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Chronic granulomatous disease

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https://www.readbyqxmd.com/read/28634011/immunomodulatory-properties-of-concholepas-concholepas-hemocyanin-against-francisellosis-in-a-zebrafish-model
#1
Leidy Lagos, Julia I Tandberg, María Inés Becker, Hanne C Winther-Larsen
The development of vaccines for aquaculture has been an important milestone in providing a continuous and sustainable production. Most of the vaccines currently on the market for aquaculture include oil as adjuvant. Nevertheless, several studies reported an occurrence of side effects after their use in farmed fish. As a result, there is a need for new and improved adjuvants that can stimulate the immune system while causing as few side-effects as possible. Hemocyanins are versatile macromolecules with strong immunogenic and immunomodulatory properties...
June 17, 2017: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/28626633/mediastinal-hemangioendothelioma-case-report-and-review-of-the-literature
#2
Davide Patrini, Laura Scolamiero, Reena Khiroya, David Lawrence, Elaine Borg, Martin Hayward, Nikolaos Panagiotopoulos
BACKGROUND: Epithelioid haemangioendothelioma (EHE) is a rare low-grade vascular neoplasm that can arise in the lung, liver, soft tissues or, less commonly, bone. Due to its low prevalence of less than one in a million and its non-specific clinical features, EHE is often misdiagnosed and managed inappropriately. Here we discuss the case of a 58 year-old gentleman with mediastinal EHE and review existing literature on pulmonary EHE (PEH). CASE HISTORY: A 58 year-old gentleman presented to our outpatient Clinic with chest discomfort and palpitations...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28625882/analysis-of-sarcoidosis-in-the-oporto-region-portugal
#3
A V Cardoso, P C Mota, N Melo, S Guimarães, C Souto Moura, J M Jesus, R Cunha, A Morais
BACKGROUND: Sarcoidosis is a systemic granulomatous disease of unknown etiology. Epidemiological studies of different populations are essential because clinical presentation, organ involvement, disease severity, and prognosis vary significantly according to region and population. The aim of this study was to assess epidemiological and clinical characteristics, staging factors, and clinical course in patients with sarcoidosis from a tertiary hospital in Oporto, Portugal. METHODS: A retrospective analysis of patients with sarcoidosis and at least 2 years of follow-up evaluated at the Centro Hospitalar de São João between 2000 and 2014...
June 15, 2017: Revista Portuguesa de Pneumologia
https://www.readbyqxmd.com/read/28621249/tuberculosis-masked-by-immunodeficiency-a-review-of-two-cases-diagnosed-with-chronic-granulomatous-disease
#4
Hikmet Tekin Nacaroğlu, Semiha Bahçeci Erdem, Nesrin Gülez, Canan Şule Ünsal Karkıner, İlker Devrim, Ferah Genel, Mustafa Yavuz Köker, Demet Can
Chronic granulomatous disease (CGD) is a genetically heterogeneous primary immunodeficiency that is characterized by recurrent and life-threatening infections resulting from defects in phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase system and granuloma formation due to increased inflammatory response. The most commonly involved organs are the lungs, skin, lymph nodes, and liver due to infection. It may present with recurrent pneumonia, hilar lymphadenopathy, empyema, abscess, reticulonodular patterns, and granulomas due to lung involvement...
March 2017: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/28611513/brain-changes-detected-by-functional-magnetic-resonance-imaging-and-spectroscopy-in-patients-with-crohn-s-disease
#5
REVIEW
Kun Lv, Yi-Hong Fan, Li Xu, Mao-Sheng Xu
Crohn's disease (CD) is a chronic, non-specific granulomatous inflammatory disorder that commonly affects the small intestine and is a phenotype of inflammatory bowel disease (IBD). CD is prone to relapse, and its incidence displays a persistent increase in developing countries. However, the pathogenesis of CD is poorly understood, with some studies emphasizing the link between CD and the intestinal microbiota. Specifically, studies point to the brain-gut-enteric microbiota axis as a key player in the occurrence and development of CD...
May 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28597826/pathology-of-tuberculosis-how-the-pathology-of-human-tuberculosis-informs-and-directs-animal-models
#6
Randall J Basaraba, Robert L Hunter
Tuberculosis (TB) is a chronic inflammatory disease caused by the pathogenic bacterium Mycobacterium tuberculosis. A wide variety of host- and pathogen-associated variables influence the clinical manifestation of TB in different individuals within the human population. As a consequence, the characteristic granulomatous lesions that develop within the lung are heterogeneous in size and cellular composition. Due to the lack of appropriate tissues from human TB patients, a variety of animal models are used as surrogates to study the basic pathogenesis and to test experimental vaccines and new drug therapies...
June 2017: Microbiology Spectrum
https://www.readbyqxmd.com/read/28592787/rare-gastric-lesions-associated-with-helicobacter-pylori-infection-a-histopathological-review
#7
Mee Joo
Helicobacter pylori infection is associated with chronic gastritis, peptic ulcer disease, gastric adenocarcinoma, and mucosa-associated lymphoid tissue lymphoma. However, some rare gastric lesions exhibiting distinctive histological features may also be associated with H. pylori infection, including lymphocytic gastritis, granulomatous gastritis, Russell body gastritis, or crystal-storing histiocytosis. Although diverse factors can contribute to their development, there is convincing evidence that H. pylori infection may play a pathogenic role...
June 5, 2017: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/28591264/sarcoid-like-cutaneous-lesions-in-chronic-adult-paracoccidioidomycosis-report-of-two-cases
#8
Nurimar C Fernandes, Júlia Gomes Côrtes, Tiyomi Akitti, Danielle Carvalho Quintella, Tulia Cuzzi
This study reports two cases of chronic paracoccidioidomycosis with sarcoid-like cutaneous lesions. The patients began the treatment in 2013 at Hospital Universitário Clementino Fraga Filho (HUCFF) of the Universidade Federal do Rio de Janeiro (UFRJ). The first case (mild form) was treated with trimethoprim-sulfamethoxazole (8 mg /kg per day, orally) for three months and, then, with half the dose for nine months; the second (moderate form), with itraconazole (200 mg per day, orally) for 12 months. We point out the rareness of the sarcoid-like cutaneous lesions and the differential diagnoses for other granulomatous diseases...
June 1, 2017: Revista do Instituto de Medicina Tropical de São Paulo
https://www.readbyqxmd.com/read/28579215/-vogt-koyanagi-harada-disease
#9
REVIEW
C Bonnet, J-B Daudin, D Monnet, A Brézin
Vogt-Koyanagi-Harada (VKH) disease is defined as a severe bilateral, chronic granulomatous panuveitis associated with serous retinal detachments, disk edema, and vitritis, with central nervous system, auditory, and integumentary manifestations. It is an autoimmune inflammatory condition mediated by T cells that target melanocytes in individuals genetically susceptible to the disease. Vogt-Koyanagi-Harada disease presents clinically in 4 different phases: prodromal, acute inflammatory, chronic, and recurrent, with extraocular manifestations including headache, meningitis, hearing loss, poliosis, and vitiligo...
May 31, 2017: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/28577521/seven-chronic-granulomatous-disease-cases-in-a-single-center-experience-and-a-review-of-the-literature
#10
Şeyhan Kutluğ, Gülnar Şensoy, Asuman Birinci, Berkay Saraymen, Mustafa Yavuz Köker, Alişan YΙldΙran
BACKGROUND: Chronic granulomatous disease (CGD) is a rare primary immunodeficiency caused by defects in the nicotinamide adenine dinucleotide phosphate (NADPH) oxidase enzyme system. This disease causes the disordered functioning of phagocytic cells. It is characterized by life-threatening and/or recurrent infections by bacteria and fungi. CGD has one X-linked recessive subtype (X-CGD) and four autosomal recessive subtypes (AR-CGD), and the differential diagnosis is important in such chronic inflammatory disorders...
June 1, 2017: Asian Pacific Journal of Allergy and Immunology
https://www.readbyqxmd.com/read/28577054/-what-is-a-granuloma
#11
REVIEW
E von Stebut
The histogenesis of granulomata is dependent on various cell types. The typical composition is a center of macrophages/histiocytes with lymphocytes at the border. The sequence of events leading to granuloma formation is regulated by various cell types and cytokines: While Th1-associated mechanisms promote granuloma development, it appears that regulatory T cells as well as M2 macrophages together with interleukin (IL)-10 and IL-13 lead to their dissociation and tissue healing. There is a strong correlation between structure and function of granulomas...
June 2, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28575744/decreased-neural-precursor-cell-pool-in-nadph-oxidase-2-deficiency-from-mouse-brain-to-neural-differentiation-of-patient-derived-ipsc
#12
Zeynab Nayernia, Marilena Colaianna, Natalia Robledinos-Antón, Eveline Gutzwiller, Frédérique Sloan-Béna, Elisavet Stathaki, Yousef Hibaoui, Antonio Cuadrado, Jürgen Hescheler, Marie José Stasia, Tomo Saric, Vincent Jaquet, Karl-Heinz Krause
There is emerging evidence for the involvement of reactive oxygen species (ROS) in the regulation of stem cells and cellular differentiation. Absence of the ROS-generating NADPH oxidase NOX2 in chronic granulomatous disease (CGD) patients, predominantly manifests as immune deficiency, but has also been associated with decreased cognition. Here, we investigate the role of NOX enzymes in neuronal homeostasis in adult mouse brain and in neural cells derived from human induced pluripotent stem cells (iPSC). High levels of NOX2 were found in mouse adult neurogenic regions...
April 24, 2017: Redox Biology
https://www.readbyqxmd.com/read/28574339/diverse-stimuli-engage-different-neutrophil-extracellular-trap-pathways
#13
Elaine F Kenny, Alf Herzig, Renate Krüger, Aaron Muth, Santanu Mondal, Paul R Thompson, Volker Brinkmann, Horst Von Bernuth, Arturo Zychlinsky
Neutrophils release neutrophil extracellular traps (NETs) which ensnare pathogens and have pathogenic functions in diverse diseases. We examined the NETosis pathways induced by five stimuli; PMA, the calcium ionophore A23187, nigericin, Candida albicans and Group B Streptococcus. We studied NET production in neutrophils from healthy donors with inhibitors of molecules crucial to PMA induced NETs including protein kinase C, calcium, reactive oxygen species, the enzymes myeloperoxidase (MPO) and neutrophil elastase...
June 2, 2017: ELife
https://www.readbyqxmd.com/read/28573071/differential-diagnosis-in-ulcerative-colitis-in-an-adolescent-chronic-granulomatous-disease-needs-extra-attention
#14
Daniel Kotlarz, Odul Egritas Gurkan, Zehra Sule Haskologlu, Ozgur Ekinci, Aysel Aksu Unlusoy, Neslihan Gürcan Kaya, Jacek Puchalka, Cristoph Klein, Buket Dalgic
Chronic granulomatous disease (CGD) is a primary immune deficiency that is commonly diagnosed under the age of 5 years (95%) and is rarely seen in adulthood. CGD may manifest as inflammatory bowel disease (IBD) in childhood. Without proper diagnosis, these patients may be monitored for years as IBD; some may even be regarded as steroid-resistant ulcerative colitis (UC) and end up having a colectomy. In this case report, we described a patient who had been followed-up for years as UC and subsequently underwent colectomy, but was finally diagnosed in adulthood as primary immune deficiency...
May 15, 2017: World Journal of Gastrointestinal Pathophysiology
https://www.readbyqxmd.com/read/28571290/leprosy-in-an-eight-year-old-child-an-exceptional-case-with-unusual-oral-manifestation
#15
Mayuri Jain
Leprosy is a contagious and granulomatous disease which is caused by Mycobacterium leprae. The incubation period for leprosy is five to seven years and it can take as long as about 30 years before signs and symptoms of leprosy develop in some patients. It is a chronic systemic disease which mostly affects the skin and peripheral nerves. It has been seen that indeterminate leprosy is the most common type seen in childhood followed by tuberculoid variant. Borderline lepromatous and lepromatous leprosy are only occasionally encountered in children and rarely affects the oral cavity...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28567068/serum-enolase-2-high-sensitivity-c-reactive-protein-and-serum-cholesterol-in-smear-positive-drug-na%C3%A3-ve-pulmonary-tuberculosis
#16
Suhail Ahmed Almani, Tariq Zaffar Shaikh, Haji Khan Khoharo, Ikramuddin Ujjan
BACKGROUND: Pulmonary tuberculosis (PTB) is a chronic granulomatous disease caused by Mycobacterium tuberculosis. The present study determined the serum human enolase-2 (ENO-2), high-sensitive C-reactive protein (hs-CRP), and serum cholesterol levels as biological marker of disease activity and treatment response in smear-positive drug-naïve PTB. MATERIALS AND METHODS: This case-control study was done in the Department of Medicine, Liaquat University of Medical and Health Sciences (LUMHS), Jamshoro/Hyderabad, Sindh, from January 2015 to April 2016...
2017: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/28562770/paracoccidioidomycosis-with-sarcoid-like-lesions-a-diagnostic-challenge
#17
Marilda Aparecida Milanez Morgado de Abreu, Joel Carlos Lastória, Antonio Luiz de Arruda Mattos, Maurício Mota de Avelar Alchorne
Clinical presentation of paracoccidioidomycosis (PCM) can be diverse. Morphology and quantity of skin lesions depends on interactions between host immunity and fungus virulence. Diagnosis can be a challenge considering that this fungus has low virulence and some individuals have immunity to microorganism, which results in well-marked granulomas without visible microorganisms. We report herein a clinical presentation of sarcoid-like PCM, initially diagnosed as tuberculoid leprosy. This rare type of PCM is often mistaken for other types of chronic granulomatous diseases...
March 2017: Revista da Sociedade Brasileira de Medicina Tropical
https://www.readbyqxmd.com/read/28560517/a-contemporary-analysis-of-clinical-and-demographic-factors-of-chronic-rhinosinusitis-patients-and-their-association-with-disease-severity
#18
L P Hoehle, K M Phillips, D S Caradonna, S T Gray, A R Sedaghat
BACKGROUND: Chronic rhinosinusitis (CRS) is highly prevalent, significantly decreases quality of life and leads to tremendous health care costs every year. No recent study has characterised the prevalence of potentially CRS-modifying patient characteristics and simultaneously shown their impact on CRS severity. AIMS: We sought to determine the prevalence of potential clinical and demographic CRS-modifying characteristics and their associations with CRS symptom severity in a large contemporary cohort of CRS patients...
May 30, 2017: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/28558308/age-predicts-cytokine-kinetics-and-innate-immune-cell-activation-following-intranasal-delivery-of-ifn%C3%AE-and-gm-csf-in-a-mouse-model-of-rsv-infection
#19
Katherine M Eichinger, Erin Resetar, Jacob Orend, Kacey Anderson, Kerry M Empey
Respiratory syncytial virus (RSV) is the leading cause of lower respiratory tract infections in young children and is further associated with increased healthcare utilization and cost of care in the first years of life. Severe RSV disease during infancy has also been linked to the later development of allergic asthma, yet there remains no licensed RSV vaccine or effective treatment. Pre-clinical and clinical studies have shown that disease severity and development of allergic asthma are associated with differences in cytokine production...
May 27, 2017: Cytokine
https://www.readbyqxmd.com/read/28553289/major-cd4-t-cell-depletion-and-immune-senescence-in-a-patient-with-chronic-granulomatous-disease
#20
Adriana S Albuquerque, Susana M Fernandes, Rita Tendeiro, Rémi Cheynier, Margarida Lucas, Susana L Silva, Rui M M Victorino, Ana E Sousa
Chronic granulomatous disease (CGD) results from primary defects in phagocytic reactive oxygen species (ROS) production. T-cell evaluation is usually neglected during patients' follow-up, although T-cell depletion has been reported in CGD through unknown mechanisms. We describe here a 36-year-old patient with X-linked CGD with severe CD4 T-cell depletion <200 CD4 T-cells/μl, providing insights into the mechanisms that underlie T-cell loss in the context of oxidative burst defects. In addition to the typical infections, the patient featured a progressive T-cell loss associated with persistent lymphocyte activation, expansion of interleukin (IL)-17-producing CD4 T-cells, and impaired thymic activity, leading to a reduced replenishment of the T-cell pool...
2017: Frontiers in Immunology
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