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Primary immune deficiency

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https://www.readbyqxmd.com/read/27922232/changing-epidemiology-of-non-cystic-fibrosis-bronchiectasis
#1
Semiha Bahçeci, Sait Karaman, Hikmet Tekin Nacaroğlu, Selçuk Yazıcı, Saniye Girit, Şule Ünsal-Karkıner, Demet Can
Non-cystic fibrosis bronchiectasis again becomes a major health problem due to inappropriate antibiotic use and increasing frequency of protracted bacterial bronchitis. The aim was to determine the changes in etiology of bronchiectasis. Patients who admitted to Behçet Uz Children Hospital between 2005 and 2015 (n=110) were retrospectively examined. The etiology of bronchiectasis was detected as; primary ciliary dyskinesia 26.4%, protracted bacterial bronchitis 22.8%, primary immune deficiency 11.8%, bronchiolitis obliterans 8...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27918258/prolonged-excretion-of-type-2-poliovirus-from-a-primary-immune-deficient-patient-during-the-transition-to-a-type-2-poliovirus-free-world-israel-2016
#2
Merav Weil, Lester M Shulman, Sophia Heiman, Tali Stauber, Jacqueline Alfandari, Leah Weiss, Ilana Silberstein, Viki Indenbaum, Ella Mendelson, Danit Sofer
Wild poliovirus type-2 has been eradicated, use of live type-2 vaccine has been terminated globally, and all type-2 polioviruses are under strict laboratory containment protocols. Re-emergence may arise from prolonged asymptomatic excretion of poliovirus by hospitalised primary immune deficient (PID) patients, as described here, through repeated exposure of close contacts to high titres of infected material. At this transition time, PID patients should be screened and hospital containment protocols updated in parallel with laboratory containment...
November 24, 2016: Euro Surveillance: Bulletin Européen sur les Maladies Transmissibles, European Communicable Disease Bulletin
https://www.readbyqxmd.com/read/27914961/expanding-the-clinical-and-genetic-spectrum-of-g6pd-deficiency-the-occurrence-of-bcgitis-and-novel-missense-mutation
#3
Taj Ali Khan, Humaira Mazhar, Mehboob Nawaz, Kalsoom Kalsoom, Muhammad Ishfaq, Huma Asif, Hazir Rahman, Muhammad Qasim, Farkhanda Naz, Mubashir Hussain, Baharullah Khattak, Waheed Ullah, Otavio Cabral-Marques, Jawad Butt, Asif Iqbal
Glucose-6-phosphate dehydrogenase (G6PD) is a key enzyme in the pentose phosphate pathway that ensures sufficient production of coenzyme nicotinamide adenine dinucleotide phosphate (NADPH) by catalyzing the reduction of NADP+ to NADPH. Noteworthy, the latter mediates the production of reactive oxygen species (ROS) by phagocytic cells such as neutrophils and monocytes. Therefore, patients with severe forms of G6PD deficiency may present impaired NADPH oxidase activity and become susceptible to recurrent infections...
November 30, 2016: Microbial Pathogenesis
https://www.readbyqxmd.com/read/27913461/primary-immune-deficiencies-with-defects-in-neutrophil-function
#4
Mary C Dinauer
Immune deficiencies resulting from inherited defects in neutrophil function have revealed important features of the innate immune response. Although sharing an increased susceptibility to bacterial and fungal infections, these disorders each have distinctive features in their clinical manifestations and characteristic microbial pathogens. This review provides an update on several genetic disorders with impaired neutrophil function, their pathogenesis, and treatment strategies. These include chronic granulomatous disease, which results from inactivating mutations in the superoxide-generating nicotinamide dinucleotide phosphate oxidase...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27889323/-primary-immunodeficiency-and-autoimmunity
#5
A Guffroy, V Gies, M Martin, A-S Korganow
Many evidences highlight that immunodeficiency and autoimmunity are two sides of a same coin. Primary immune deficiencies (PIDs), which are rare mono- or multigenic defects of innate or adaptative immunity, frequently associate with autoimmunity. Analyses of single-gene defects in immune pathways of families with PIDs, by new tools of molecular biology (next genome sequencing technologies), allowed a better understanding of the ways that could both drive immune defect with immune deficiency and autoimmunity...
November 23, 2016: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/27885757/role-of-primary-t-cell-immunodeficiency-and-hepatitis-b-coinfection-on-spontaneous-clearance-of-hepatitis-c-the-bc-hepatitis-testers-cohort
#6
N Islam, M Krajden, M Gilbert, P Gustafson, A Yu, M Kuo, M Chong, M Alvarez, J Wong, M W Tyndall, N Z Janjua
T-cell host immune response against hepatitis C virus (HCV) has been suggested to play an important role in determining HCV infection outcome. However, data from human studies are not available. This study examined the effect of primary T-cell deficiency along with other factors on the spontaneous clearance of HCV in a large population-based cohort in British Columbia, Canada. The BC Hepatitis Testers Cohort includes all individuals tested for HCV in BC in 1990-2013 linked with data on their medical visits, hospitalizations and prescription drugs...
November 25, 2016: Journal of Viral Hepatitis
https://www.readbyqxmd.com/read/27884454/-management-of-adverse-effects-related-to-human-immunoglobulin-therapy-recommendations-for-clinical-practice
#7
I Marie, P Chérin, M Michallet, E Pelus, J Dantal, J-C Crave, J-C Delain, J-F Viallard
Both intravenous and subcutaneous immunoglobulins are therapeutic modalities approved in various conditions, including primary and secondary immune deficiencies and autoimmune disorders. To date, immunoglobulins have more often been considered as a safe medication, with minor adverse effects such as hypertension, fever and chills, nausea, myalgia or headache. However, with the wider use of immunoglobulins in the treatment of autoimmune diseases, severe side effects have also been reported to occur in immunoglobulin-treated patients, especially anaphylaxis, aseptic meningitis, acute renal impairment, thrombotic events as well as haematological manifestations...
November 21, 2016: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/27880797/regulation-and-maintenance-of-an-adoptive-t-cell-dependent-memory-b-cell-pool
#8
Marie Anson, Inês Amado, Marie-Pierre Mailhé, Emmanuel Donnadieu, Sylvie Garcia, François Huetz, Antonio A Freitas
We investigated the ability of monoclonal B cells to restore primary and secondary T-cell dependent antibody responses in adoptive immune-deficient hosts. Priming induced B cell activation and expansion, AID expression, antibody production and the generation of IgM+IgG- and IgM-IgG+ antigen-experienced B-cell subsets that persisted in the lymphopenic environment by cell division. Upon secondary transfer and recall the IgM-IgG+ cells responded by the production of antigen-specific IgG while the IgM+ memory cells secreted mainly IgM and little IgG, but generated new B cells expressing germinal center markers...
2016: PloS One
https://www.readbyqxmd.com/read/27876685/efficacy-of-mobile-serious-games-in-increasing-hiv-risk-perception-in-swaziland-a-randomized-control-trial-sgprev-trial-research-protocol
#9
Bhekumusa Wellington Lukhele, Patou Musumari, Christina El-Saaidi, Teeranee Techasrivichien, S Pilar Suguimoto, Masako Ono Kihara, Masahiro Kihara
BACKGROUND: The human immunodeficiency virus (HIV) and acquired immune deficiency syndrome (AIDS) continue to be a major public health problem in Sub-Saharan Africa (SSA), particularly in Swaziland, which has the highest HIV prevalence in this region. A wide range of strategies and interventions have been used to promote behavior change, though almost all such interventions have involved mass media. Therefore, innovative behavior change strategies beyond mass media communication are urgently needed...
November 22, 2016: JMIR Research Protocols
https://www.readbyqxmd.com/read/27873456/visceral-leishmaniasis-in-two-patients-with-il-12p40-and-il-12r%C3%AE-1-deficiencies
#10
Nima Parvaneh, Vincent Barlogis, Abdolvahab Alborzi, Caroline Deswarte, Stéphanie Boisson-Dupuis, Mélanie Migaud, Catherine Farnaria, Janet Markle, Leila Parvaneh, Jean-Laurent Casanova, Jacinta Bustamante
Mutations of the IL12B and IL12RB1 genes underlie the development of IL-12 p40 and IL-12Rβ1 deficiencies, respectively, both of which cause predisposition to infection with weakly virulent mycobacteria and Salmonella. Infections with other intramacrophagic organisms have only been rarely observed. We identified two patients with visceral leishmaniasis who had autosomal recessive IL-12 p40 and IL-12Rβ1 deficiencies, respectively. This finding demonstrates the importance of IFN-γ immunity in the control of leishmaniasis...
November 22, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27873163/an-update-on-the-use-of-immunomodulators-in-primary-immunodeficiencies
#11
REVIEW
Pandiarajan Vignesh, Amit Rawat, Surjit Singh
The genomic revolution in the past decade fuelled by breathtaking advances in sequencing technologies has defined several new genetic diseases of the immune system. Many of these newly characterized diseases are a result of defects in genes involved in immune regulation. The discovery of these diseases has opened a vista of new therapeutic possibilities. Immunomodulatory agents, a hitherto unexplored therapeutic option in primary immunodeficiency diseases have been tried in a host of these newly described maladies...
November 21, 2016: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/27869591/vertebrate-host-susceptibility-to-heartland-virus
#12
Angela M Bosco-Lauth, Amanda E Calvert, J Jeffrey Root, Tom Gidlewski, Brian H Bird, Richard A Bowen, Atis Muehlenbachs, Sherif R Zaki, Aaron C Brault
Heartland virus (HRTV) is a recently described phlebovirus initially isolated in 2009 from 2 humans who had leukopenia and thrombocytopenia. Serologic assessment of domestic and wild animal populations near the residence of 1 of these persons showed high exposure rates to raccoons, white-tailed deer, and horses. To our knowledge, no laboratory-based assessments of viremic potential of animals infected with HRTV have been performed. We experimentally inoculated several vertebrates (raccoons, goats, chickens, rabbits, hamsters, C57BL/6 mice, and interferon-α/β/γ receptor-deficient [Ag129]) mice with this virus...
December 2016: Emerging Infectious Diseases
https://www.readbyqxmd.com/read/27864558/prospective-study-of-the-innate-cellular-immune-response-in-low-vaccine-responder-children
#13
Naveen Surendran, Ted Nicolosi, Ravinder Kaur, Matthew Morris, Michael Pichichero
We recently reported our findings from a longitudinal, prospective study where we identified 10% infants who were low vaccine responders (LVR) at age 9-12 mo following routine primary series vaccine schedule. We found multiple cellular deficiencies in LVR children, including low number of memory B cells, reduced polyclonal stimulation of naïve/memory T cell response and suboptimal APC response. These children outgrew their poor vaccine response by the time they received booster doses of vaccine. Studies in human infant innate immunity are rare because of the unique challenges in specimen collection...
November 18, 2016: Innate Immunity
https://www.readbyqxmd.com/read/27857838/hyponatraemia-secondary-to-nivolumab-induced-primary-adrenal-failure
#14
Harris Trainer, Paul Hulse, Claire E Higham, Peter Trainer, Paul Lorigan
: Checkpoint inhibitors, such as ipilimumab and pembrolizumab, have transformed the prognosis for patients with advanced malignant melanoma and squamous non-small-cell lung cancer, and their use will only expand as experience is gained in a variety of other malignancies, for instance, renal and lymphoma. As the use of checkpoint inhibitors increases, so too will the incidence of their unique side effects, termed immune-related adverse events (irAEs), which can affect dermatological, gastrointestinal, hepatic, endocrine and other systems...
2016: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/27857715/complementary-roles-of-the-classical-and-lectin-complement-pathways-in-the-defense-against-aspergillus-fumigatus
#15
Anne Rosbjerg, Ninette Genster, Katrine Pilely, Mikkel-Ole Skjoedt, Gregory L Stahl, Peter Garred
Aspergillus fumigatus infections are associated with a high mortality rate for immunocompromised patients. The complement system is considered to be important in protection against this fungus, yet the course of activation is unclear. The aim of this study was to unravel the role of the classical, lectin, and alternative pathways under both immunocompetent and immunocompromised conditions to provide a relevant dual-perspective on the response against A. fumigatus. Conidia (spores) from a clinical isolate of A...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27853628/tir-domain-containing-adapter-inducing-interferon-%C3%AE-trif-regulates-cxcr5-t-helper-cells-in-the-intestine
#16
Saravana Kanagavelu, Claudia Flores, Shinichiro Hagiwara, Jose Ruiz, Jinhee Hyun, Ei E Cho, Frank Sun, Laura Romero, David Q Shih, Masayuki Fukata
OBJECTIVE: Establishing an effective humoral immunity is an important host defense mechanism in intestinal mucosa. T follicular helper (Tfh) cells are a spectrum of CXCR5 expressing T helper cells that induce antigen-specific B cell differentiation. Because the differentiation of T helper cells is largely regulated by innate immunity, we addressed whether TRIF signaling regulates Tfh cell differentiation and its ability to trigger humoral immune responses in the intestine. METHOD: CD4(+)CXCR5(+) T cells, B cells, and plasma cells in the Peyer's patches (PPs) of WT and TRIF-deficient (Trif(LPS2)) mice were analyzed by flow cytometry at the baseline, 9 days post primary infection, and 7 days post-secondary infection with Y...
October 2016: Journal of Clinical & Cellular Immunology
https://www.readbyqxmd.com/read/27849554/systemic-toxoplasma-infection-triggers-a-long-term-defect-in-the-generation-and-function-of-naive-t-lymphocytes
#17
David G Kugler, Francis A Flomerfelt, Diego L Costa, Karen Laky, Olena Kamenyeva, Paul R Mittelstadt, Ronald E Gress, Stephan P Rosshart, Barbara Rehermann, Jonathan D Ashwell, Alan Sher, Dragana Jankovic
Because antigen-stimulated naive T cells either die as effectors or enter the activated/memory pool, continuous egress of new T lymphocytes from thymus is essential for maintenance of peripheral immune homeostasis. Unexpectedly, we found that systemic infection with the protozoan Toxoplasma gondii triggers not only a transient increase in activated CD4(+) Th1 cells but also a persistent decrease in the size of the naive CD4(+) T lymphocyte pool. This immune defect is associated with decreased thymic output and parasite-induced destruction of the thymic epithelium, as well as disruption of the overall architecture of that primary lymphoid organ...
November 14, 2016: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/27836059/primary-immune-deficiencies-in-the-adult-a-previously-underrecognized-common-condition
#18
Elli Rosenberg, Peter B Dent, Judah A Denburg
The large majority of classified primary immune deficiency (PID) diseases present in childhood. Yet, most patients with PID are adults, with a large proportion experiencing onset of symptoms beyond their childhood years. Most of these are diagnosed predominantly with antibody defects, but cellular and other disorders are increasingly being identified in older patients as well. Moreover, advances in clinical immunology are allowing pediatric patients, even those with severe disease, to reach adulthood. Because of differences in the physiology and pathophysiology of children and adults, the presentation, diagnosis, and management of a complex chronic disease could differ significantly between these patient populations and therefore require modifications in approach...
November 2016: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/27836056/vaccination-in-primary-immunodeficiency-disorders
#19
Ali Sobh, Francisco A Bonilla
Immunocompromised patients have increased susceptibility to vaccine-preventable infections. Thus, vaccination is a critical issue in this population. Vaccines are usually classified as live versus inactivated or subunit (nonviable) vaccines. In general, inactivated vaccines are safe in immunocompromised patients and should be given per the routine schedule except when they are unlikely to have any benefit as in severe antibody deficiency or combined immunodeficient patients and patients receiving immunosuppressive therapy or immunoglobulin replacement...
November 2016: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/27836055/lung-disease-in-primary-antibody-deficiencies
#20
REVIEW
Edith Schussler, Mary B Beasley, Paul J Maglione
Primary antibody deficiencies (PADs) are the most common form of primary immunodeficiency and predispose to severe and recurrent pulmonary infections, which can result in chronic lung disease including bronchiectasis. Chronic lung disease is among the most common complications of PAD and a significant source of morbidity and mortality for these patients. However, the development of lung disease in PAD may not be solely the result of recurrent bacterial infection or a consequence of bronchiectasis. Recent characterization of monogenic immune dysregulation disorders and more extensive study of common variable immunodeficiency have demonstrated that interstitial lung disease (ILD) in PAD can result from generalized immune dysregulation and frequently occurs in the absence of pneumonia history or bronchiectasis...
November 2016: Journal of Allergy and Clinical Immunology in Practice
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