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Primary Immunodeficiency

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https://www.readbyqxmd.com/read/29345621/the-circulating-t-helper-subsets-and-regulatory-t-cells-in-patients-with-common-variable-immunodeficiency-with-no-known-monogenic-disease
#1
G Azizi, A Mirshafiey, H Abolhassani, R Yazdani, F J Ansariha, M Shaghaghi, S S Mortazavi-Jahromi, F Noorbakhsh, N Rezaei, A Aghamohammadi
BACKGROUND: Common variable immunodeficiency (CVID) is the most common symptomatic primary immunodeficiency (PID), characterized by heterogeneous clinical manifestations and defects in B- and T- cells. In the present study, we investigated the T helper (Th) cell subsets and regulatory T (Treg) cells, and their related cytokines and transcription factors in the CVID patients with no definite genetic diagnosis. METHODS: The study population comprised 13 CVID patients and 13 healthy controls (HC)...
January 18, 2018: Journal of Investigational Allergology & Clinical Immunology
https://www.readbyqxmd.com/read/29345341/novel-insights-into-fas-defects-underlying-autoimmune-lymphoproliferative-syndrome-revealed-by-studies-in-consanguineous-patients
#2
REVIEW
Imen Ben-Mustapha, Nourhen Agrebi, Mohamed-Ridha Barbouche
Autoimmune lymphoproliferative syndrome (ALPS) is a primary immunodeficiency disease due to impaired Fas-Fas ligand apoptotic pathway. It is characterized by chronic nonmalignant, noninfectious lymphadenopathy and/or splenomegaly associated with autoimmune manifestations primarily directed against blood cells. Herein, we review the heterogeneous ALPS molecular bases and discuss recent findings revealed by the study of consanguineous patients. Indeed, this peculiar genetic background favored the identification of a novel form of AR ALPS-FAS associated with normal or residual protein expression, expanding the spectrum of ALPS types...
December 27, 2017: Journal of Leukocyte Biology
https://www.readbyqxmd.com/read/29344211/prevalence-of-hpv-infection-among-hiv-positive-and-hiv-negative-women-in-central-eastern-italy-strategies-of-prevention
#3
Edoardo Tartaglia, Katia Falasca, Jacopo Vecchiet, Giovanna Paola Sabusco, Giovanna Picciano, Roberto Di Marco, Claudio Ucciferri
The present cross-sectional-study aimed to determine the prevalence of human papillomavirus (HPV)-genotypes among human immunodeficiency virus (HIV)-positive and -negative women in Central/Eastern Italy, and to identify the optimal strategies for effective HPV-prevention in each group. A representative sample of HIV-negative (150/200) and -positive (50/200) women, who underwent cervico-vaginal-swabbing. Swabs were analysed for a cytological screening and for a HPV-DNA-genotyping-test. A total of 66/200 swabs resulted HPV-positive...
December 2017: Oncology Letters
https://www.readbyqxmd.com/read/29339125/prevalence-and-outcomes-of-primary-immunodeficiency-in-hospitalized-children-in-the-united-states
#4
Zachary Rubin, Andrea Pappalardo, Alan Schwartz, James W Antoon
BACKGROUND: Primary immunodeficiency diseases (PIDDs) are rare yet life-threatening chronic conditions in children. The prevalence and outcomes of PIDDs in the pediatric population in the United States are not well understood. OBJECTIVE: The objectives of this study were to (1) determine the epidemiology of children hospitalized with PIDD in the United States and (2) characterize the clinical outcomes of hospitalized children with PIDDs. METHODS: Retrospective cohort analysis of the 2003-2012 Kids' Inpatient Database of children aged 2-18 years admitted with a primary or secondary diagnosis code of PIDD was performed...
January 12, 2018: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/29337163/utility-of-ultra-deep-sequencing-for-detection-of-varicella-zoster-virus-antiviral-resistance-mutations
#5
Mélanie Mercier-Darty, David Boutolleau, Raphaël Lepeule, Christophe Rodriguez, Sonia Burrel
We report the first application of ultra-deep sequencing (UDS) to varicella-zoster virus (VZV) genotypic antiviral testing in a case of acyclovir-resistant VZV infection initially detected by Sanger sequencing within a deeply immunocompromised heart transplant recipient. As added-value compared to Sanger analysis, UDS revealed complex dynamics of viral population under antiviral pressure. Varicella-zoster virus (VZV) is a ubiquitous human herpesvirus affecting populations worldwide. VZV is commonly acquired in youth whose primary infection usually manifests as benign varicella (chickenpox)...
January 11, 2018: Antiviral Research
https://www.readbyqxmd.com/read/29335887/sphingadienes-show-therapeutic-efficacy-in-neuroblastoma-in-vitro-and-in-vivo-by-targeting-the-akt-signaling-pathway
#6
Piming Zhao, Ana E Aguilar, Joanna Y Lee, Lucy A Paul, Jung H Suh, Latika Puri, Meng Zhang, Jennifer Beckstead, Andrzej Witkowski, Robert O Ryan, Julie D Saba
Neuroblastoma is a childhood malignancy that accounts for approximately 15% of childhood cancer deaths. Only 20-35% of children with metastatic neuroblastoma survive with standard therapy. Identification of more effective therapies is essential to improving the outcome of children with high-stage disease. Sphingadienes (SD) are growth-inhibitory sphingolipids found in natural sources including soy. They exhibit chemopreventive activity in mouse models of colon cancer, where they mediate cytotoxicity by inhibiting key pro-carcinogenic signaling pathways...
January 16, 2018: Investigational New Drugs
https://www.readbyqxmd.com/read/29333106/which-point-of-care-tests-would-be-most-beneficial-to-add-to-clinical-practice-findings-from-a-survey-of-3-family-medicine-clinics-in-the-united-states
#7
Victoria Hardy, William Alto, Gina A Keppel, Laura-Mae Baldwin, Matthew Thompson
Background: Point-of-care tests (POCTs) are increasingly used in family medicine to facilitate screening, diagnosis, monitoring, treatment, and referral decisions for a variety of conditions. Point-of-care tests that clinicians believe might be beneficial to add to clinical practice and the conditions for which they would be most useful in family medicine remain poorly understood in the United States. Methods: Forty-two clinicians at 3 family medicine residency clinics completed a brief survey asking which POCTs they believed would be beneficial to add to their clinical practice and the conditions POCTs would be most useful for...
December 2017: Point of Care
https://www.readbyqxmd.com/read/29332326/carcinogenic-potential-of-antitumor-therapies-is-the-risk-predictable
#8
Ivanka Nenova, Janet Grudeva-Popova
The growing number of successfully cured cancer patients has created a new field in oncogenesis. The life expectancy of such patients has increased, however this favorable event may create enough time for epigenetic events to occur which can cause a new carcinognic event, i.e. a secondary malignancy. The terms in use are second primary malignancies as well as therapy-related neoplasms in case the treatment of the first neoplasm is a direct cause. Second primary malignancies can be hematological neoplasms or solid tumors, with solid tumors having higher frequency...
November 2017: Journal of B.U.ON.: Official Journal of the Balkan Union of Oncology
https://www.readbyqxmd.com/read/29327783/nonsurgical-management-of-human-immunodeficiency-virus-associated-parotid-cysts-a-systematic-review-and-meta-analysis
#9
Akash N Naik, William B Clinkscales, Masanari G Kato, Shaun A Nguyen, M Boyd Gillespie
BACKGROUND: The purpose of this clinical review was to analyze the effectiveness of nonsurgical management options for human immunodeficiency virus (HIV)-associated parotid cysts. METHODS: We conducted systematic and meta-analysis reviews. Primary outcomes were complete or partial responses. RESULTS: Systematic review identified 12 relevant studies. The average rates of complete response for antiretroviral therapy (ART), sclerotherapy, and fine-needle aspiration (FNA) were 52...
January 12, 2018: Head & Neck
https://www.readbyqxmd.com/read/29325543/persistent-mammalian-orthoreovirus-coxsackievirus-and-adenovirus-co-infection-in-a-child-with-a-primary-immunodeficiency-detected-by-metagenomic-sequencing-a-case-report
#10
Dagmara W Lewandowska, Riccarda Capaul, Seraina Prader, Osvaldo Zagordi, Fabienne-Desirée Geissberger, Martin Kügler, Marcus Knorr, Christoph Berger, Tayfun Güngör, Janine Reichenbach, Cyril Shah, Jürg Böni, Andrea Zbinden, Alexandra Trkola, Jana Pachlopnik Schmid, Michael Huber
BACKGROUND: We report a rare case of Mammalian orthoreovirus (MRV) infection in a child with a primary immunodeficiency (PID). Infections with Mammalian orthoreovirus are very rare and probably of zoonotic origin. Only a few cases have been described so far, including one with similar pathogenesis as in our case. CASE PRESENTATION: The patient, age 11, presented with flu-like symptoms and persistent severe diarrhea. Enterovirus has been detected over several months, however, exact typing of a positive cell culture remained inconclusive...
January 11, 2018: BMC Infectious Diseases
https://www.readbyqxmd.com/read/29325248/-survival-of-patients-with-primary-central-nervous-system-diffuse-large-b-cell-lymphoma-impact-of-gene-aberrations-and-protein-overexpression-of-bcl-2-and-c-myc-and-selection-of-chemotherapy-regimens
#11
W J Yin, X Zhu, H Y Yang, W Y Sun, M J Wu
Objective: To investigate the impact of clinicopathological features, gene rearrangements and protein expression of bcl-6, bcl-2, C-MYC and chemotherapy regime on the prognosis of patients with primary central nervous system diffuse large B-cell lymphoma (PCNS-DLBCL). Methods: Thirty-three cases of PCNS-DLBCL diagnosed from January 2006 to December 2016 at Zhejiang Cancer Hospital were collected. The expression of CD10, bcl-6, bcl-2, MUM1 and MYC were detected by immunohistochemical staining (IHC). The presence of EB virus was detected by in situ hybridization(EBER)...
January 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29320464/sti-knowledge-in-berlin-adolescents
#12
Frederik Tilmann von Rosen, Antonella Juline von Rosen, Falk Müller-Riemenschneider, Inken Damberg, Peter Tinnemann
Sexually transmitted infections (STIs) pose a significant threat to individual and public health. They disproportionately affect adolescents and young adults. In a cross-sectional study, we assessed self-rated and factual STI knowledge in a sample of 9th graders in 13 secondary schools in Berlin, Germany. Differences by age, gender, migrant background, and school type were quantified using bivariate and multivariable analyses. A total of 1177 students in 61 classes participated. The mean age was 14.6 (SD = 0...
January 10, 2018: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/29317678/peptidomimetic-blockade-of-myb-in-acute-myeloid-leukemia
#13
Kavitha Ramaswamy, Lauren Forbes, Gerard Minuesa, Tatyana Gindin, Fiona Brown, Michael G Kharas, Andrei V Krivtsov, Scott A Armstrong, Eric Still, Elisa de Stanchina, Birgit Knoechel, Richard Koche, Alex Kentsis
Aberrant gene expression is a hallmark of acute leukemias. MYB-driven transcriptional coactivation with CREB-binding protein (CBP)/P300 is required for acute lymphoblastic and myeloid leukemias, including refractory MLL-rearranged leukemias. Using structure-guided molecular design, we developed a peptidomimetic inhibitor MYBMIM that interferes with the assembly of the molecular MYB:CBP/P300 complex and rapidly accumulates in the nuclei of AML cells. Treatment of AML cells with MYBMIM led to the dissociation of the MYB:CBP/P300 complex in cells, its displacement from oncogenic enhancers enriched for MYB binding sites, and downregulation of MYB-dependent gene expression, including of MYC and BCL2 oncogenes...
January 9, 2018: Nature Communications
https://www.readbyqxmd.com/read/29316919/use-of-an-electronic-medical-record-reminder-improves-hiv-screening
#14
Colleen Kershaw, Jessica L Taylor, Gary Horowitz, Diane Brockmeyer, Howard Libman, Gila Kriegel, Long Ngo
BACKGROUND: More than 1 in 7 patients with human immunodeficiency virus (HIV) infection in the United States are unaware of their serostatus despite recommendations of US agencies that all adults through age 65 be screened for HIV at least once. To facilitate universal screening, an electronic medical record (EMR) reminder was created for our primary care practice. Screening rates before and after implementation were assessed to determine the impact of the reminder on screening rates...
January 10, 2018: BMC Health Services Research
https://www.readbyqxmd.com/read/29310365/ebv-lymphoproliferative-associated-disease-and-primary-cardiac-t-cell-lymphoma-in-a-stk4-deficient-patient-a-case-report
#15
Roya Sherkat, Mohammad Reza Sabri, Bahar Dehghan, Hamid Bigdelian, Nahid Reisi, Nooshin Afsharmoghadam, Hamid Rahimi, Narges Rahmanian, Cristoph Klein
RATIONALE: Primary cardiac lymphoma (PLC) is an extremely uncommon malignancy. PCL is more common in secondary immunodeficient patients. In this report, we describe a unique case of PLC who had been diagnosed as a STK4 deficient patient. This case is the first Primary immunodeficiency (PID) patient developing PCL in the world. PATIENT CONCERNS: An eleven-year-old girl, a known case of PID, was referred to the pediatric cardiology department because of chest pain and dyspnea...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29307770/a-young-girl-with-severe-cerebral-fungal-infection-due-to-card-9-deficiency
#16
Pinar Gur Cetinkaya, Deniz Cagdas Ayvaz, Betül Karaatmaca, Rahsan Gocmen, Figen Söylemezoğlu, Wayne Bainter, Janet Chou, Talal A Chatila, Ilhan Tezcan
Pattern recognition receptors (PRRs), receptors of the innate immune system, are important in interaction with pathogens. Caspase Recruitment Domain-containing protein 9 (CARD9), a member of PRRs, is an intracellular adaptor protein important in fungal defense. CARD9 deficiency causes a rare primary immunodeficiency (PID) characterized by superficial and deep fungal infections. We report a 17year-old female with a homozygous nonsense mutation in CARD9, who presented with severe cerebral fungal infection of the central nervous system...
January 4, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29307028/exercise-perception-and-behaviors-in-individuals-living-with-primary-immunodeficiency-disease
#17
Kerri L Sowers, Bini A Litwin, Alan C W Lee, Mary Lou A Galantino
BACKGROUND: Routine exercise has been established as an effective way to improve overall health. The value of exercise has been established in many diseases, however, there are no studies investigating the impact of exercise for individuals with primary immunodeficiency disease (PID). The purpose of this study was to investigate exercise perceptions and behaviors in individuals diagnosed with PID. METHODS: An online survey was distributed over a four-week period...
January 6, 2018: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29306672/the-bronchiectasis-severity-index-and-faced-score-for-assessment-of-the-severity-of-bronchiectasis
#18
J C Costa, J N Machado, C Ferreira, J Gama, C Rodrigues
INTRODUCTION: Bronchiectasis (BC) is a multidimensional and etiologically diverse disease and, therefore, no single parameter can be used to determine its overall severity and prognosis. In this regard, two different validated scores are currently used to assess the severity of non-cystic fibrosis bronchiectasis (NCFB): the FACED score and the Bronchiectasis Severity Index (BSI). OBJECTIVE: To describe the etiology of NCFB and compare the results of the assessment of NCFB severity obtained via FACED and BSI scores...
January 3, 2018: Revista Portuguesa de Pneumologia
https://www.readbyqxmd.com/read/29306654/humoral-immune-response-to-measles-and-varicella-vaccination-in-former-very-low-birth-weight-preterm-infants
#19
Carolina Schlindwein Mariano Ferreira, Maria Cristina Abrão Aued Perin, Maria Isabel de Moraes-Pinto, Raquel Maria Simão-Gurge, Ana Lucia Goulart, Lily Yin Weckx, Amélia Miyashiro Nunes Dos Santos
INTRODUCTION: Immune response to vaccination in infants born prematurely may be lower than in infants born at full-term. Some clinical factors might be associated with humoral immune response. OBJECTIVES: The objectives of this study were to compare the immune response to measles and varicella vaccination in infants born prematurely with those born at full-term and to analyze factors associated with measles and varicella antibody levels. METHODS: Prospective study including two groups of infants aged 12 months...
January 4, 2018: Brazilian Journal of Infectious Diseases
https://www.readbyqxmd.com/read/29306566/bispecific-chimeric-antigen-receptors-targeting-the-cd4-binding-site-and-high-mannose-glycans-of-gp120-optimized-for-anti-human-immunodeficiency-virus-potency-and-breadth-with-minimal-immunogenicity
#20
Mustafa H Ghanem, Sara Bolivar-Wagers, Barna Dey, Agnes Hajduczki, Diego A Vargas-Inchaustegui, David T Danielson, Virgilio Bundoc, Li Liu, Edward A Berger
BACKGROUND AIMS: Chimeric antigen receptors (CARs) offer great potential toward a functional cure of human immunodeficiency virus (HIV) infection. To achieve the necessary long-term virus suppression, we believe that CARs must be designed for optimal potency and anti-HIV specificity, and also for minimal probability of virus escape and CAR immunogenicity. CARs containing antibody-based motifs are problematic in the latter regard due to epitope mutation and anti-idiotypic immune responses against the variable regions...
January 3, 2018: Cytotherapy
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