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Primary immune deficiency

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https://www.readbyqxmd.com/read/28345437/clinical-profile-dosing-and-quality-of-life-outcomes-in-primary-immune-deficiency-patients-treated-at-home-with-immunoglobulin-g-data-from-the-ideal-patient-registry
#1
Sean Kearns, Loretta Kristofek, William Bolgar, Luqman Seidu, Samantha Kile
BACKGROUND: Patients with primary immune deficiency (PID) often require immunoglobulin G (IgG, commonly referred to as Ig) replacement therapy to prevent infections and associated comorbidities. Ig therapy can be given either through intravenous or subcutaneous routes, and both can be done in the home setting. There is limited information available on the real-world diagnosis, management, and outcomes of this patient population, given the variable disease presentation and treatment options...
April 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/28344881/immunosurveillance-and-immunoediting-in-mmtv-pymt-induced-mammary-oncogenesis
#2
Emilie T E Gross, Semi Han, Prasantha Vemu, Carlos D Peinado, Martin Marsala, Lesley G Ellies, Jack D Bui
Evidence of cancer immunosurveillance and immunoediting processes has been primarily demonstrated in mouse models of chemically induced oncogenesis. Although these models are very tractable, they are characterized by high mutational loads that represent a minority of human cancers. In this study, we sought to determine whether cancer immunosurveillance and immunoediting could be demonstrated in a more clinically relevant oncogene-induced model of carcinogenesis, the MMTV-PyMT (PyMT) mammary carcinoma model...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/28344878/targeting-cytokine-signaling-checkpoint-cis-activates-nk-cells-to-protect-from-tumor-initiation-and-metastasis
#3
Eva M Putz, Camille Guillerey, Kevin Kos, Kimberley Stannard, Kim Miles, Rebecca B Delconte, Kazuyoshi Takeda, Sandra E Nicholson, Nicholas D Huntington, Mark J Smyth
The cytokine-induced SH2-containing protein CIS belongs to the suppressor of cytokine signaling (SOCS) protein family. Here, we show the critical role of CIS in suppressing natural killer (NK) cell control of tumor initiation and metastasis. Cish-deficient mice were highly resistant to methylcholanthrene-induced sarcoma formation and protected from lung metastasis of B16F10 melanoma and RM-1 prostate carcinoma cells. In contrast, the growth of primary subcutaneous tumors, including those expressing the foreign antigen OVA, was unchanged in Cish-deficient mice...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/28344876/2b4-sap-signaling-is-required-for-the-priming-of-naive-cd8-t-cells-by-antigen-expressing-b-cells-and-b-lymphoma-cells
#4
Yu-Hsuan Huang, Kevin Tsai, Sara Y Tan, Sohyeong Kang, Mandy L Ford, Kenneth W Harder, John J Priatel
Mutations in SH2D1A gene that encodes SAP (SLAM-associated protein) result in X-linked lymphoproliferative disease (XLP), a rare primary immunodeficiency disease defined by exquisite sensitivity to the B-lymphotropic Epstein-Barr virus (EBV) and B cell lymphomas. However, the precise mechanism of how the loss of SAP function contributes to extreme vulnerability to EBV and the development of B cell lymphomas remains unclear. Here, we investigate the hypothesis that SAP is critical for CD8(+) T cell immune surveillance of antigen (Ag)-expressing B cells or B lymphoma cells under conditions of defined T cell receptor (TCR) signaling...
2017: Oncoimmunology
https://www.readbyqxmd.com/read/28341440/optimization-and-testing-of-dried-antibody-tube-the-euroflow-lst-and-pidot-tubes-as-examples
#5
V H J van der Velden, J Flores-Montero, Martin Perez, Marta Martin-Ayuso, Oliver Crespo, Elena Blanco, Tomas Kalina, Jan Philippé, Carolien Bonroy, Maaike de Bie, Jeroen Te Marvelde, Cristina Teodosio, Alba Corral Mateos, Veronika Kanderová, Mirjam van der Burg, Dennis Van Hoof, Jacques J M van Dongen, Alberto Orfao
Within EuroFlow, we recently developed screening tubes for hematological malignancies and immune deficiencies. Pipetting of antibodies for such 8-color 12-marker tubes however is time-consuming and prone to operational mistakes. We therefore evaluated dried formats of the lymphocytosis screening tube (LST) and of the primary immune deficiency orientation tube (PIDOT). Both tubes were evaluated on normal and/or on patient samples, comparing the mean fluorescence intensity of specific lymphocyte populations. Our data show that the dried tubes and liquid counterparts give highly comparable staining results, particularly when analyzed in multidimensional plots...
March 21, 2017: Journal of Immunological Methods
https://www.readbyqxmd.com/read/28335993/biomaterials-innovation-for-next-generation-ex%C3%A2-vivo-immune-tissue-engineering
#6
REVIEW
Ankur Singh
Primary and secondary lymphoid organs are tissues that facilitate differentiation of B and T cells, leading to the induction of adaptive immune responses. These organs are present in the body from birth and are also recognized as locations where self-reactive B and T cells can be eliminated during the natural selection process. Many insights into the mechanisms that control the process of immune cell development and maturation in response to infection come from the analysis of various gene-deficient mice that lack some or all hallmark features of lymphoid tissues...
March 15, 2017: Biomaterials
https://www.readbyqxmd.com/read/28335444/hiv-education-and-welfare-services-in-primary-care-an-empirical-model-of-integration-in-brazil-s-unified-health-system
#7
Rahbel Rahman, Rogério M Pinto, Melanie M Wall
Integration of health education and welfare services in primary care systems is a key strategy to solve the multiple determinants of chronic diseases, such as Human Immunodeficiency Virus Infection and Acquired Immune Deficiency Syndrome (HIV/AIDS). However, there is a scarcity of conceptual models from which to build integration strategies. We provide a model based on cross-sectional data from 168 Community Health Agents, 62 nurses, and 32 physicians in two municipalities in Brazil's Unified Health System (UHS)...
March 14, 2017: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/28325756/a-novel-role-for-myeloid-cell-specific-neuropilin-1-in-mitigating-sepsis
#8
Xiaoyan Dai, Imoh Okon, Zhaoyu Liu, Yue Wu, Huaiping Zhu, Ping Song, Ming-Hui Zou
Sepsis-typically caused by an uncontrolled and amplified host systemic inflammatory response to microbial infection-is a life-threatening complex clinical disorder and remains a major cause of infection-related deaths in the intensive care unit. Emerging evidence suggests that neuropilin 1 (Nrp1), an originally defined coreceptor for class 3 semaphorins and VEGF, plays important roles in the immune system; however, the function and regulation of macrophage Nrp1 in host immune defense against bacterial infection remain unknown...
March 21, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28303490/immune-checkpoint-inhibitors-in-gliomas
#9
REVIEW
Aaron C Tan, Amy B Heimberger, Mustafa Khasraw
PURPOSE OF REVIEW: Malignant gliomas result in disproportionately high morbidity and mortality compared with other primary tumors, and progression of disease is inevitable. Novel therapies to improve outcomes are needed and immune checkpoint inhibitors hold significant promise. RECENT FINDINGS: A limited body of preclinical evidence suggests that checkpoint inhibitors may be effective treatment for gliomas. Biomarkers to identify characteristics of gliomas responsive to these therapies will be essential...
April 2017: Current Oncology Reports
https://www.readbyqxmd.com/read/28302135/myeloid-c-ebp%C3%AE-deficiency-reshapes-microglial-gene-expression-and-is-protective-in-experimental-autoimmune-encephalomyelitis
#10
Marta Pulido-Salgado, Jose M Vidal-Taboada, Gerardo Garcia Diaz-Barriga, Joan Serratosa, Tony Valente, Paola Castillo, Jonathan Matalonga, Marco Straccia, Josep M Canals, Annabel Valledor, Carme Solà, Josep Saura
BACKGROUND: CCAAT/enhancer binding protein β (C/EBPβ) is a transcription factor that regulates the expression of important pro-inflammatory genes in microglia. Mice deficient for C/EBPβ show protection against excitotoxic and ischemic CNS damage, but the involvement in this neuroprotective effect of the various C/EBPβ-expressing cell types is not solved. Since C/EBPβ-deficient microglia show attenuated neurotoxicity in culture, we hypothesized that specific C/EBPβ deficiency in microglia could be neuroprotective in vivo...
March 16, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28301594/daxx-plays-a-novel-role-in-t-cell-survival-but-is-dispensable-in-fas-induced-apoptosis
#11
Jinghe Li, Liangyue Qian, John P Dowling, Christine Curcione, Drishya Kurup, Jianke Zhang
Daxx was originally isolated as a Fas-binding protein. However, the in vivo function of Daxx in Fas-induced apoptosis has remained enigmatic. Fas plays an important role in homeostasis in the immune system. Fas gene mutations lead to autoimmune-lymphoproliferation (lpr) diseases characterized by hyperplasia of secondary lymphoid organs. It is well established that the FADD adaptor binds to Fas, and recruits/activates caspase 8. However, additional proteins including Daxx have also been indicated to associate with Fas...
2017: PloS One
https://www.readbyqxmd.com/read/28295235/cutaneous-manifestations-of-subcutaneous-and-systemic-fungal-infections-in-tropical-regions-a-retrospective-study-from-a-referral-center-in-southern-taiwan
#12
Wen-Chien Tsai, Chih-Hung Lee, Wei-Min Wu, Shang-Hung Lin, Yi-Chien Yang, Yu-Wen Cheng, Ji-Chen Ho, Kwei-Lan Liu
BACKGROUND: Deep cutaneous fungal infections, including subcutaneous mycoses and systemic fungal infection with cutaneous involvement, cause significant morbidity and mortality in light of increasing immunocompromised patients and global warming. Although a few studies reviewed deep fungal infections in temperate regions, a relevant study in tropical regions is lacking. We evaluated features of deep cutaneous fungal infections in southern Taiwan among the tropical regions. METHODS: We retrospectively reviewed all histopathological specimens with deep cutaneous fungal infections in a single referral center from 2001 to 2014 and successfully identified 23 cases...
March 13, 2017: International Journal of Dermatology
https://www.readbyqxmd.com/read/28293726/inflammation-in-iga-nephropathy
#13
Thomas Rauen, Jürgen Floege
Immunoglobulin A nephropathy (IgAN) is the most frequently occurring primary glomerulonephritis in Caucasian and Asian populations. Nonetheless, therapeutic recommendations are based on weak evidence, large controlled trials are scarce and, in particular, the additional value of immunosuppression beyond comprehensive supportive measures is not well-established. The use of immunosuppressants is supported by experimental insights into IgAN pathogenesis that suggest an autoimmune component in disease development...
March 14, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28284485/abnormality-of-regulatory-t-cells-in-common-variable-immunodeficiency
#14
REVIEW
Gholamreza Azizi, Nasim Hafezi, Hamed Mohammadi, Reza Yazdai, Tina Alinia, Marzieh Tavakol, Asghar Aghamohammadi, Abbas Mirshafiey
Common variable immunodeficiency (CVID) is a heterogeneous group of primary antibody deficiencies (PAD) which is defined by recurrent infections, hypogammaglobulinemia and defects in B-cell differentiation into plasma cells and memory B cells. T cell abnormalities have also been described in CVID patients. Several studies reported that Treg frequencies and their functional characteristics are disturbed and might account for the aberrant immune responses observed in CVID patients. The aim of this review is to describe phenotypic and functional characteristics of Treg cells, and to review the literature with respect to the reported Treg defects and its association with the clinical manifestation in CVID...
December 29, 2016: Cellular Immunology
https://www.readbyqxmd.com/read/28283223/interferon-regulatory-factor-signaling-in-autoimmune-disease
#15
REVIEW
Bharati Matta, Su Song, Dan Li, Betsy J Barnes
Interferon regulatory factors (IRFs) play critical roles in pathogen-induced innate immune responses and the subsequent induction of adaptive immune response. Dysregulation of IRF signaling is therefore thought to contribute to autoimmune disease pathogenesis. Indeed, numerous murine in vivo studies have documented protection from or enhanced susceptibility to particular autoimmune diseases in Irf-deficient mice. What has been lacking, however, is replication of these in vivo observations in primary immune cells from patients with autoimmune disease...
March 7, 2017: Cytokine
https://www.readbyqxmd.com/read/28280951/effects-of-dietary-n-3-fatty-acids-on-toll-like-receptor-activation-in-primary-leucocytes-from-atlantic-salmon-salmo-salar
#16
Marianne Arnemo, Arturas Kavaliauskis, Adriana Magalhaes Santos Andresen, Marta Bou, Gerd Marit Berge, Bente Ruyter, Tor Gjøen
The shortage of the n-3 fatty acids eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) on the international markets has led to increasing substitution of fish oil by plant oils in Atlantic salmon (Salmo salar) feed and thereby reducing the EPA and DHA content in salmon. However, the minimum required levels of these fatty acids in fish diets for securing fish health are unknown. Fish were fed with 0, 1 or 2% EPA or DHA alone or in combination of both over a period, growing from 50 to 400 g. Primary head kidney leucocytes were isolated and stimulated with Toll-like receptor (TLR) ligands to determine if EPA and DHA deficiency can affect expression of important immune genes and eicosanoid production...
March 9, 2017: Fish Physiology and Biochemistry
https://www.readbyqxmd.com/read/28275164/plasminogen-deficiency-delays-the-onset-and-protects-from-demyelination-and-paralysis-in-autoimmune-neuroinflammatory-disease
#17
Maureen A Shaw, Zhen Gao, Kathryn E McElhinney, Sherry Thornton, Matthew J Flick, Adam Lane, Jay L Degen, Jae Kyu Ryu, Katerina Akassoglou, Eric S Mullins
Multiple Sclerosis (MS) is a neuroinflammatory, demyelinating disease of the central nervous system. Fibrin(ogen) deposition at sites of blood brain barrier breakdown is a prominent feature of neuroinflammatory disease and contributes to disease severity. Plasminogen, the primary fibrinolytic enzyme, also modifies inflammatory processes. We used a murine model of MS, experimental autoimmune encephalomyelitis (EAE), to evaluate the hypothesis that the loss of plasminogen would exacerbate neuroinflammatory disease...
March 8, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28273917/alleviation-of-microglial-activation-induced-by-p38-mapk-mk2-pge2-axis-by-capsaicin-potential-involvement-of-other-than-trpv1-mechanism-s
#18
Harsharan S Bhatia, Nora Roelofs, Eduardo Muñoz, Bernd L Fiebich
Exaggerated inflammatory responses in microglia represent one of the major risk factors for various central nervous system's (CNS) associated pathologies. Release of excessive inflammatory mediators such as prostaglandins and cytokines are the hallmark of hyper-activated microglia. Here we have investigated the hitherto unknown effects of capsaicin (cap) - a transient receptor potential vanilloid 1 (TRPV1) agonist- in murine primary microglia, organotypic hippocampal slice cultures (OHSCs) and human primary monocytes...
December 2017: Scientific Reports
https://www.readbyqxmd.com/read/28271593/norovirus-infection-in-primary-immune-deficiency
#19
REVIEW
Li-An K Brown, Ian Clark, Julianne R Brown, Judith Breuer, David M Lowe
Norovirus is acknowledged to be a leading cause of acute gastroenteritis worldwide, and its importance as a cause of chronic infection in immune deficient hosts is increasingly recognised. Current evidence suggests that a coordinated response of innate immune mechanisms, CD8+ cytotoxicity and a humoral response, with CD4+ orchestration, is necessary for norovirus clearance. We explain how primary immune deficiency impairs these host defences and predisposes to chronic infection, associated with protracted diarrhoea, weight loss, and requirement for parenteral nutrition...
March 8, 2017: Reviews in Medical Virology
https://www.readbyqxmd.com/read/28270364/new-frontiers-in-the-therapy-of-primary-immunodeficiency-from-gene-addition-to-gene-editing
#20
REVIEW
Donald B Kohn, Caroline Y Kuo
The most severe primary immune deficiency diseases (PIDs) have been successfully treated with allogeneic hematopoietic stem cell transplantation for more than 4 decades. However, such transplantations have the best outcomes when there is a well-matched donor available because immune complications, such as graft-versus-host disease, are greater without a matched sibling donor. Gene therapy has been developed as a method to perform autologous transplantations of a patient's own stem cells that are genetically corrected...
March 2017: Journal of Allergy and Clinical Immunology
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