keyword
MENU ▼
Read by QxMD icon Read
search

Primary immune deficiency

keyword
https://www.readbyqxmd.com/read/28086824/d-dimer-is-an-essential-accompaniment-of-circulating-tumor-cells-in-gastric-cancer
#1
Dongmei Diao, Yao Cheng, Yongchun Song, Hao Zhang, Zhangjian Zhou, Chengxue Dang
BACKGROUND: Fibrinogen (FIB) is an important source of fibrin, which plays a crucial role in circulating tumor cells (CTCs) extravasation and distant metastasis development. We hypothesize it's stable final product, plasma D-dimer, may be associated with CTCs appearance and can reflect the metastatic phenotype in cancer patients. METHODS: We first verified our hypothesis in different murine gastric cancer metastasis models in vivo, plasma D-dimer and fibrinogen as well as its degradation products were directly examined in three metastasis immune-deficient mouse models and in controls...
January 13, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28079912/intravenous-immunoglobulin-in-the-treatment-of-primary-immunodeficiency-diseases
#2
Deirdre De Ranieri, Nana Sarkoah Fenny
Intravenous immunoglobulin (IVIG) has been used as antibody replacement therapy in primary immunodeficiency diseases (PIDDs) for more than 50 years. Its role as a therapeutic agent has expanded over the past couple of decades as its anti-inflammatory and immune-modulatory mechanisms of action have been elucidated. It is now used "off-label" to treat other autoimmune diseases. This article focuses on the role of IVIG in the treatment of PIDDs characterized by absent or deficient antibody production. Replacement doses are given on a monthly basis in these conditions as a prophylactic measure to prevent acute and serious bacterial infections...
January 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28077132/foxn1-deficient-nude-severe-combined-immunodeficiency
#3
REVIEW
Ioanna A Rota, Fatima Dhalla
Nude severe combined immunodeficiency is a rare inherited disease caused by autosomal recessive loss-of-function mutations in FOXN1. This gene encodes a transcription factor essential for the development of the thymus, the primary lymphoid organ that supports T-cell development and selection. To date nine cases have been reported presenting with the clinical triad of absent thymus resulting in severe T-cell immunodeficiency, congenital alopecia universalis and nail dystrophy. Diagnosis relies on testing for FOXN1 mutations, which allows genetic counselling and guides therapeutic management...
January 11, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28070898/clearance-of-recalcitrant-warts-in-a-patient-with-idiopathic-immune-deficiency-following-administration-of-the-quadrivalent-human-papillomavirus-vaccine
#4
S P Smith, H E Baxendale, J C Sterling
Human papillomavirus (HPV)-induced cutaneous warts are potentially serious and debilitating. In immunosuppressed patients, these warts may be resistant to standard therapies. We report a case of a young patient with a primary immune deficiency whose recalcitrant cutaneous warts regressed completely following administration of a quadrivalent HPV vaccine.
January 10, 2017: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/28068853/chronic-lung-disease-in-common-variable-immune-deficiency-cvid-a-pathophysiological-role-for-microbial-and-non-b-cell-immune-factors
#5
Denver Mooney, David Edgar, Gisli Einarsson, Damian Downey, Stuart Elborn, Michael Tunney
One of the most common and most severe forms of primary antibody deficiency encountered in the clinical setting is a heterogeneous group of syndromes termed common variable immune deficiency (CVID). This disorder is characterized by reduced immunoglobulin production and increased susceptibility to infection, particularly of the respiratory tract. Infection and subsequent immunological/inflammatory processes may contribute to the development of pulmonary complications such as bronchiectasis and interstitial lung disease...
January 10, 2017: Critical Reviews in Microbiology
https://www.readbyqxmd.com/read/28067908/genome-wide-association-study-implicates-immune-activation-of-multiple-integrin-genes-in-inflammatory-bowel-disease
#6
Katrina M de Lange, Loukas Moutsianas, James C Lee, Christopher A Lamb, Yang Luo, Nicholas A Kennedy, Luke Jostins, Daniel L Rice, Javier Gutierrez-Achury, Sun-Gou Ji, Graham Heap, Elaine R Nimmo, Cathryn Edwards, Paul Henderson, Craig Mowat, Jeremy Sanderson, Jack Satsangi, Alison Simmons, David C Wilson, Mark Tremelling, Ailsa Hart, Christopher G Mathew, William G Newman, Miles Parkes, Charlie W Lees, Holm Uhlig, Chris Hawkey, Natalie J Prescott, Tariq Ahmad, John C Mansfield, Carl A Anderson, Jeffrey C Barrett
Genetic association studies have identified 215 risk loci for inflammatory bowel disease, thereby uncovering fundamental aspects of its molecular biology. We performed a genome-wide association study of 25,305 individuals and conducted a meta-analysis with published summary statistics, yielding a total sample size of 59,957 subjects. We identified 25 new susceptibility loci, 3 of which contain integrin genes that encode proteins in pathways that have been identified as important therapeutic targets in inflammatory bowel disease...
January 9, 2017: Nature Genetics
https://www.readbyqxmd.com/read/28066964/mechanisms-of-the-prevention-and-inhibition-of-the-progression-and-development-of-nash-by-genetic-and-pharmacological-dcr3-supplementation
#7
Pei-Chang Lee, Ling-Yu Yang, Ying-Wen Wang, Shiang-Fen Huang, Kuei-Chuan Lee, Yun-Cheng Hsieh, Ying-Ying Yang, Shie-Liang Hsieh, Ming-Chih Hou, Han-Chieh Lin, Fa-Yuah Lee, Shou-Dong Lee
AIMS: Treatment to non-alcoholic steatohepatitis (NASH) is difficult due to the absence of a proven treatment and its comprehensive mechanisms. In the NASH animal model, up-regulated hepatic inflammation and oxidative stress with the resultant M1 polarization of macrophage as well as the imbalanced adipocytokines all accelerate NASH progression. As a member of TNF receptor superfamily, decoy receptor 3 (DcR3) not only neutralizes the death ligands, but also performs immune modulations...
January 8, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28066800/cd36-deficiency-impairs-the-small-intestinal-barrier-and-induces-subclinical-inflammation-in-mice
#8
Vincenza Cifarelli, Stoyan Ivanov, Yan Xie, Ni-Huiping Son, Brian T Saunders, Terri A Pietka, Trevor M Shew, Jun Yoshino, Sinju Sundaresan, Nicholas O Davidson, Ira J Goldberg, Andrew E Gelman, Bernd H Zinselmeyer, Gwendalyn J Randolph, Nada A Abumrad
BACKGROUND & AIMS: CD36 has immuno-metabolic actions and is abundant in the small intestine on epithelial, endothelial and immune cells. We examined the role of CD36 in gut homeostasis using mice null for CD36 (CD36KO) and with CD36 deletion specific to enterocytes (Ent-CD36KO) or endothelial cells (EC-CD36KO). METHODS: Intestinal morphology was evaluated using immunohistochemistry and electron microscopy (EM). Intestinal inflammation was determined from neutrophil infiltration and expression of cytokines, toll-like receptors and COX-2...
January 2017: Cellular and Molecular Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28065678/construction-and-validation-of-a-novel-disease-specific-quality-of-life-instrument-for-patients-with-primary-antibody-deficiency-disease-padqol-16
#9
Mark Ballow, Mark R Conaway, Panida Sriaroon, Rima A Rachid, Filiz O Seeborg, Carla M Duff, Francisco A Bonilla, M Elizabeth M Younger, Ralph Shapiro, Ted M Burns
A disease-specific Health-related Quality of Life survey was developed for adult patients with antibody immune deficiency disorders as a tool (PADQOL-16) for assessing a patient's QOL issues and their relation to clinical status.
January 5, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28059079/trim14-is-a-putative-tumor-suppressor-and-regulator-of-innate-immune-response-in-non-small-cell-lung-cancer
#10
Josephine Hai, Chang-Qi Zhu, Tao Wang, Shawna L Organ, Frances A Shepherd, Ming-Sound Tsao
Non-small-cell lung carcinoma (NSCLC) accounts for 85% of malignant lung tumors and is the leading cause of cancer deaths. Our group previously identified Tripartite Motif 14 (TRIM14) as a component of a prognostic multigene expression signature for NSCLC. Little is known about the function of TRIM14 protein in normal or disease states. We investigated the functional and prognostic role of TRIM14 in NSCLC using in vitro and in vivo perturbation model systems. Firstly, a pooled RNAi screen identified TRIM14 to effect cell proliferation/survival in NSCLC cells...
January 6, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28056867/risk-factors-and-characteristics-of-blood-stream-infections-in-patients-with-newly-diagnosed-multiple-myeloma
#11
Chun-Teng Huang, Chia-Jen Liu, Po-Shen Ko, Han-Tsung Liu, Yuan-Bin Yu, Liang-Tsai Hsiao, Jyh-Pyng Gau, Cheng-Hwai Tzeng, Tzeon-Jye Chiou, Jin-Hwang Liu, Muh-Hwa Yang, Ling-Ju Huang, Chun-Yu Liu
BACKGROUND: Patients with multiple myeloma are generally immune-compromised either due to pronounced depression in primary antibody responses or because of anti-myeloma therapy. Infection is a major risk factor for early deaths among these patients. The impact of blood stream infections (BSI) on newly diagnosed myeloma patients has been less studied. We aimed to study the incidence and risk factors of BSI within 3 months after diagnosis of multiple myeloma in a tertiary referral center...
January 6, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28051085/intrinsic-4-1bb-signals-are-indispensable-for-the-establishment-of-an-influenza-specific-tissue-resident-memory-cd8-t-cell-population-in-the-lung
#12
A C Zhou, L E Wagar, M E Wortzman, T H Watts
The induction of long-lived heterotypic T-cell protection against influenza virus remains elusive, despite the conservation of T-cell epitopes. T-cell protection against influenza is critically dependent on lung-resident memory T cells (Trm). Here we show that intranasal administration of 4-1BBL along with influenza nucleoprotein in a replication-defective adenovirus vector to influenza pre-immune mice induces a remarkably stable circulating effector memory CD8 T-cell population characterized by higher IL-7Rα expression than control-boosted T cells, as well as a substantial lung parenchymal CD69(+) CD8 Trm population, including both CD103(+) and CD103(-) cells...
January 4, 2017: Mucosal Immunology
https://www.readbyqxmd.com/read/28049839/gas6-is-a-key-homeostatic-immunological-regulator-of-host-commensal-interactions-in-the-oral-mucosa
#13
Maria Nassar, Yaara Tabib, Tal Capucha, Gabriel Mizraji, Tsipora Nir, Meirav Pevsner-Fischer, Gili Zilberman-Schapira, Oded Heyman, Gabriel Nussbaum, Herve Bercovier, Asaf Wilensky, Eran Elinav, Tal Burstyn-Cohen, Avi-Hai Hovav
The oral epithelium contributes to innate immunity and oral mucosal homeostasis, which is critical for preventing local inflammation and the associated adverse systemic conditions. Nevertheless, the mechanisms by which the oral epithelium maintains homeostasis are poorly understood. Here, we studied the role of growth arrest specific 6 (GAS6), a ligand of the TYRO3-AXL-MERTK (TAM) receptor family, in regulating oral mucosal homeostasis. Expression of GAS6 was restricted to the outer layers of the oral epithelium...
January 3, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28038357/streptococcus-gordonii-induces-nitric-oxide-production-through-its-lipoproteins-stimulating-toll-like-receptor-2-in-murine-macrophages
#14
Hyun Young Kim, Jung Eun Baik, Ki Bum Ahn, Ho Seong Seo, Cheol-Heui Yun, Seung Hyun Han
Streptococcus gordonii, a Gram-positive commensal in the oral cavity, is an opportunistic pathogen that can cause endodontic and systemic infections resulting in infective endocarditis. Lipoteichoic acid (LTA) and lipoprotein are major virulence factors of Gram-positive bacteria that are preferentially recognized by Toll-like receptor 2 (TLR2) on immune cells. In the present study, we investigated the effect of S. gordonii LTA and lipoprotein on the production of the representative inflammatory mediator nitric oxide (NO) by the mouse macrophages...
December 27, 2016: Molecular Immunology
https://www.readbyqxmd.com/read/28031339/staphylococcus-aureus-protein-a-disrupts-immunity-mediated-by-long-lived-plasma-cells
#15
Amanda B Keener, Lance T Thurlow, SunAh Kang, Nicholas A Spidale, Stephen H Clarke, Kenji M Cunnion, Roland Tisch, Anthony R Richardson, Barbara J Vilen
Infection with Staphylococcus aureus does not induce long-lived protective immunity for reasons that are not completely understood. Human and murine vaccine studies support a role for Abs in protecting against recurring infections, but S. aureus modulates the B cell response through expression of staphylococcus protein A (SpA), a surface protein that drives polyclonal B cell expansion and induces cell death in the absence of costimulation. In this murine study, we show that SpA altered the fate of plasmablasts and plasma cells (PCs) by enhancing the short-lived extrafollicular response and reducing the pool of bone marrow (BM)-resident long-lived PCs...
December 28, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28030522/prevalence-of-cryptosporidium-carriage-and-disease-in-children-with-primary-immune-deficiencies-undergoing-hematopoietic-stem-cell-transplant-in-northern-europe
#16
Angharad P Davies, Mary Slatter, Andrew R Gennery, Guy Robinson, Nigel Crouch, Kristin Elwin, Stephen J Hadfield, Andrew J Cant, E Graham Davies, Rachel M Chalmers
A prospective cohort study of children with primary immunodeficiencies undergoing hematopoietic stem cell transplant (HSCT) in the UK investigated the extent and significance of Cryptosporidium carriage in this high-risk group. Three of 42 children recruited were infected with Cryptosporidium, a lower proportion than previously described. One had serious disease. The underlying immunodeficiency likely had a bearing on the clinical presentation and possible outcome of infection.
December 27, 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28013299/autoimmunity-in-primary-antibody-deficiencies
#17
Gholamreza Azizi, Moslem Ahmadi, Hassan Abolhassani, Reza Yazdani, Hamed Mohammadi, Abbas Mirshafiey, Nima Rezaei, Asghar Aghamohammadi
Primary antibody deficiencies (PADs) are the most common inherited primary immunodeficiencies in humans, characterized by hypogammaglobulinemia, an inability to produce specific antibodies, and recurrent infections mainly caused by encapsulated bacteria. However, it has been shown that inflammatory disorders, granulomatous lesions, lymphoproliferative diseases, cancer, and autoimmunity are associated with the various types of PAD. Both systemic and organ-specific autoimmune diseases could be attributed to B-cell defects in PAD patients...
December 24, 2016: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/28011864/combined-immunodeficiency-and-epstein-barr-virus-induced-b-cell-malignancy-in-humans-with-inherited-cd70-deficiency
#18
Hassan Abolhassani, Emily S J Edwards, Aydan Ikinciogullari, Huie Jing, Stephan Borte, Marcus Buggert, Likun Du, Mami Matsuda-Lennikov, Rosa Romano, Rozina Caridha, Sangeeta Bade, Yu Zhang, Juliet Frederiksen, Mingyan Fang, Sevgi Kostel Bal, Sule Haskologlu, Figen Dogu, Nurdan Tacyildiz, Helen F Matthews, Joshua J McElwee, Emma Gostick, David A Price, Umaimainthan Palendira, Asghar Aghamohammadi, Bertrand Boisson, Nima Rezaei, Annika C Karlsson, Michael J Lenardo, Jean-Laurent Casanova, Lennart Hammarström, Stuart G Tangye, Helen C Su, Qiang Pan-Hammarström
In this study, we describe four patients from two unrelated families of different ethnicities with a primary immunodeficiency, predominantly manifesting as susceptibility to Epstein-Barr virus (EBV)-related diseases. Three patients presented with EBV-associated Hodgkin's lymphoma and hypogammaglobulinemia; one also had severe varicella infection. The fourth had viral encephalitis during infancy. Homozygous frameshift or in-frame deletions in CD70 in these patients abolished either CD70 surface expression or binding to its cognate receptor CD27...
January 2017: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28009288/engineered-murine-hscs-reconstitute-multi-lineage-hematopoiesis-and-adaptive-immunity
#19
Yi-Fen Lu, Patrick Cahan, Samantha Ross, Julie Sahalie, Patricia M Sousa, Brandon K Hadland, Wenqing Cai, Erik Serrao, Alan N Engelman, Irwin D Bernstein, George Q Daley
Hematopoietic stem cell (HSC) transplantation is curative for malignant and genetic blood disorders, but is limited by donor availability and immune-mismatch. Deriving HSCs from patient-matched embryonic/induced-pluripotent stem cells (ESCs/iPSCs) could address these limitations. Prior efforts in murine models exploited ectopic HoxB4 expression to drive self-renewal and enable multi-lineage reconstitution, yet fell short in delivering robust lymphoid engraftment. Here, by titrating exposure of HoxB4-ESC-HSC to Notch ligands, we report derivation of engineered HSCs that self-renew, repopulate multi-lineage hematopoiesis in primary and secondary engrafted mice, and endow adaptive immunity in immune-deficient recipients...
December 20, 2016: Cell Reports
https://www.readbyqxmd.com/read/28008925/biallelic-jak1-mutations-in-immunodeficient-patient-with-mycobacterial-infection
#20
Davide Eletto, Siobhan O Burns, Ivan Angulo, Vincent Plagnol, Kimberly C Gilmour, Frances Henriquez, James Curtis, Miguel Gaspar, Karolin Nowak, Vanessa Daza-Cajigal, Dinakantha Kumararatne, Rainer Doffinger, Adrian J Thrasher, Sergey Nejentsev
Mutations in genes encoding components of the immune system cause primary immunodeficiencies. Here, we study a patient with recurrent atypical mycobacterial infection and early-onset metastatic bladder carcinoma. Exome sequencing identified two homozygous missense germline mutations, P733L and P832S, in the JAK1 protein that mediates signalling from multiple cytokine receptors. Cells from this patient exhibit reduced JAK1 and STAT phosphorylation following cytokine stimulations, reduced induction of expression of interferon-regulated genes and dysregulated cytokine production; which are indicative of signalling defects in multiple immune response pathways including Interferon-γ production...
December 23, 2016: Nature Communications
keyword
keyword
17398
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"