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Primary immune deficiency

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https://www.readbyqxmd.com/read/29790481/antiviral-combination-therapy-for-cytomegalovirus-infection-in-high-risk-infants
#1
Surabhi B Vora, Adam W Brothers, Alpana Waghmare, Janet A Englund
BACKGROUND: Cytomegalovirus (CMV) infection is a major risk factor for mortality in infants with severe combined immunodeficiency (SCID) and other profound immune defects. Specific antiviral therapy must be initiated early and aggressively because of the potential for antiviral resistance, rapid dissemination and poor transplant outcomes. Combination antiviral therapy is routinely administered for some viral infections, but the value of this approach for the treatment of CMV is unclear...
May 23, 2018: Antiviral Therapy
https://www.readbyqxmd.com/read/29786788/transplantable-animal-studies-and-whole-body-optical-imaging-in-prostate-carcinoma
#2
Geertje van der Horst, Maaike van der Mark, Henry Cheung, Gabri van der Pluijm
Current treatments of advanced prostate cancer only marginally increase overall survival and can be regarded as predominantly palliative. Hence, there is an urgent need for novel therapeutic strategies for the treatment of primary tumors and, more importantly perhaps, for the prevention of tumor progression and metastasis formation. Clinically relevant preclinical models are therefore urgently needed. An ideal, clinically relevant preclinical model would mimic the genetic and phenotypic changes that occur at the different stages of human prostate cancer progression and subsequent metastasis...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29781680/provision-of-antiretroviral-therapy-for-children-in-nelson-mandela-bay-health-care-professionals-challenges
#3
Margaret Williams, Dalena R M Van Rooyen, Esmeralda J Ricks
BACKGROUND:  The human immunodeficiency virus and/or acquired immune deficiency syndrome (HIV/AIDS) pandemic continues to increase in prevalence worldwide, particularly in South Africa, and includes the often overlooked paediatric population. The provision of paediatric antiretroviral treatment (ART) is as essential for children as for adults, and has numerous obstacles, not least of which is lack of decentralisation of facilities to provide essential treatment. Optimising ART, care and support for HIV-positive children, and their caregivers, at public sector primary health care (PHC) clinics is crucial to improve morbidity and mortality rates in children...
March 12, 2018: African Journal of Primary Health Care & Family Medicine
https://www.readbyqxmd.com/read/29777306/respiratory-manifestations-in-lps-responsive-beige-like-anchor-lrba-protein-deficient-patients
#4
Oded Shamriz, Bella Shadur, Adeeb NaserEddin, Irina Zaidman, Natalia Simanovsky, Orly Elpeleg, Eitan Kerem, Joel Reiter, Polina Stepensky
Lipopolysaccharide (LPS)-responsive beige-like anchor (LRBA) protein deficiency is a rare syndrome of primary immune deficiency and immune dysregulation. In this study, we sought to summarize our experience with respiratory manifestations in LRBA-deficient patients. We conducted a retrospective analysis of the medical records of LRBA-deficient patients treated at Hadassah-Hebrew University Medical Center, Jerusalem, Israel. Data retrieved included pulmonary workup, disease course, treatment, and outcome. Ten patients were included...
May 18, 2018: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29776993/tumor-immune-evasion-arises-through-loss-of-tnf-sensitivity
#5
Conor J Kearney, Stephin J Vervoort, Simon J Hogg, Kelly M Ramsbottom, Andrew J Freeman, Najoua Lalaoui, Lizzy Pijpers, Jessica Michie, Kristin K Brown, Deborah A Knight, Vivien Sutton, Paul A Beavis, Ilia Voskoboinik, Phil K Darcy, John Silke, Joseph A Trapani, Ricky W Johnstone, Jane Oliaro
Immunotherapy has revolutionized outcomes for cancer patients, but the mechanisms of resistance remain poorly defined. We used a series of whole-genome clustered regularly interspaced short palindromic repeat (CRISPR)-based screens performed in vitro and in vivo to identify mechanisms of tumor immune evasion from cytotoxic lymphocytes [CD8+ T cells and natural killer (NK) cells]. Deletion of key genes within the tumor necrosis factor (TNF) signaling, interferon-γ (IFN-γ) signaling, and antigen presentation pathways provided protection of tumor cells from CD8+ T cell-mediated killing and blunted antitumor immune responses in vivo...
May 18, 2018: Science Immunology
https://www.readbyqxmd.com/read/29769344/efficient-delivery-of-hcmv-t-cell-antigens-by-attenuated-sendai-virus-vectors
#6
Richard Kiener, Markus Fleischmann, Marian Alexander Wiegand, Niels A W Lemmermann, Christiane Schwegler, Christine Kaufmann, Angelique Renzaho, Simone Thomas, Eva Felder, Hans Helmut Niller, Benedikt Asbach, Ralf Wagner
Human Cytomegalovirus (HCMV) represents a major cause of clinical complications during pregnancy as well as immunosuppression and the licensing of a protective HCMV vaccine remains an unmet global need. Herein, we designed and validated novel Sendai virus (SeV) vectors delivering T cell immunogens IE-1 and pp65. To enhance vector safety, we used a replication-deficient strain (rdSeV) that infects target cells in a non-productive manner while retaining viral gene expression. In this study, we explored the impact that transduction with rdSeV has on human dendritic cells (DCs) by comparing it to the parental, replication-competent Sendai virus strain (rcSeV) as well as the poxvirus strain Modified Vaccinia Ankara (MVA)...
May 16, 2018: Journal of Virology
https://www.readbyqxmd.com/read/29761169/prolonged-cenicriviroc-therapy-reduces-hepatic-fibrosis-despite-steatohepatitis-in-a-diet-induced-mouse-model-of-nonalcoholic-steatohepatitis
#7
Annie J Kruger, Bryan C Fuchs, Ricard Masia, Jacinta A Holmes, Shadi Salloum, Mozhdeh Sojoodi, Diego S Ferreira, Stephanie M Rutledge, Peter Caravan, Nadia Alatrakchi, Pam Vig, Eric Lefebvre, Raymond T Chung
Nonalcoholic steatohepatitis (NASH) is a progressive liver disease projected to become the leading cause of cirrhosis and liver transplantation in the next decade. Cenicriviroc (CVC), a dual chemokine receptor 2 and 5 antagonist, prevents macrophage trafficking and is under clinical investigation for the treatment of human NASH fibrosis. We assessed the efficacy and durability of short and prolonged CVC therapy in a diet-induced mouse model of NASH, the choline deficient, L-amino acid-defined, high-fat diet (CDAHFD) model...
May 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29744787/ada-deficiency-evaluation-of-the-clinical-and-laboratory-features-and-the-outcome
#8
Deniz Cagdas, Pınar Gur Cetinkaya, Betül Karaatmaca, Saliha Esenboga, Cagman Tan, Togay Yılmaz, Ersin Gümüş, Safa Barış, Barış Kuşkonmaz, Tuba Turul Ozgur, Pawan Bali, Ines Santisteban, Diclehan Orhan, Aysel Yüce, Duygu Cetinkaya, Kaan Boztug, Michael Hershfield, Ozden Sanal, İlhan Tezcan
INTRODUCTION: Adenosine deaminase (ADA) deficiency is an autosomal recessive primary immunodeficiency. It results in the intracellular accumulation of toxic metabolites which have effects particularly on lymphocytes and the brain. The aim of this study was to evaluate the outcome of 13 ADA-deficient patients. We planned to evaluate their clinical and laboratory findings before and after enzyme replacement therapy (ERT), allogeneic hematopoietic stem cell transplantation (aHSCT), and hematopoietic stem cell gene therapy (HSCGT)...
May 9, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29743364/t-cell-derived-lymphotoxin-is-essential-for-anti-hsv-1-humoral-immune-response
#9
Kaiting Yang, Yong Liang, Zhichen Sun, Diyuan Xue, Hairong Xu, Mingzhao Zhu, Yang-Xin Fu, Hua Peng
B cell-derived lymphotoxin (LT) is required for the development of follicular dendritic cell clusters for the formation of primary and secondary lymphoid follicles, but the role of T cell-derived LT in antibody response has not been well demonstrated. We observed that lymphotoxin-β-receptor (LTβR) signaling is essential for optimal humoral immune response and protection against an acute HSV-1 infection. Blocking the LTβR pathway caused poor maintenance of germinal center B (GC-B) cells and follicular helper T (Tfh) cells...
May 9, 2018: Journal of Virology
https://www.readbyqxmd.com/read/29743177/hypomorphic-rag1-mutations-alter-the-pre-immune-repertoire-at-early-stages-of-lymphoid-development
#10
Lisa M Ott de Bruin, Marita Bosticardo, Alessandro Barbieri, Sherry G Lin, Jared H Rowe, Pietro L Poliani, Kimberly Ching, Daniel Eriksson, Nils Landegren, Olle Kämpe, John P Manis, Luigi D Notarangelo
Hypomorphic RAG1 mutations allowing residual T and B cell development have been found in patients presenting with delayed-onset combined immune deficiency with granulomas and/or autoimmunity (CID-G/AI) and abnormalities of the peripheral T and B cell repertoire. To examine how hypomorphic Rag1 mutations affect the earliest stages of lymphocyte development, we used CRISPR/Cas9 to generate mouse models with equivalent mutations found in patients with CID-G/AI. Immunological characterization showed partial development of T and B lymphocytes, with persistence of naïve cells, preserved serum immunoglobulin, but impaired antibody responses and presence of autoantibodies, thereby recapitulating the phenotype seen in patients with CID-G/AI...
May 9, 2018: Blood
https://www.readbyqxmd.com/read/29740582/anti-high-density-lipoprotein-antibodies-and-antioxidant-dysfunction-in-immune-driven-diseases
#11
Javier Rodríguez-Carrio, Lourdes Mozo, Patricia López, Elena Nikiphorou, Ana Suárez
Introduction: Impaired high-density lipoprotein (HDL) levels and antioxidant functionality of HDL, mainly attributed to a decreased paraoxonase-1 (PON1) functionality, have been described in autoimmune conditions. In this setting, a role for humoral response in cardiovascular disease is emerging. This study evaluates the role of immunoglobulin G (IgG) antibodies against HDL and disease-related autoantibodies on HDL dysfunction in immune-driven diseases. Methods: Serum IgG anti-HDL antibodies, PON1 activity, and total antioxidant capacity (TAC) were quantified in 381 patients with different immune-driven diseases [18 mixed connective tissue disease (MCTD), 35 primary Sjögren syndrome (pSS), 38 systemic sclerosis (SSc), 33 ANCA-associated vasculitis (AAV), 60 diabetes mellitus 1, 29 autoimmune B12 deficiency/pernicious anemia, 29 primary biliary cirrhosis, 46 IBD/Crohn, 54 IBD/UC, and 39 celiac disease (CD)] and 138 healthy controls...
2018: Frontiers in Medicine
https://www.readbyqxmd.com/read/29736312/focal-adhesion-kinase-fak-deficiency-in-mononuclear-phagocytes-alters-murine-breast-tumor-progression
#12
Ryan A Llewellyn, Michael F Gutknecht, Keena S Thomas, Mark R Conaway, Amy H Bouton
While it has long been recognized that mononuclear phagocytes play a significant role in determining breast tumor progression, the molecular factors that contribute to these events are not fully understood. In this report, we sought to determine whether focal adhesion kinase (FAK) expression in this cell population influences primary breast tumor initiation and growth. Using the MMTV-polyoma middle T (PyVmT) murine model of spontaneous breast cancer, we found that FAK expression in mononuclear phagocytes accelerates tumor initiation/progression during the early stages of PyVmT tumor growth but subsequently restricts tumor growth once the tumors have transitioned to malignancy...
2018: American Journal of Cancer Research
https://www.readbyqxmd.com/read/29732319/epstein-barr-virus-in-gliomas-cause-association-or-artifact
#13
REVIEW
Saghir Akhtar, Semir Vranic, Farhan Sachal Cyprian, Ala-Eddin Al Moustafa
Gliomas are the most common malignant brain tumors and account for around 60% of all primary central nervous system cancers. Glioblastoma multiforme (GBM) is a grade IV glioma associated with a poor outcome despite recent advances in chemotherapy. The etiology of gliomas is unknown, but neurotropic viruses including the Epstein-Barr virus (EBV) that is transmitted via salivary and genital fluids have been implicated recently. EBV is a member of the gamma herpes simplex family of DNA viruses that is known to cause infectious mononucleosis (glandular fever) and is strongly linked with the oncogenesis of several cancers, including B-cell lymphomas, nasopharyngeal, and gastric carcinomas...
2018: Frontiers in Oncology
https://www.readbyqxmd.com/read/29729154/the-effect-of-the-proteolisis-system-activity-for-the-trophological-status-of-patients-with-osteoarthrosis-and-excretory-insufficiency-of-pancreas
#14
Liliya S Babinets, Iryna M Halabitska, Yuliia Ya Kotsaba, Iryna O Borovyk, Bogdan O Migenko, Svitlana S Ryabokon, Lydmila S Tsybulska
OBJECTIVE: Introduction:The excretory insufficiency of pancreas in patients with primary osteoarthrosis is formed at the comorbid pathologies and as a result of long-term treatment of osteoarthrosis using the non-steroidal anti-inflammatory drugs, steroids, chondroprotectors and chondrostimulators etc. The aim: to study the state of the proteolysis system and immune status, the presence and depth of the dysbiosis of colon in patients with primary osteoarthrosis against a violation of their excretory insufficiency of pancreas...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29727410/improved-diagnosis-and-treatment-of-bone-and-joint-infections-using-an-evidence-based-treatment-guideline
#15
Rachel D Quick, John Williams, Marisol Fernandez, Hilton Gottschalk, Peter Cosgrove, Kyle Kahlden, Kathryn Merkel, Lynn Thoreson, Patrick Boswell, Sarmistha B Hauger
BACKGROUND: Our institution created a multidisciplinary guideline for treatment of acute hematogenous osteomyelitis (AHO) and septic arthritis (SA) in response to updates in evidence-based literature in the field and existing provider variability in treatment. This guideline aims to improve the care of these patients by standardizing diagnosis and treatment and incorporating up to date evidence-based research into practice. The primary objective of this study is to compare cases before versus after the implementation of the guideline to determine concrete effects the guideline has had in the care of patients with AHO and SA...
May 2, 2018: Journal of Pediatric Orthopedics
https://www.readbyqxmd.com/read/29720226/increased-a20-e3-ubiquitin-ligase-interactions-in-bid-deficient-glia-attenuate-tlr3-and-tlr4-induced-inflammation
#16
Sinéad Kinsella, Michael Fichtner, Orla Watters, Hans-Georg König, Jochen H M Prehn
BACKGROUND: Chronic pro-inflammatory signaling propagates damage to neural tissue and affects the rate of disease progression. Increased activation of Toll-like receptors (TLRs), master regulators of the innate immune response, is implicated in the etiology of several neuropathologies including amyotrophic lateral sclerosis, Alzheimer's disease, and Parkinson's disease. Previously, we identified that the Bcl-2 family protein BH3-interacting domain death agonist (Bid) potentiates the TLR4-NF-κB pro-inflammatory response in glia, and specifically characterized an interaction between Bid and TNF receptor associated factor 6 (TRAF6) in microglia in response to TLR4 activation...
May 2, 2018: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/29715493/spleen-development-is-modulated-by-neonatal-gut-microbiota
#17
M Manuela Rosado, Alaitz Aranburu, Marco Scarsella, Simona Cascioli, Ezio Giorda, Federica Del Chierico, Stefano Levi Mortera, Eva Piano Mortari, Stefania Petrini, Lorenza Putignani, Rita Carsetti
The full development of the mammalian immune system occurs after birth upon exposure to non self-antigens. The gut is the first site of bacterial colonization where it is crucial to create the appropriate microenvironment able to balance effector or tolerogenic responses to external stimuli. It is a well-established fact that at mucosal sites bacteria play a key role in developing the immune system but we ignore how colonising bacteria impact the maturation of the spleen. Here we addressed this issue. Taking advantage of the fact that milk SIgA regulates bacterial colonization of the newborn intestine, we generated immunocompetent mice born either from IgA pro-efficient or IgA deficient females...
April 28, 2018: Immunology Letters
https://www.readbyqxmd.com/read/29713003/multi-omics-profiling-of-younger-asian-breast-cancers-reveals-distinctive-molecular-signatures
#18
Zhengyan Kan, Ying Ding, Jinho Kim, Hae Hyun Jung, Woosung Chung, Samir Lal, Soonweng Cho, Julio Fernandez-Banet, Se Kyung Lee, Seok Won Kim, Jeong Eon Lee, Yoon-La Choi, Shibing Deng, Ji-Yeon Kim, Jin Seok Ahn, Ying Sha, Xinmeng Jasmine Mu, Jae-Yong Nam, Young-Hyuck Im, Soohyeon Lee, Woong-Yang Park, Seok Jin Nam, Yeon Hee Park
Breast cancer (BC) in the Asia Pacific regions is enriched in younger patients and rapidly rising in incidence yet its molecular bases remain poorly characterized. Here we analyze the whole exomes and transcriptomes of 187 primary tumors from a Korean BC cohort (SMC) enriched in pre-menopausal patients and perform systematic comparison with a primarily Caucasian and post-menopausal BC cohort (TCGA). SMC harbors higher proportions of HER2+ and Luminal B subtypes, lower proportion of Luminal A with decreased ESR1 expression compared to TCGA...
April 30, 2018: Nature Communications
https://www.readbyqxmd.com/read/29701871/pediatric-intestinal-failure-the-key-outcomes-for-the-first-100-patients-treated-in-a-national-tertiary-referral-center-during-1984-2017
#19
Laura Merras-Salmio, Annika Mutanen, Elisa Ylinen, Risto Rintala, Antti Koivusalo, Mikko P Pakarinen
BACKGROUND: Pediatric-onset intestinal failure (IF) remains a severe illness with life-threatening consequences. In this study, we analyzed a single center's outcomes of IF over 3 decades. METHODS: All children with IF who required parenteral nutrition (PN) >2 months or small-intestinal resection ≥50% managed since 1984 were included for retrospective outcome analyses. RESULTS: In total, 100 patients with median PN duration of 1.2 (interquartile range, 0...
April 27, 2018: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29698474/interferon-induced-protein-35-exacerbates-h5n1-influenza-disease-through-the-expression-of-il-12p40-homodimer
#20
Anshu P Gounder, Christine C Yokoyama, Nicholas N Jarjour, Traci L Bricker, Brian T Edelson, Adrianus C M Boon
Pro-inflammatory cytokinemia is a hallmark of highly pathogenic H5N1 influenza virus (IAV) disease yet little is known about the role of host proteins in modulating a pathogenic innate immune response. The host Interferon Induced Protein 35 (Ifi35) has been implicated in increased susceptibility to H5N1-IAV infection. Here, we show that Ifi35 deficiency leads to reduced morbidity in mouse models of highly pathogenic H5N1- and pandemic H1N1-IAV infection. Reduced weight loss in Ifi35-/- mice following H5N1-IAV challenge was associated with reduced cellular infiltration and decreased production of specific cytokines and chemokines including IL-12p40...
April 2018: PLoS Pathogens
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