International Consensus Document (ICON): common variable immunodeficiency disorders

FA Bonilla, I Barlan, H Chapel… - The Journal of …, 2016 - jaci-inpractice.org
The International Collaboration in Asthma, Allergy and Immunology initiated an international
coalition among the American Academy of Allergy, Asthma & Immunology; the European …

Lung disease in primary antibody deficiencies

E Schussler, MB Beasley, PJ Maglione - The Journal of Allergy and Clinical …, 2016 - Elsevier
Primary antibody deficiencies (PADs) are the most common form of primary
immunodeficiency and predispose to severe and recurrent pulmonary infections, which can …

Flow cytometry, a versatile tool for diagnosis and monitoring of primary immunodeficiencies

RS Abraham, G Aubert - Clinical and Vaccine Immunology, 2016 - Am Soc Microbiol
Genetic defects of the immune system are referred to as primary immunodeficiencies (PIDs).
These immunodeficiencies are clinically and immunologically heterogeneous and …

[HTML][HTML] Redefined clinical features and diagnostic criteria in autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy

EMN Ferre, SR Rose, SD Rosenzweig, PD Burbelo… - JCI insight, 2016 - ncbi.nlm.nih.gov
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare
primary immunodeficiency disorder typically caused by homozygous AIRE mutations. It …

A cross-sectional study of the prevalence of gastrointestinal symptoms and pathology in patients with common variable immunodeficiency

SF Jørgensen, HM Reims, D Frydenlund… - Official journal of the …, 2016 - journals.lww.com
OBJECTIVES: The objective of this study was to study the prevalence of gastrointestinal (GI)
symptoms and histopathology in patients with common variable immunodeficiency (CVID) …

[HTML][HTML] Altered gut microbiota profile in common variable immunodeficiency associates with levels of lipopolysaccharide and markers of systemic immune activation

SF Jørgensen, M Trøseid, M Kummen… - Mucosal …, 2016 - Elsevier
Common variable immunodeficiency (CVID) is the most common symptomatic primary
immunodeficiency characterized by low immunoglobulin (Ig) G and IgA, and/or IgM. In …

Immunopathogenesis of granulomas in chronic autoinflammatory diseases

WMC Timmermans, JAM Van Laar… - Clinical & …, 2016 - Wiley Online Library
Granulomas are clusters of immune cells. These structures can be formed in reaction to
infection and display signs of necrosis, such as in tuberculosis. Alternatively, in several …

Defects of B-cell terminal differentiation in patients with type-1 Kabuki syndrome

AW Lindsley, HM Saal, TA Burrow, RJ Hopkin… - Journal of Allergy and …, 2016 - Elsevier
Background Kabuki syndrome (KS) is a complex multisystem developmental disorder
associated with mutation of genes encoding histone-modifying proteins. In addition to …

[HTML][HTML] Low circulating natural killer cell counts are associated with severe disease in patients with common variable immunodeficiency

M Ebbo, L Gérard, S Carpentier, F Vély, S Cypowyj… - …, 2016 - thelancet.com
Natural Killer (NK) cells have been shown to exert antiviral and antitumoural activities.
Nevertheless most available data are derived from mouse models and functions of these …

Autoimmune and lymphoproliferative complications of common variable immunodeficiency

PJ Maglione - Current allergy and asthma reports, 2016 - Springer
Common variable immunodeficiency (CVID) is frequently complicated by the development of
autoimmune and lymphoproliferative diseases. With widespread use of immunoglobulin …