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Common Variable Immune disease

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https://www.readbyqxmd.com/read/28430642/immunocompetent-mouse-allograft-models-for-development-of-therapies-to-target-breast-cancer-metastasis
#1
Yuan Yang, Howard H Yang, Ying Hu, Peter H Watson, Huaitian Liu, Thomas R Geiger, Miriam R Anver, Diana C Haines, Philip Martin, Jeffrey E Green, Maxwell P Lee, Kent W Hunter, Lalage M Wakefield
Effective drug development to combat metastatic disease in breast cancer would be aided by the availability of well-characterized preclinical animal models that (a) metastasize with high efficiency, (b) metastasize in a reasonable time-frame, (c) have an intact immune system, and (d) capture some of the heterogeneity of the human disease. To address these issues, we have assembled a panel of twelve mouse mammary cancer cell lines that can metastasize efficiently on implantation into syngeneic immunocompetent hosts...
February 25, 2017: Oncotarget
https://www.readbyqxmd.com/read/28429127/uveitis-in-siriraj-hospital-pattern-differences-between-immune-related-uveitis-and-infectious-uveitis-in-a-university-based-tertiary-care-hospital
#2
Nattaporn Tesavibul, Sutasinee Boonsopon, Pitipol Choopong, Sujintana Tanterdtham
PURPOSE: To describe a proportion of uveitis and to analyse differences between immune-related uveitis and infectious uveitis groups. METHODS: A retrospective study of 458 uveitis patients were categorized into immune-related uveitis, infectious uveitis, masquerade, and undetermined groups. The pattern of inflammation was described. Subgroup analysis was performed to compare pattern differences between immune-related uveitis and infectious uveitis groups. RESULTS: The most common location of inflammation was the anterior eye segment...
April 20, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28416017/patient-complexity-and-genotype-phenotype-correlations-in-biliary-atresia-a-cross-sectional-analysis
#3
Guo Cheng, Patrick Ho-Yu Chung, Edwin Kin-Wai Chan, Man-Ting So, Pak-Chung Sham, Stacey S Cherny, Paul Kwong-Hang Tam, Maria-Mercè Garcia-Barceló
BACKGROUND: Biliary Atresia (BA) is rare and genetically complex, and the pathogenesis is elusive. The disease course is variable and can represent heterogeneity, which hinders effective disease management. Deciphering the BA phenotypic variance is a priority in clinics and can be achieved by the integrative analysis of genotype and phenotype. We aim to explore the BA phenotypic features and to delineate the source of its variance. METHODS: The study is a cross-sectional observational study collating with case/control association analysis...
April 17, 2017: BMC Medical Genomics
https://www.readbyqxmd.com/read/28411962/epidemiology-and-pathophysiology-of-malignancy-in-common-variable-immunodeficiency
#4
REVIEW
A Tak Manesh, G Azizi, A Heydari, F Kiaee, M Shaghaghi, N Hossein-Khannazer, R Yazdani, H Abolhassani, A Aghamohammadi
Common variable immunodeficiency (CVID) is a diagnostic category of primary immunodeficiency (PID) which may present with heterogeneous disorders including recurrent infections, autoimmunity, granulomatous diseases, lymphoid and other types of malignancies. Generally, the incidence of malignancy in CVID patients is around 1.5-20.7% and usually occurs during the 4th-6th decade of life. Non-Hodgkin lymphoma is the most frequent malignancy, followed by epithelial tumours of stomach, breast, bladder and cervix...
April 12, 2017: Allergologia et Immunopathologia
https://www.readbyqxmd.com/read/28400082/immune-dysregulation-in-immunodeficiency-disorders-the-role-of-t-cell-receptor-sequencing
#5
REVIEW
Gabriel K Wong, James M Heather, Sara Barmettler, Mark Cobbold
Immune dysregulation is a prominent feature of primary immunodeficiency disorders, which commonly manifested as autoimmunity, cytopenias and inflammatory bowel disease. In partial T-cell immunodeficiency disorders, it has been proposed that the imbalance between effector and regulatory T-cells drives the breakdown of peripheral tolerance. While there is no robust test for immune dysregulation, the T-cell receptor repertoire is used as a surrogate marker, and has been shown to be perturbed in a number of immunodeficiency disorders featuring immune dysregulation including Omenn's Syndrome, Wiskott-Aldrich Syndrome, and common variable immunodeficiency...
April 8, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28398597/results-from-the-first-in-human-study-with-ozanimod-a-novel-selective-sphingosine-1-phosphate-receptor-modulator
#6
Jonathan Q Tran, Jeffrey P Hartung, Robert J Peach, Marcus F Boehm, Hugh Rosen, Heather Smith, Jennifer L Brooks, Gregg A Timony, Allan D Olson, Sheila Gujrathi, Paul A Frohna
The sphingosine-1-phosphate 1 receptor (S1P1R ) is expressed by lymphocytes, dendritic cells, and vascular endothelial cells and plays a role in the regulation of chronic inflammation and lymphocyte egress from peripheral lymphoid organs. Ozanimod is an oral selective modulator of S1P1R and S1P5R receptors in clinical development for the treatment of chronic immune-mediated, inflammatory diseases. This first-in-human study characterized the safety, pharmacokinetics (PK), and pharmacodynamics (PD) of ozanimod in 88 healthy volunteers using a range of single and multiple doses (7 and 28 days) and a dose-escalation regimen...
April 11, 2017: Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28390597/association-of-airway-abnormalities-with-22q11-2-deletion-syndrome
#7
Rosalba Sacca, Karen B Zur, T Blaine Crowley, Elaine H Zackai, Kathleen D Valverde, Donna M McDonald-McGinn
INTRODUCTION: 22q11.2 deletion syndrome (22q11.2DS) presents with complex but variable symptoms, including cardiac, immune, palatal, endocrine, cognitive, and psychiatric issues. However, an association of 22q11.2DS with structural airway abnormalities has not been formally described. The aim of this study was to document the frequency of this association. METHODS: We retrospectively reviewed medical records of patients with 22q11.2DS evaluated in the 22q and You Center at the Children's Hospital of Philadelphia between 1999 and 2015 referred to otolaryngology for an airway assessment...
May 2017: International Journal of Pediatric Otorhinolaryngology
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
#8
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/28339709/clinicopathologic-correlations-of-renal-pathology-in-the-adult-population-of-poland
#9
Agnieszka Perkowska-Ptasinska, Artur Bartczak, Malgorzata Wagrowska-Danilewicz, Agnieszka Halon, Krzysztof Okon, Aldona Wozniak, Marian Danilewicz, Henryk Karkoszka, Andrzej Marszalek, Jolanta Kowalewska, Andrzej Mroz, Agnieszka Korolczuk, Andrzej Oko, Alicja Debska-Slizien, Beata Naumnik, Zbigniew Hruby, Marian Klinger, Kazimierz Ciechanowski, Marek Myslak, Wladyslaw Sulowicz, Andrzej Rydzewski, Andrzej Wiecek, Jacek Manitius, Tadeusz Gregorczyk, Stanislaw Niemczyk, Michal Nowicki, Ryszard Gellert, Tomasz Stompor, Monika Wieliczko, Krzysztof Marczewski, Leszek Paczek, Olga Rostkowska, Dominika Deborska-Materkowska, Grazyna Bogdanowicz, Andrzej Milkowski, Magdalena Durlik
Background: This is the first report on the epidemiology of biopsy-proven kidney diseases in Poland. Methods: The Polish Registry of Renal Biopsies has collected information on all (n = 9394) native renal biopsies performed in Poland from 2009 to 2014. Patients' clinical data collected at the time of biopsy, and histopathological diagnoses were used for epidemiological and clinicopathologic analysis. Results: There was a gradual increase in the number of native renal biopsies performed per million people (PMP) per year in Poland in 2009-14, starting from 36 PMP in 2009 to 44 PMP in 2014...
April 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28323147/increase-of-circulating-%C3%AE-4%C3%AE-7-conventional-memory-cd4-and-regulatory-t-cells-in-patients-with-common-variable-immunodeficiency-cvid
#10
Karina Mescouto de Melo, Susanne Unger, Baerbel Keller, Sylvia Gutenberger, Ina Stumpf, Sigune Goldacker, Klaus Warnatz
This study investigated whether circulating α4β7(+) expressing T cells could serve as a potential marker for gastrointestinal (GI) disease activity in patients with CVID. The analysis of α4β7(+) T cells in the peripheral blood of 36 patients and 22 healthy donors (HD) revealed increased percentages of α4β7(+) conventional memory CD4 T cells and Tregs, but not among CD8 T-cell populations in patients with CVID compared to HD. No differences between patients with and without chronic or acute GI symptoms were observed...
March 17, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28302512/seroepidemiology-of-hepatitis-a-hepatitis-b-and-varicella-virus-in-people-living-with-hiv-in-ireland
#11
Corinna Sadlier, Anna O'Rourke, Alison Carr, Colm Bergin
Epidemiological studies investigating seroprevalence of vaccine preventable infections at both individual and population level are important in guiding screening and vaccination practices. Data on seroprevalence of common vaccine preventable infections in HIV-infected individuals is lacking. We carried out a retrospective cohort study to investigate serological immunity and factors associated with immunity to hepatitis A virus (HAV), hepatitis B virus (HBV) and varicalla virus (VZV) in a cohort of HIV-infected individuals attending a large ambulatory HIV specialist centre in Ireland...
March 13, 2017: Journal of Infection and Public Health
https://www.readbyqxmd.com/read/28254202/predictors-of-granulomatous-lymphocytic-interstitial-lung-disease-in-common-variable-immunodeficiency
#12
Stella Hartono, Megan S Motosue, Shakila Khan, Vilmarie Rodriguez, Vivek N Iyer, Rohit Divekar, Avni Y Joshi
BACKGROUND: A subset of patients with common variable immunodeficiency (CVID) develop granulomatous lymphocytic interstitial lung disease (GLILD), which is associated with early mortality. OBJECTIVE: To determine a set of clinical and/or laboratory parameters that correlate with GLILD. METHODS: A retrospective, nested case-control (patients with CVID diagnosed with GLILD compared with patients with CVID without a diagnosis of GLILD) medical record review was undertaken at Mayo Clinic, Rochester, MN...
February 18, 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28240614/diagnostic-and-therapeutic-considerations-in-patients-with-hypogammaglobulinemia-after-rituximab-therapy
#13
Ruba Kado, Georgiana Sanders, W Joseph McCune
PURPOSE OF REVIEW: There are no established guidelines for evaluating and treating hypogammaglobulinemia in patients with rheumatic disease who receive B-cell depleting agents. The purpose of this article is to review findings in the work-up and treatment of common variable immunodeficiency (CVID) that can guide our evaluation of patients with autoimmune disease who develop hypogammaglobulinemia after rituximab/B-cell depleting therapy. RECENT FINDINGS: Infection rates are higher in rheumatic disease patients who develop hypogammaglobulinemia than those who do not...
May 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28222802/counts-of-bovine-monocyte-subsets-prior-to-calving-are-predictive-for-postpartum-occurrence-of-mastitis-and-metritis
#14
Brianna Pomeroy, Anja Sipka, Jamal Hussen, Melanie Eger, Ynte Schukken, Hans-Joachim Schuberth
The heightened susceptibility to infectious diseases in postpartum dairy cows is often attributed to immune dysfunction associated with the transition period. However, the cell populations involved in this immune dysfunction and the dynamics between those populations are not well defined. Monocytes play a crucial role in governing initial immune response in bacterial infections. Bovine monocytes are subdivided in classical (CD14(+)/CD16(-)), intermediate (CD14(+)/CD16(+)) and non-classical monocytes (CD14(-)/CD16(+)) with distinct phenotypic and functional differences...
February 21, 2017: Veterinary Research
https://www.readbyqxmd.com/read/28212436/antibody-deficiency-in-patients-with-frequent-exacerbations-of-chronic-obstructive-pulmonary-disease-copd
#15
Brian N McCullagh, Alejandro P Comellas, Zuhair K Ballas, John D Newell, M Bridget Zimmerman, Antoine E Azar
Chronic Obstructive Pulmonary Disease is the third leading cause of death in the US, and is associated with periodic exacerbations, which account for the largest proportion of health care utilization, and lead to significant morbidity, mortality, and worsening lung function. A subset of patients with COPD have frequent exacerbations, occurring 2 or more times per year. Despite many interventions to reduce COPD exacerbations, there is a significant lack of knowledge in regards to their mechanisms and predisposing factors...
2017: PloS One
https://www.readbyqxmd.com/read/28198725/adult-pneumococcal-vaccination-advances-impact-and-unmet-needs
#16
Ricardo J José, Jeremy S Brown
PURPOSE OF REVIEW: Preventing pneumonia in the elderly and individuals with comorbidities is an unmet clinical need. Streptococcus pneumoniae is the commonest bacterial cause of pneumonia, and we summarize recent findings regarding current S. pneumoniae vaccines, and debate their efficacy and cost-effectiveness in risk groups. We also discuss potential future vaccine strategies such as protein antigen vaccines. RECENT FINDINGS: Current vaccination with pneumococcal polysaccharide vaccine does not prevent S...
May 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28192579/infectious-diseases-and-immunizations-in-international-adoption
#17
Emily Obringer, Linda Walsh
Children who are adopted internationally have an increased risk of infectious diseases due to endemic conditions and variable access to preventive health care, such as vaccines, in their country of origin. Pediatricians and other providers who care for children should be familiar with the recommended screening for newly arrived international adoptees. Testing for gastrointestinal pathogens, tuberculosis, hepatitis, syphilis, and HIV should be routinely performed. Other endemic diseases and common skin infections may need to be assessed...
February 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28192146/autoimmune-and-inflammatory-manifestations-occur-frequently-in-patients-with-primary-immunodeficiencies
#18
Alain Fischer, Johan Provot, Jean-Philippe Jais, Alexandre Alcais, Nizar Mahlaoui
BACKGROUND: Primary immunodeficiencies (PIDs) are inherited diseases associated with a considerable increase in susceptibility to infections. It is known that PIDs can also predispose to cancer and immune diseases, including allergy, autoimmunity, and inflammation. OBJECTIVE: We aimed at determining the incidence of autoimmunity and inflammation in patients with PIDs. METHODS: We have retrospectively screened 2183 consecutive cases of PID in the Centre de Référence Déficits Immunitaires Héréditaires registry (CEREDIH; the French national PID registry) for the occurrence of autoimmunity and inflammation...
February 10, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28188718/-hematopoietic-stem-cells-transplant-in-patients-with-common-variable-immunodeficiency-is-a-therapeutic-option
#19
Julio César Cambray-Gutiérrez, Diana Andrea Herrera-Sánchez, Patricia López-Pérez, Aurora Chávez-García, Marco Antonio Yamazaki-Nakashimada
BACKGROUND: Patients with common variable immunodeficiency show higher incidence of sinopulmonary and gastrointestinal infections, as well as lymphoproliferative and autoimmune diseases. The treatment of choice is replacement therapy with human gamma-globulin. Hematopoietic stem cell transplantation is a non-conventional therapeutic modality. CASE REPORT: Twenty-six-year old woman with no family or hereditary history of primary immune deficiencies or consanguinity, with repeated episodes of otitis, sinusitis, gastroenteritis and bronchitis since childhood...
January 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/28160307/treatment-of-presumptive-primary-immune-mediated-thrombocytopenia-with-mycophenolate-mofetil-versus-cyclosporine-in-dogs
#20
F O Cummings, S A Rizzo
OBJECTIVES: The objective of this study was to compare hospitalisation duration, survival times, adverse events and cost of therapy in dogs with presumptive primary immune-mediated thrombocytopenia undergoing therapy with mycophenolate mofetil and corticosteroids versus cyclosporine and corticosteroids. METHODS: A retrospective study of medical case records of dogs with presumed primary immune-mediated thrombocytopenia was conducted. Data collected included signalment, presenting complaints, haematologic and biochemical profiles, vector-borne disease testing, thoracic and abdominal radiographs, abdominal ultrasound, medications administered, duration of hospitalisation, 30- and 60-day survival, adverse events and cost of therapy...
February 2017: Journal of Small Animal Practice
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