifestations. However, many different presentations have been described in the literature. We report here the first proven case of muscular vasculitis related to Whipple's disease, associated with an expansion of circulating activated γδ T lymphocytes.
May 3, 2018: Infection
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