Results 71 to 80 of about 10,179 (209)

AUTOINFLAMMATORY DISEASES IN CHILDREN(The Lecture from 18th of September 2013, Conference «Topical Problems of Diagnostics and Treatment of Juvenile Rheumatoid Arthritis» (18–20 of September, 2013, St. Petersburg))

Вопросы современной педиатрии, 2014
Data about clinical signs, diagnostics and treatment of hereditary autoinflammatory syndromes, e.g. cryopyrin-associated periodic syndrome (CAPS), familial Mediterranean fever (FMF), TNF-receptor associated periodic syndrome (TRAPS-syndrome ...
M. Gattorno
doaj   +1 more source

Inborn errors of immunity in children with neuroinflammation

Developmental Medicine &Child Neurology, EarlyView.
Abstract Inborn errors of immunity (IEIs), an expanding group of monogenic disorders with diverse clinical manifestations, are increasingly recognized to include neuroinflammatory disease. Examples of diseases included under this umbrella are Aicardi–Goutières syndrome, deficiency of adenosine deaminase 2, familial haemophagocytic lymphohistiocytosis ...
Eppie M Yiu, Dilan Dissanayake, Jessie Cunningham, Helen Branson, Ori Scott, E Ann Yeh   +5 more
wiley   +1 more source

Functional immune profiling reveals CD4+ T cell dysregulation in coeliac disease

Immunology &Cell Biology, EarlyView.
The T cell momentum assay quantifies division, survival and activation decay to reveal intrinsic CD4+ programming defects marked by prolonged activation and impaired feedback control in coeliac disease. This scalable platform provides a functional screen for early T cell dysregulation across autoimmune and immune‐mediated diseases.
Anthony J Farchione, HoChan Cheon, David Vremec, Julika Neumann, Gwenny M Verstappen, Melinda Y Hardy, Mai B Margetts, Lauren J Howson, Lee M Henneken, Maureen Forde, Jason A Tye‐Din, Susanne Heinzel, Philip D Hodgkin, Vanessa L Bryant   +13 more
wiley   +1 more source

The Therapeutic Role of Interleukin-1 Inhibition in Idiopathic Recurrent Pericarditis: Current Evidence and Future Challenges

Frontiers in Medicine, 2017
Recurrent pericarditis is a common complication of acute pericarditis (15–30%) for which, in most cases, no underlying etiology is found [idiopathic recurrent pericarditis (IRP)]. IRP is currently viewed as an autoinflammatory disease with characteristic
George Lazaros, Katerina Antonatou, Dimitrios Vassilopoulos   +2 more
doaj   +1 more source

Australian clinical practice guideline: diagnosis and treatment of idiopathic multicentric Castleman disease

Internal Medicine Journal, EarlyView.
Abstract Idiopathic multicentric Castleman disease (iMCD) is a rare condition. The pathogenesis is incompletely understood; however, interleukin‐6 (IL‐6) is a major mediator. The clinical presentation is heterogeneous, from mild constitutional symptoms to severe multi‐organ failure.
Dipti Talaulikar, Jun Yen Ng, Adam Bryant, Annmarie Bosco, Hugh C. Caterson, Pratyush Giri, Pravin Hissaria, Simon J. Harrison, Stephen Lade, Connull Leslie, Jacinta Perram, H. Miles Prince, Dejan Radeski, Maureen Rischmueller, Craig Wallington‐Gates, Nicholas Weber, Alberta Hoi   +16 more
wiley   +1 more source

Principles of clinical genetics for rheumatologists: clinical indications and interpretation of broad-based genetic testing

Advances in Rheumatology
Advances in DNA sequencing technologies, especially next-generation sequencing (NGS), which is the basis for whole-exome sequencing (WES) and whole-genome sequencing (WGS), have profoundly transformed immune-mediated rheumatic disease diagnosis. Recently,
Renan Rodrigues Neves Ribeiro do Nascimento, Caio Robledo D’Angioli Costa Quaio, Christine Hsiaoyun Chung, Dewton de Moraes Vasconcelos, Flavio Roberto Sztajnbok, Nilton Salles Rosa Neto, Sandro Félix Perazzio   +6 more
doaj   +1 more source

When to consider an inborn error of immunity: clues for physicians

Internal Medicine Journal, EarlyView.
Abstract The term inborn errors of immunity (IEIs) refers to the rapidly expanding group of genetic disorders causing dysregulation of the immune system. With improved genetic testing in recent years, the number of defined IEIs and their range of phenotypic presentations has grown vastly, with more than 550 IEIs now described.
Meera Thangarajah, Lucinda J. Berglund
wiley   +1 more source

Autoinflammatory diseases in children.

, 2010
Autoinflammatory diseases encompass a growing number of multisystem clinical entities with genetic or acquired defects in the innate immune system. Distinct conditions can be identified in this expanding sphere: familiar Mediterranean fever, mevalonate ...
Rigante, Donato
core  

How to identify a patient with autoinflammatory syndrome: Clinical and diagnostic algorithms

Современная ревматология, 2013
Autoinflammatory syndromes (AISs) are a group of predominantly hereditary diseases associated with the spontaneous uncontrolled production of proinflammatory cytokines. Most diseases are known to have molecular mechanisms and an inheritance pattern.
Mikhail Mikhailovich Kostik, L S Snegireva, M F Dubko, V V Masalova, T S Likhacheva, O V Kalashnikova, V G Chasnyk   +6 more
doaj   +1 more source

The Transcription Factor EGR2 Plays a Central Role in the Expansion and Function of TCRαβ+CD4−CD8− Double Negative T Cells in lpr Lupus Mice

Immunology, EarlyView.
The highly expanded TCRβ+ DNT cells in autoimmune‐prone B6/lpr mice are phenotypically and functionally different from the TCRβ+DNT cells of normal B6 mice. Conditional Egr2 deletion in B6/lpr mice not only reduces TCRβ+ DNT cell numbers but also tends to correct the phenotypic and functional abnormalities of DNT cells in B6/lpr mice. In addition, Egr2
Rujuan Dai, Zhuang Wang, Bettina Heid, Christopher M. Reilly, S. Ansar Ahmed   +4 more
wiley   +1 more source