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Chronic granulomatous disease

Qinhua Zhou, Xiaoying Hui, Wenjing Ying, Jia Hou, Wenjie Wang, Danru Liu, Ying Wang, Yeheng Yu, Jingyi Wang, Jinqiao Sun, Qian Zhang, Xiaochuan Wang
PURPOSE: Clinical diagnosis and treatment for chronic granulomatous disease (CGD) have advanced greatly in recent years. However, CGD patients in China have unique clinical features and infection spectrums, which are challenging to their caretakers. Here, we summarized the clinical characteristics, genetic features, treatment, and prognosis of CGD in a single center in Shanghai. METHODS: One hundred sixty-nine CGD patients were recruited between January 2004 and May 2017 based on clinical diagnosis...
March 20, 2018: Journal of Clinical Immunology
Valérie Besnard, Alain Calender, Diane Bouvry, Yves Pacheco, Catherine Chapelon-Abric, Florence Jeny, Hilario Nunes, Carole Planès, Dominique Valeyre
BACKGROUND: Sarcoidosis is a systemic disease characterized by the formation of immune granulomas in various organs, mainly the lungs and the lymphatic system. Exaggerated granulomatous reaction might be triggered in response to unidentified antigens in individuals with genetic susceptibility. The present study aimed to determine the genetic variants implicated in a familial case of sarcoidosis. METHODS: Sarcoidosis presentation and history, NOD2 profile, NF-κB and cytokine production in blood monocytes/macrophages were evaluated in individuals from a family with late appearance of sarcoidosis...
March 20, 2018: Respiratory Research
Djalma A Alves da Silva, Marcos V da Silva, Cleyson C Oliveira Barros, Patrícia B Dias Alexandre, Rodolfo P Timóteo, Jonatas S Catarino, Helioswilton Sales-Campos, Juliana R Machado, Denise B R Rodrigues, Carlo J Oliveira, Virmondes Rodrigues
Tuberculosis (TB) is a granulomatous disease that has affected humanity for thousands of years. The production of cytokines, such as IFN-γ and TNF-α, is fundamental in the formation and maintenance of granulomas and in the control of the disease. Recently, the introduction of TNF-α-blocking monoclonal antibodies, such as Infliximab, has brought improvements in the treatment of patients with chronic inflammatory diseases, but this treatment also increases the risk of reactivation of latent tuberculosis. Our objective was to analyze, in an in vitro model, the influence of Infliximab on the granulomatous reactions and on the production of antigen-specific cytokines (TNF-α, IFN-γ, IL-12p40, IL-10 and IL-17) from beads sensitized with soluble Bacillus Calmette-Guérin (BCG) antigens cultured in the presence of peripheral blood mononuclear cells (PBMC) from TB patients...
2018: PloS One
You-Hong Fang, You-You Luo, Jin-Dan Yu, Jin-Gan Lou, Jie Chen
AIM: To analyze clinical differences between monogenic and nonmonogenic very-early-onset inflammatory bowel disease (VEO-IBD) and to characterize monogenic IBD phenotypically and genotypically via genetic testing. METHODS: A retrospective analysis of children aged 0 to 6 years diagnosed with VEO-IBD in a tertiary hospital in southern China from 2005 to 2017 was performed. Clinical data for VEO-IBD patients were collected, and genetic characteristics were analyzed using whole exome sequencing or target gene panel sequencing...
March 7, 2018: World Journal of Gastroenterology: WJG
Edward Hadas, Marcin Ozarowski, Monika Derda, Barbara Thiem, Marcin Cholewinski, Lukasz Skrzypczak, Agnieszka Gryszczynska, Anna Piasecka
Chronic progressive diseases of the central nervous system such as granulomatous amoebic encephalitis, amoebic keratitis, amoebic pneumonitis and also skin infections caused by free-living amoebae (Acanhamoeba spp.) are a significant challenge for pharmacotherapy. This is due to the lack of effective treatment because of encystation, which makes the amoebae highly resistant to anti-amoebic drugs. A very inter- esting and promising source of future drugs in this area are plant materials obtained not only from the habitat but also from plant in vitro culture as an alternative source of biomaterials...
May 2017: Acta Poloniae Pharmaceutica
Joana Barroso Amaral, Artur Augusto Paiva, Fabiana Viana Ramos, Marie José Stasia, Sónia Gaspar Lemos
No abstract text is available yet for this article.
March 2018: Annals of Allergy, Asthma & Immunology
Duran Sürün, Joachim Schwäble, Ana Tomasovic, Roy Ehling, Stefan Stein, Nina Kurrle, Harald von Melchner, Frank Schnütgen
The CRISPR/Cas9 prokaryotic adaptive immune system and its swift repurposing for genome editing enables modification of any prespecified genomic sequence with unprecedented accuracy and efficiency, including targeted gene repair. We used the CRISPR/Cas9 system for targeted repair of patient-specific point mutations in the Cytochrome b-245 heavy chain gene (CYBB), whose inactivation causes chronic granulomatous disease (XCGD)-a life-threatening immunodeficiency disorder characterized by the inability of neutrophils and macrophages to produce microbicidal reactive oxygen species (ROS)...
March 2, 2018: Molecular Therapy. Nucleic Acids
Waseem Hajjar, Iftikhar Ahmed, Samiha Aljetaily, Tarfah Al-Obaidan, Adnan W Hajjar
Diagnosing endobronchial tuberculosis (EBTB) can be difficult due to the lack of specific signs and symptoms that differentiate it from other respiratory diseases, such as lung tumors. We hereby report a case of a very rare presentation of tuberculosis (TB) in a patient who presented with a dry cough and significant weight loss for 3 months. Chest X-ray and CT scan of the chest showed partial atelectasis and a segmental collapse of the right upper lobe and tumor-like arising from its bronchus along with a large right para-tracheal mediastinal lymphadenopathy, mimicking a metastatic (N2) disease...
March 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Eloise Ballard, Willem J G Melchers, Jan Zoll, Alistair J P Brown, Paul E Verweij, Adilia Warris
In order to survive, Aspergillus fumigatus must adapt to specific niche environments. Adaptation to the human host includes modifications facilitating persistent colonisation and the development of azole resistance. The aim of this study is to advance understanding of the genetic and physiological adaptation of A. fumigatus in patients during infection and treatment. Thirteen A. fumigatus strains were isolated from a single chronic granulomatous disease patient suffering from persistent and recurrent invasive aspergillosis over a period of 2 years...
February 22, 2018: Fungal Genetics and Biology: FG & B
Carlos A M Silva, John T Belisle
The spectrum of clinical forms observed in leprosy and its pathogenesis are dictated by the host's immune response against Mycobacterium leprae , the etiological agent of leprosy. Previous results, based on metabolomics studies, demonstrated a strong relationship between clinical manifestations of leprosy and alterations in the metabolism of ω3 and ω6 polyunsaturated fatty acids (PUFAs), and the diverse set of lipid mediators derived from PUFAs. PUFA-derived lipid mediators provide multiple functions during acute inflammation, and some lipid mediators are able to induce both pro- and anti-inflammatory responses as determined by the cell surface receptors being expressed, as well as the cell type expressing the receptors...
2018: Frontiers in Immunology
M Hensel, A Rodrigues Hoffmann, M Gonzales, M A Owston, E J Dick
Histoplasma capsulatum var. duboisii (Hcd) infections have been well documented to cause chronic granulomatous disease, mainly involving the skin of baboons and humans in African countries primarily. This retrospective study classified the subspecies of Histoplasma and developed a phylogenetic tree utilizing DNA sequences extracted from formalin-fixed, paraffin embedded (FFPE) tissues from 9 baboons from a research colony in Texas histologically diagnosed with Hcd. Based on sequence analysis of ITS-2, Tub-1, and ARF, Hcd isolated from the archived samples closely aligns with the African clade and has 88% sequence homology with a sample isolated from an individual in Senegal...
February 16, 2018: Medical Mycology: Official Publication of the International Society for Human and Animal Mycology
Paola U Facco, Michele Baziotti Man, Cristiane Martin, Angelo Sementilli, Luiza F Rocha, Maria Celia C Ciaccia, José Roberto P Almeida, Sandra Dinato, Paola Vendramini, Ellen O Dantas, Nuria Zurro, Antonio Condino-Neto, Vera E V Rullo
The original version of abstract PO-162 "Chronic Granulomatous Disease in a Brazilian Patient Mimetizing Sarcoidosis" incorrectly listed the name of the second author as Micheli Barsioti. The correct spelling of the author's name is Michele Baziotti Man.
February 22, 2018: Journal of Clinical Immunology
M Rato, F Gil, A F Monteiro, J Aranha, E Tavares
A 55-year-old man presented with a history of asymptomatic, bilateral, figurate dermatosis consisting of erythematous, annular, linear, andarciform, indurated nodules and plaques on the lateral walls of thorax, flanks, and hypogastrium. His medical history was positive for chronic hepatitis C. Skin biopsy was compatible with the diagnosisof interstitial granulomatous dermatitis. Additionalinvestigation revealed positive rheumatoid factor, antinuclear antibodies, and cryoglobulins. Thepatient started treatment with high-potency topical corticosteroid...
January 15, 2018: Dermatology Online Journal
Fan Di Xia, Amy Ly, Gideon P Smith
Idiopathic granulomatous mastitis (IGM) is a benign chronic inflammatory breast disease of unknown etiology. No consensus exists as to the best therapeutic approach, though treatment choices include antibiotics, drainage, surgical excision, steroids, methotrexate, and observation. Herein we report a case of idiopathic granulomatous mastitis that was refractory to methotrexate and intralesional and systemic steroids but responded well to mycophenolate mofetil 1500mg twice daily. To our knowledge, this is the first report of the effective use of mycophenolate mofetil in idiopathic granulomatous mastitis...
July 15, 2017: Dermatology Online Journal
Seth M Daly, Carolyn R Sturge, Kimberly R Marshall-Batty, Christina F Felder-Scott, Raksha Jain, Bruce Geller, David Greenberg
The Burkholderia cepacia complex is a group of gram-negative bacteria that are opportunistic pathogens in immunocompromised individuals, such as those with cystic fibrosis (CF) or chronic granulomatous disease (CGD). Burkholderia are intrinsically resistant to many antibiotics and the lack of antibiotic development necessitates novel therapeutics. Peptide-conjugated phosphorodiamidate morpholino oligomers are antisense molecules that inhibit bacterial mRNA translation. Targeting of PPMOs to the gene acpP, which is essential for membrane synthesis, lead to defects in the membrane and ultimately bactericidal activity...
February 20, 2018: ACS Infectious Diseases
Devra D Huey, Brad Bolon, Krista M D La Perle, Priya Kannian, Steven Jacobson, Lee Ratner, Patrick L Green, Stefan Niewiesk
Chronic infection with human T-cell leukemia virus type 1 (HTLV1) can lead to adult T-cell leukemia (ATL). In contrast, infection with HTLV2 does not lead to leukemia, potentially because of distinct virus-host interactions and an active immune response that controls virus replication and, therefore, leukemia development. We created a humanized mouse model by injecting human umbilical-cord stem cells into the livers of immunodeficient neonatal NSG mice, resulting in the development of human lymphocytes that cannot mount an adaptive immune response...
February 1, 2018: Comparative Medicine
J W Baddley, F Cantini, D Goletti, J J Gómez-Reino, E Mylonakis, R San-Juan, M Fernández-Ruiz, J Torre-Cisneros
BACKGROUND: The present review is part of the ESCMID Study Group for Infections in Compromised Hosts (ESGICH) Consensus Document on the safety of targeted and biological therapies. AIMS: To review, from an Infectious Diseases perspective, the safety profile of agents targeting tumour necrosis factor-α (TNF-α) and to suggest preventive recommendations. SOURCES: Computer-based MEDLINE searches with MeSH terms pertaining to each agent or therapeutic family...
February 6, 2018: Clinical Microbiology and Infection
Shanmugasundaram Karuppusamy, Lucy Mutharia, David Kelton, Niel Karrow, Gordon Kirby
Johne's disease (JD) is a contagious, chronic granulomatous enteritis of ruminants caused by Mycobacterium avium subsp. paratuberculosis (MAP). The aim of this study was to identify antigenic proteins from the MAP cell envelope (i.e. cell wall and cytoplasmic membranes) by comparing MAP, M. avium subsp. hominissuis (MAH) and M. smegmatis (MS) cell envelope protein profiles using a proteomic approach. Composite two-dimensional (2D) difference gel electrophoresis images revealed 13 spots present only in the image of the MAP cell envelope proteins...
January 24, 2018: Microbiology
Kadir González, Rosendo Diaz, Aurea F Ferreira, Víctor García, Héctor Paz, José E Calzada, Michelle Ruíz, Márcia Laurenti, Azael Saldaña
Cutaneous leishmaniasis (CL) is an endemic disease in the Republic of Panama, caused by Leishmania (Viannia) parasites, whose most common clinical manifestation is the presence of ulcerated lesions on the skin. These lesions usually present a chronic inflammatory reaction, sometimes granulomatous, with the presence of lymphocytes, plasma cells and macrophages. This study describes the histopathological characteristics found in the skin lesions of patients with CL caused by Leishmania (V.) panamensis in Panama...
2018: Revista do Instituto de Medicina Tropical de São Paulo
Khalid Shahin, Jose Gustavo Ramirez-Paredes, Graham Harold, Benjamin Lopez-Jimena, Alexandra Adams, Manfred Weidmann
Francisella noatunensis subsp. orientalis (Fno) is the causative agent of piscine francisellosis in warm water fish including tilapia. The disease induces chronic granulomatous inflammation with high morbidity and can result in high mortality. Early and accurate detection of Fno is crucial to set appropriate outbreak control measures in tilapia farms. Laboratory detection of Fno mainly depends on bacterial culture and molecular techniques. Recombinase polymerase amplification (RPA) is a novel isothermal technology that has been widely used for the molecular diagnosis of various infectious diseases...
2018: PloS One
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