Results 51 to 60 of about 7,759 (229)

Novel genetic variants of inborn errors of immunity.

PLoS ONE, 2021
ObjectivesInborn errors of immunity (IEI) are prevalent in tribal cultures due to frequent consanguineous marriages. Many of these disorders are autosomal recessive, resulting from founder mutations; hence they are amenable to prevention.
Farida Almarzooqi, Abdul-Kader Souid, Ranjit Vijayan, Suleiman Al-Hammadi   +3 more
doaj   +1 more source

Inborn Errors of Immunity With Immune Dysregulation: From Bench to Bedside

Frontiers in Pediatrics, 2019
Inborn errors of immunity are genetic disorders with broad clinical manifestations, ranging from increased susceptibility to infections to significant immune dysregulation, often leading to multiple autoimmune phenomena, lymphoproliferation, and ...
Ottavia Maria Delmonte, Riccardo Castagnoli, Riccardo Castagnoli, Enrica Calzoni, Enrica Calzoni, Luigi Daniele Notarangelo   +5 more
doaj   +1 more source

Abatacept for Prophylaxis of Acute and Chronic Graft‐Versus‐Host‐Disease in Children Undergoing Allogeneic Hematopoietic Stem Cell Transplantation—A Systematic Review and Meta‐Analysis of Cohort Studies

Pediatric Blood &Cancer, EarlyView.
ABSTRACT Background Graft‐versus‐host disease (GvHD) remains one of the most significant complications of hematopoietic stem cell transplantation (HSCT). Abatacept, a selective T‐cell costimulation modulator, is a relatively novel prophylactic agent, though pediatric data remain limited.
Matthijs D. Kruizinga, Anne B. Verbeek, Caroline A. Lindemans, Emilie P. Buddingh   +3 more
wiley   +1 more source

ADA2-deficient cells exhibit increased levels of cell death and metabolic disturbances

Cell Death Discovery
Deficiency of adenosine deaminase 2 (DADA2) causes a complex phenotype of autoinflammation and immunodeficiency. Bone marrow failure is often refractory to treatment with tumour necrosis factor-alpha (TNF-alpha) inhibitors and additional treatment ...
Lisa Ehlers, Marjon Wouters, Bethany Pillay, Selket Delafontaine, Giorgia Bucciol, Marco Baggio, Mariia Dzhus, Anneleen Hombrouck, Alexandra Damerau, Lien De Somer, Rik Schrijvers, Steven Vanderschueren, Maarten Jacquemyn, Tilmann Kallinich, Dirk Daelemans, Bart Ghesquière, Patrizia Agostinis, Leen Moens, Isabelle Meyts   +18 more
doaj   +1 more source

Future of Therapy for Inborn Errors of Immunity [PDF]

Clinical Reviews in Allergy & Immunology, 2022
Over the past 20 years, the rapid evolution in the diagnosis and treatment of primary immunodeficiencies (PI) and the recognition of immune dysregulation as a feature in some have prompted the use of "inborn errors of immunity" (IEI) as a more encompassing term used to describe these disorders [1, 2] .
openaire   +2 more sources

Inborn Errors of Immunity

Abstract Inborn errors of immunity (IEI), commonly known as primary immunodeficiency, are a collection of inherited disorders presenting as susceptibility to uncommon and severe infections, autoimmune diseases, allergy, and malignancy.
Heather K. Lehman, Giulia M. Martone, Ariana Yakuby   +2 more
  +5 more sources

Going viral − Virus‐targeting nanobodies for research, diagnostics and therapy

FEBS Letters, EarlyView.
The increasing prevalence of viral diseases represents a major threat for global human health. Nanobodies, small binding fragments derived from camelid heavy‐chain antibodies, offer versatile tools for viral research, diagnostics, and antiviral therapy.
Domenic Ebert, Ulrich Rothbauer
wiley   +1 more source

Machine Learning‐Driven Design of Multicomponent Bone Inorganic Matrix Mimicking Scaffolds for Osteogenesis Enhanced by Neurogenesis

Advanced Science, EarlyView.
Schematic illustration of development of experimental datasets and algorithm models, screening and preparation of the scaffolds and their applications in vivo. ABSTRACT Bone defects require materials with osteogenic, neurogenic, and angiogenic activity, yet designing such materials within high‐dimensional compositional spaces remains challenging. Here,
Kunlu Lin, Ying Yang, Chuyao Zhou, Hanyue Mao, Zhiquan Zhang, Libangxi Liu, Xiaoying Yu, Haoming Liu, Long Liu, Xiaoyan Wang   +9 more
wiley   +1 more source

Recent advances in primary immunodeficiency: from molecular diagnosis to treatment [version 1; peer review: 3 approved]

F1000Research, 2020
The technological advances in diagnostics and therapy of primary immunodeficiency are progressing at a fast pace. This review examines recent developments in the field of inborn errors of immunity, from their definition to their treatment.
Giorgia Bucciol, Isabelle Meyts
doaj   +1 more source

WINTER SPECIALIZED IMMUNOLOGY SCHOOL, “INBORN ERRORS OF IMMUNITY”

Proceedings of the Shevchenko Scientific Society: Medical Sciences, 2023
On December 15–17, 2022, the Winter Specialized Immunology School, “Inborn Errors of Immunity,” was held in Uzhgorod of Zakarpattia Region with the support of Danylo Halytsky Lviv National Medical University, the International Interdisciplinary ...
Anastasiia Bondarenko, Valentyna Chopyak, Yuriy Stepanovskyi, Khrystyna Lishchuk-Yakymovych, Anna Hilfanova   +4 more
doaj   +1 more source