Results 21 to 30 of about 468,207 (168)

Assessing the Functional Relevance of Variants in the IKAROS Family Zinc Finger Protein 1 (IKZF1) in a Cohort of Patients With Primary Immunodeficiency

Frontiers in Immunology, 2019
Common variable immunodeficiency (CVID) is the most frequent symptomatic primary immunodeficiency. Patients with CVID are prone to recurrent bacterial infection due to the failure of adequate immunoglobulin production.
Zoya Eskandarian, Zoya Eskandarian, Manfred Fliegauf, Manfred Fliegauf, Alla Bulashevska, Michele Proietti, Rosie Hague, Cristian Roberto Smulski, Desirée Schubert, Klaus Warnatz, Bodo Grimbacher, Bodo Grimbacher, Bodo Grimbacher, Bodo Grimbacher, Bodo Grimbacher   +14 more
doaj   +1 more source

Development of a Disease Activity Score to Assess Treatment Success in Patients with NFKB1 Mutations

Journal of Human Immunity
BackgroundNFKB1 encodes for the p105/p50 nuclear factor-kappa-B (NF-kB1) transcription factors. Heterozygous mutations in NFKB1 may lead to NF-kB1 insufficiency, which may result in a multiorgan disease. Here, we aimed to develop a disease activity score
Katharina Thoma, Pia Hassunah, Manfred Fliegauf, Gabriele Müller, Andres Caballero Garcia de Oteyza, IPavla Mrovecova, Helene Kraus, Prerana Agarwal, Maximilian Frederik Russe, Birgit Sawitzki, Lev Petrov, Katrin Vogt, Nadezhda Nadezhda Camacho Ordonez, Aleksandra Hirsch, Christoph B. Geier, Máté Krausz, Sigune Goldacker, Natalie Frede, Stephan Ehl, Sarah Salou, Ansgar Schulz, Shahrzad Bakhtiar, Maria Fasshauer, Oliver Hausmann, Anja-Dorothee Hüfner, Martin Stanulla, Anna Luijendijk, Faranaz Atschekzei, Georgios Sogkas, Ulrich Salzer, Baerbel Keller, Michele Proietti, Klaus Warnatz, Bodo Grimbacher   +33 more
doaj   +1 more source

Molecular changes associated with increased TNF-α-induced apoptotis in naïve (TN) and central memory (TCM) CD8+ T cells in aged humans

Immunity & Ageing, 2018
Background Progressive T cell decline in aged humans is associated with a deficiency of naïve (TN) and central memory (TCM) T cells. We have previously reported increased Tumor necrosis factor-α (TNF-α)-induced apoptosis in TN and TCM T cells in aged ...
Sudhir Gupta, Houfen Su, Sudhanshu Agrawal, Sastry Gollapudi   +3 more
doaj   +1 more source

Cytomegaloviruses and Macrophages—Friends and Foes From Early on?

Frontiers in Immunology, 2020
Starting at birth, newborn infants are exposed to numerous microorganisms. Adaptation of the innate immune system to them is a delicate process, with potentially advantageous and harmful implications for health development.
Sebastian Baasch, Sebastian Baasch, Zsolt Ruzsics, Philipp Henneke, Philipp Henneke   +4 more
doaj   +1 more source

Case Report: Hemophagocytic Lymphohistiocytosis and Non-Tuberculous Mycobacteriosis Caused by a Novel GATA2 Variant

Frontiers in Immunology, 2021
Hemophagocytic lymphohistiocytosis (HLH) is a disorder of uncontrolled immune activation with distinct clinical features including fever, cytopenia, splenomegaly, and sepsis-like symptoms.
Thomas Mika, Deepak Vangala, Matthias Eckhardt, Paul La Rosée, Christoph Lange, Christoph Lange, Kai Lehmberg, Charlotte Wohlschläger, Saskia Biskup, Ilka Fuchs, Jasmin Mann, Stephan Ehl, Klaus Warnatz, Klaus Warnatz, Roland Schroers   +14 more
doaj   +1 more source

How I Treat: STAT3 hyper IgE syndromeHow I Treat: STAT3 hyper IgE syndrome [PDF]

Journal of Human Immunity
The hyper IgE syndrome (formerly also called Job syndrome or Buckley syndrome) is a rare and syndromic primary immunodeficiency. It is an autosomal-dominant disorder caused by heterozygous dominant-negative mutations in the transcription factor STAT3 ...
Cliodhna Ella Murray, Eyad Jannoud, Seraina Prader, Bodo Grimbacher   +3 more
doaj   +1 more source

Obesity and risk of respiratory tract infections: results of an infection-diary based cohort study

BMC Public Health, 2018
Background Respiratory tract infections (RTIs) are a major morbidity factor contributing largely to health care costs and individual quality of life. The aim of the study was to test whether obesity (BMI ≥ 30 kg/m2) is one of the risk factors underlying ...
Livia Maccioni, Susanne Weber, Magdeldin Elgizouli, Anne-Sophie Stoehlker, Ilona Geist, Hans-Hartmut Peter, Werner Vach, Alexandra Nieters   +7 more
doaj   +1 more source

Altered Microbiota, Impaired Quality of Life, Malabsorption, Infection, and Inflammation in CVID Patients With Diarrhoea

Frontiers in Immunology, 2020
Background: Diarrhoea is the commonest gastrointestinal symptom in patients with common variable immunodeficiency (CVID).Objective: The aim of this study was to describe the prevalence and clinical presentation of chronic and recurrent diarrhoea in the ...
Cornelia M. van Schewick, Cornelia M. van Schewick, Christina Nöltner, Svenja Abel, Svenja Abel, Siobhan O. Burns, Sarita Workman, Andrew Symes, David Guzman, Michele Proietti, Alla Bulashevska, Fernando Moreira, Veronika Soetedjo, David M. Lowe, Bodo Grimbacher, Bodo Grimbacher, Bodo Grimbacher, Bodo Grimbacher, Bodo Grimbacher   +18 more
doaj   +1 more source

Pregnancy, child bearing and prevention of giving birth to the affected children in patients with primary immunodeficiency disease; a case-series

BMC Pregnancy and Childbirth, 2018
Background Patients with primary immunodeficiency disease (PID) who survive to adulthood and willing to have a child mostly are worried whether their disease affects their fertility and/or pregnancy and also if their child would be predisposed to PID ...
Saba Sheikhbahaei, Roya Sherkat, Nadezhda Camacho-Ordonez, Razie Khoshnevisan, Asadollah Kalantari, Mansour Salehi, Seyed Saman Nazemian, Mohammad Hossein Nasr-esfahani, Christophe Klein   +8 more
doaj   +1 more source

Modeling MyD88 Deficiency In Vitro Provides New Insights in Its Function

Frontiers in Immunology, 2020
Inherited defects in MyD88 and IRAK4, two regulators in Toll-like receptor (TLR) signaling, are clinically highly relevant, but still incompletely understood.
Nils Craig-Mueller, Nils Craig-Mueller, Nils Craig-Mueller, Ruba Hammad, Ruba Hammad, Ruba Hammad, Roland Elling, Roland Elling, Roland Elling, Jamal Alzubi, Jamal Alzubi, Barbara Timm, Barbara Timm, Julia Kolter, Julia Kolter, Nele Knelangen, Nele Knelangen, Christien Bednarski, Christien Bednarski, Birgitta Gläser, Sandra Ammann, Sandra Ammann, Zoltán Ivics, Judith Fischer, Judith Fischer, Carsten Speckmann, Carsten Speckmann, Carsten Speckmann, Klaus Schwarz, Nico Lachmann, Nico Lachmann, Stephan Ehl, Stephan Ehl, Stephan Ehl, Thomas Moritz, Thomas Moritz, Philipp Henneke, Philipp Henneke, Philipp Henneke, Philipp Henneke, Toni Cathomen, Toni Cathomen, Toni Cathomen   +42 more
doaj   +1 more source