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Chronic granulomatous disease

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https://www.readbyqxmd.com/read/27913461/primary-immune-deficiencies-with-defects-in-neutrophil-function
#1
Mary C Dinauer
Immune deficiencies resulting from inherited defects in neutrophil function have revealed important features of the innate immune response. Although sharing an increased susceptibility to bacterial and fungal infections, these disorders each have distinctive features in their clinical manifestations and characteristic microbial pathogens. This review provides an update on several genetic disorders with impaired neutrophil function, their pathogenesis, and treatment strategies. These include chronic granulomatous disease, which results from inactivating mutations in the superoxide-generating nicotinamide dinucleotide phosphate oxidase...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27905994/the-role-of-il-10-in-mycobacterium-avium-subsp-paratuberculosis-infection
#2
REVIEW
Tariq Hussain, Syed Zahid Ali Shah, Deming Zhao, Srinand Sreevatsan, Xiangmei Zhou
Mycobacterium avium subsp. paratuberculosis (MAP) is an intracellular pathogen and is the causative agent of Johne's disease of domestic and wild ruminants. Johne's disease is characterized by chronic granulomatous enteritis leading to substantial economic losses to the livestock sector across the world. MAP persistently survives in phagocytic cells, most commonly in macrophages by disrupting its early antibacterial activity. MAP triggers several signaling pathways after attachment to pathogen recognition receptors (PRRs) of phagocytic cells...
December 1, 2016: Cell Communication and Signaling: CCS
https://www.readbyqxmd.com/read/27905281/identification-of-acanthamoeba-genotypes-in-pools-and-stagnant-water-in-ponds-in-sistan-region-in-southeast-iran
#3
Ali Aghajani, Mansour Dabirzadeh, Yahya Maroufi, Hossein Hooshyar
OBJECTIVE: Acanthamoeba is one of the most abundant free-living amoebas that is widely distributed in natural and artificial environment resources. Acanthamoeba pathogenic genotypes cause chronic human diseases including amoebic keratitis and granulomatous amoebic encephalitis. The aim of this study was to determine and identify Acanthamoeba genotypes residing in pools and stagnant water in ponds in Sistan region in southeast Iran. This descriptive study was conducted at the Parasitology Laboratory, School of Medicine, Zabol University of Medical Sciences...
September 2016: Türkiye Parazitolojii Dergisi
https://www.readbyqxmd.com/read/27904945/-sarcoidosis-and-uveitis-an-update
#4
REVIEW
J G Garweg
Ocular involvement in sarcoidosis is present in up to one third of patients and is frequently manifested before the underlying systemic disease has been diagnosed. With a view to the therapeutic consequences an early diagnosis of the underlying disease is advantageous. In cases of visual loss early diagnostic measures include invasive procedures, such as ultrasound-guided bronchoalveolar lavage and transbronchial biopsy, if nodular conjunctival or cutaneous manifestations which would confirm the diagnosis are not present...
November 30, 2016: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
https://www.readbyqxmd.com/read/27904195/neutrophilic-eccrine-hidradenitis-in-a-child-with-chronic-granulomatous-disease
#5
Vibhu Mendiratta, Sarita Sanke, Ram Chander
No abstract text is available yet for this article.
November 2016: Indian Journal of Dermatology
https://www.readbyqxmd.com/read/27896060/diffuse-bronchiectasis-as-the-primary-manifestation-of-endobronchial-sarcoidosis
#6
Paul D Hiles, Kenneth R Kemp, Jean M Coviello
Sarcoidosis is an idiopathic disease that most commonly involves the lungs and is characterized by granulomatous inflammation. Bronchiectasis is one pulmonary manifestation of sarcoidosis, although it is almost always observed as traction bronchiectasis in the setting of fibrotic lung disease. A 50-year-old woman was evaluated for chronic cough and bronchiectasis with a small amount of peripheral upper lobe honeycombing and no significant pulmonary fibrosis or lymphadenopathy. After an extensive laboratory and imaging evaluation did not identify a cause of her bronchiectasis, bronchoscopy was performed to assess for primary ciliary dyskinesia and revealed a diffuse cobblestone appearance of the airway mucosa...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/27891014/chronic-granulomatous-disease-may-be-an-underlying-cause-for-invasive-nocardiosis
#7
Sujoy Khan, Parthasarathi Bhattacharya
No abstract text is available yet for this article.
November 2016: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/27890651/biofilm-is-associated-with-chronic-streptococcal-meningoencephalitis-in-fish
#8
Abdulsalam Isiaku, Sabri M Yusoff, Ina-Salwany M Yasin, Hassan M Daud, Tanko N Polycarp, Muhammad B Bello
Biofilms are aggregates of attached microbial organisms whose existence on tissues is often recognised as a mechanism for the establishment of most chronic diseases. Herein we investigated the ability of piscine Streptococcus agalactiae, an important aquatic pathogen, for adaptation to this sessile lifestyle in vitro and in the brain of a tilapia fish model. Piscine S. agalactiae exhibited a weak attachment to polystyrene plates and expressed a low biofilm phenotype under the study conditions. Furthermore, fluorescent in situ hybridization and confocal laser scanning microscopy revealed discrete aggregates of attached S...
November 24, 2016: Microbial Pathogenesis
https://www.readbyqxmd.com/read/27889463/noncanonical-fungal-autophagy-inhibits-inflammation-in-response-to-ifn-%C3%AE-via-dapk1
#9
Vasilis Oikonomou, Silvia Moretti, Giorgia Renga, Claudia Galosi, Monica Borghi, Marilena Pariano, Matteo Puccetti, Carlo A Palmerini, Lucia Amico, Alessandra Carotti, Lucia Prezioso, Angelica Spolzino, Andrea Finocchi, Paolo Rossi, Andrea Velardi, Franco Aversa, Valerio Napolioni, Luigina Romani
Defects in a form of noncanonical autophagy, known as LC3-associated phagocytosis (LAP), lead to increased inflammatory pathology during fungal infection. Although LAP contributes to fungal degradation, the molecular mechanisms underlying LAP-mediated modulation of inflammation are unknown. We describe a mechanism by which inflammation is regulated during LAP through the death-associated protein kinase 1 (DAPK1). The ATF6/C/EBP-β/DAPK1 axis activated by IFN-γ not only mediates LAP to Aspergillus fumigatus but also concomitantly inhibits Nod-like receptor protein 3 (NLRP3) activation and restrains pathogenic inflammation...
November 16, 2016: Cell Host & Microbe
https://www.readbyqxmd.com/read/27889190/diagnosis-of-multiple-sclerosis-progress-and-challenges
#10
REVIEW
Wallace J Brownlee, Todd A Hardy, Franz Fazekas, David H Miller
The diagnosis of multiple sclerosis is based on neurological symptoms and signs, alongside evidence of dissemination of CNS lesions in space and time. MRI is often sufficient to confirm the diagnosis when characteristic lesions accompany a typical clinical syndrome, but in some patients, further supportive information is obtained from cerebrospinal fluid examination and neurophysiological testing. Differentiation is important from other diseases in which demyelination is a feature (eg, neuromyelitis optica spectrum disorder and acute disseminated encephalomyelitis) and from non-demyelinating disorders such as chronic small vessel disease and other inflammatory, granulomatous, infective, metabolic, and genetic causes that can mimic multiple sclerosis...
November 23, 2016: Lancet
https://www.readbyqxmd.com/read/27888674/qpcr-detection-of-mycobacterium-leprae-in-biopsies-and-slit-skin-smear-of-different-leprosy-clinical-forms
#11
Michelle de Campos Soriani Azevedo, Natália Mortari Ramuno, Luciana Raquel Vincenzi Fachin, Mônica Tassa, Patrícia Sammarco Rosa, Andrea de Faria Fernandes Belone, Suzana Madeira Diório, Cleverson Teixeira Soares, Gustavo Pompermaier Garlet, Ana Paula Favaro Trombone
Leprosy, whose etiological agent is Mycobacterium leprae, is a chronic infectious disease that mainly affects the skin and peripheral nervous system. The diagnosis of leprosy is based on clinical evaluation, whereas histopathological analysis and bacilloscopy are complementary diagnostic tools. Quantitative PCR (qPCR), a current useful tool for diagnosis of infectious diseases, has been used to detect several pathogens including Mycobacterium leprae. The validation of this technique in a robust set of samples comprising the different clinical forms of leprosy is still necessary...
November 23, 2016: Brazilian Journal of Infectious Diseases
https://www.readbyqxmd.com/read/27885760/haploidentical-hematopoietic-stem-cell-transplantation-for-a-case-with-x-linked-chronic-granulomatous-disease
#12
Ling Zhou, Lu-Jia Dong, Zhi-Yong Gao, Xin-Jian Yu, Dao-Pei Lu
CGD is a rare primary immunodeficiency with high mortality rates when treated conventionally, especially for the X-chromosome-linked form. HSCT is the only curative therapy for CGD; however, haploidentical transplantation in CGD is rare. Here, we report a case of X-linked CGD treated successfully by haploidentical HSCT. The patient showed a positive result with full donor chimerism, good quality of life, and the absence of recurrent infectious diseases at follow-up (68 months). Thus, haploidentical HSCT may serve as an acceptable treatment approach for patients who have CGD, but no HLA-matched related or unrelated donor...
November 25, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27884839/morillo-gutierrez-b-beier-r-rao-k-et-al-treosulfan-based-conditioning-for-allogeneic-hsct-in-children-with-chronic-granulomatous-disease-a-multicenter-experience-blood-2016-128-3-440-448
#13
https://www.readbyqxmd.com/read/27873163/an-update-on-the-use-of-immunomodulators-in-primary-immunodeficiencies
#14
REVIEW
Pandiarajan Vignesh, Amit Rawat, Surjit Singh
The genomic revolution in the past decade fuelled by breathtaking advances in sequencing technologies has defined several new genetic diseases of the immune system. Many of these newly characterized diseases are a result of defects in genes involved in immune regulation. The discovery of these diseases has opened a vista of new therapeutic possibilities. Immunomodulatory agents, a hitherto unexplored therapeutic option in primary immunodeficiency diseases have been tried in a host of these newly described maladies...
November 21, 2016: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/27872772/an-uncommon-feature-of-chronic-granulomatous-disease-in-a-neonate
#15
Razieh Afrough, Sayyed Shahabeddin Mohseni, Setareh Sagheb
Chronic Granulomatous Disease (CGD) represents recurrent life-threatening bacterial and fungal infections and granuloma formation with a high mortality rate. CGD's sign and symptoms usually appear in infancy and children before the age of five; therefore, its presentation in neonatal period with some uncommon features may be easily overlooked. Here we describe a case of CGD in a 24-day-old boy, presenting with a diffuse purulent vesiculopustular rash and multiple osteomyelitis.
2016: Case Reports in Infectious Diseases
https://www.readbyqxmd.com/read/27867673/central-retinal-artery-occlusion-in-takayasu-s-arteritis-as-the-first-presentation-of-the-disease
#16
Hande Guclu, Vuslat Pelitli Gurlu, Sadık Altan Ozal, Orkut Guclu
Takayasu's arteritis (TA) is a chronic inflammatory granulomatous vasculitis which affects large and medium arterial vessels. The disease involves especially subclavian arteries and aortic branches but it can consist of any arteries. The major pathology is granulomatous panarteritis with intima proliferation and defects of the elastic lamina of the vessels. We present a case of central retinal artery occlusion in TA as the first presentation of the disease. To the best of our knowledge, the present case is the first case that demonstrates central retinal artery occlusion as an initial manifestation in TA...
2016: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/27865245/adolescent-with-recurrent-tuberculosis-can-it-be-chronic-granulomatous-disease
#17
Yashashree Gupta, Ira Shah
Chronic granulomatous disease (CGD) is an inherited primary immunodeficiency disorder with recurrent bacterial and fungal infections like Staphylococcus aureus, Nocardia spp, Serratia marcescens, Burkholderia cepacia, Salmonella spp. and Aspergillus species. We present a 13-year-old male child who had 3 episodes of tuberculosis (TB) at 5 years, 8 years and 13 years of age, respectively, with no other intercurrent infections and who was diagnosed as CGD at the age of 13 years. This case highlights the possibility of phenotypic variations of CGD...
July 2016: Indian Journal of Tuberculosis
https://www.readbyqxmd.com/read/27861181/genetic-risk-for-inflammatory-bowel-disease-is-a-determinant-of-crohn-s-disease-development-in-chronic-granulomatous-disease
#18
Chengrui Huang, Suk See De Ravin, Adam R Paul, Theo Heller, Nancy Ho, Lisa Wu Datta, Christa S Zerbe, Beatriz E Marciano, Douglas B Kuhns, Howard A Kader, Steven M Holland, Harry L Malech, Steven R Brant
BACKGROUND: Approximately, one-third to one-half of children with chronic granulomatous disease (CGD) develop gastrointestinal inflammation characteristic of idiopathic inflammatory bowel disease (IBD), usually Crohn's disease. We hypothesized that the overall IBD genetic risk, determined by IBD genetic risk score (GRS), might in part determine IBD development in CGD. METHODS: We reviewed medical records to establish IBD diagnoses in CGD subjects seen at NIAID. IBD risk single nucleotide polymorphism genotypes were determined using the Immunochip, and GRS were estimated by Mangrove...
December 2016: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/27856522/chromoblastomycosis
#19
REVIEW
Flavio Queiroz-Telles, Sybren de Hoog, Daniel Wagner C L Santos, Claudio Guedes Salgado, Vania Aparecida Vicente, Alexandro Bonifaz, Emmanuel Roilides, Liyan Xi, Conceição de Maria Pedrozo E Silva Azevedo, Moises Batista da Silva, Zoe Dorothea Pana, Arnaldo Lopes Colombo, Thomas J Walsh
Chromoblastomycosis (CBM), also known as chromomycosis, is one of the most prevalent implantation fungal infections, being the most common of the gamut of mycoses caused by melanized or brown-pigmented fungi. CBM is mainly a tropical or subtropical disease that may affect individuals with certain risk factors around the world. The following characteristics are associated with this disease: (i) traumatic inoculation by implantation from an environmental source, leading to an initial cutaneous lesion at the inoculation site; (ii) chronic and progressive cutaneous and subcutaneous tissular involvement associated with fibrotic and granulomatous reactions associated with microabscesses and often with tissue proliferation; (iii) a nonprotective T helper type 2 (Th2) immune response with ineffective humoral involvement; and (iv) the presence of muriform (sclerotic) cells embedded in the affected tissue...
January 2017: Clinical Microbiology Reviews
https://www.readbyqxmd.com/read/27853979/erratum-to-clinical-and-molecular-features-of-38-children-with-chronic-granulomatous-disease-in-mainland-china
#20
Huan Xu, Wen Tian, Shu-Juan Li, Lu-Ying Zhang, Wei Liu, Yao Zhao, Zhi-Yong Zhang, Xue-Mei Tang, Mo Wang, Dao-Qi Wu, Ji-Sheng Shi, Yuan Ding, Xiao-Dong Zhao, Xi-Qiang Yang, Li-Ping Jiang
No abstract text is available yet for this article.
November 16, 2016: Journal of Clinical Immunology
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