[HTML][HTML] The international WAO/EAACI guideline for the management of hereditary angioedema–The 2021 revision and update

M Maurer, M Magerl, S Betschel, W Aberer… - World Allergy …, 2022 - Elsevier
Hereditary Angioedema (HAE) is a rare and disabling disease for which early diagnosis and
effective therapy are critical. This revision and update of the global WAO/EAACI guideline on …

KLRG1 signaling induces defective Akt (ser473) phosphorylation and proliferative dysfunction of highly differentiated CD8+ T cells

SM Henson, O Franzese, R Macaulay… - Blood, The Journal …, 2009 - ashpublications.org
Abstract Highly differentiated CD8+ CD28− CD27− T cells have short telomeres, defective
telomerase activity, and reduced capacity for proliferation, indicating that they are close to …

[HTML][HTML] Loss-of-function nuclear factor κB subunit 1 (NFKB1) variants are the most common monogenic cause of common variable immunodeficiency in Europeans

P Tuijnenburg, HL Allen, SO Burns, D Greene… - Journal of allergy and …, 2018 - Elsevier
Background The genetic cause of primary immunodeficiency disease (PID) carries
prognostic information. Objective We conducted a whole-genome sequencing study …

[HTML][HTML] Treatment of COVID-19 with remdesivir in the absence of humoral immunity: a case report

MS Buckland, JB Galloway, CN Fhogartaigh… - Nature …, 2020 - nature.com
The response to the coronavirus disease 2019 (COVID-19) pandemic has been hampered
by lack of an effective severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) …

Immunoglobulin replacement for secondary immunodeficiency after B-cell targeted therapies in autoimmune rheumatic disease: systematic literature review

S Wijetilleka, C Mukhtyar, D Jayne, A Ala, P Bright… - Autoimmunity …, 2019 - Elsevier
Background Consensus guidelines are not available for the use of immunoglobulin
replacement therapy (IGRT) in patients developing iatrogenic secondary antibody deficiency …

An investigational oral plasma kallikrein inhibitor for on-demand treatment of hereditary angioedema: a two-part, randomised, double-blind, placebo-controlled …

E Aygören-Pürsün, A Zanichelli, DM Cohn, M Cancian… - The Lancet, 2023 - thelancet.com
Background Guidelines recommend effective on-demand therapy for all individuals with
hereditary angioedema. We aimed to assess the novel oral plasma kallikrein inhibitor …

Gastrointestinal symptoms and nutritional issues in patients with hypermobility disorders: assessment, diagnosis and management

C Lam, G Amarasinghe, N Zarate-Lopez… - Frontline …, 2023 - fg.bmj.com
Patients diagnosed with hypermobile Ehlers-Danlos syndrome and hypermobile spectrum
disorders are increasingly presenting to secondary and tertiary care centres with …

Long‐term safety and effectiveness of berotralstat for hereditary angioedema: The open‐label APeX‐S study

H Farkas, M Stobiecki, J Peter… - Clinical and …, 2021 - Wiley Online Library
Background Berotralstat (BCX7353) is an oral, once‐daily inhibitor of plasma kallikrein
recently approved for prevention of angioedema attacks in adults and adolescents with …

[HTML][HTML] Randomized trial of the efficacy and safety of berotralstat (BCX7353) as an oral prophylactic therapy for hereditary angioedema: results of APeX-2 through 48 …

HJ Wedner, E Aygören-Pürsün, J Bernstein… - The Journal of Allergy …, 2021 - Elsevier
Background Berotralstat (BCX7353) is a recently approved, oral, once-daily kallikrein
inhibitor for hereditary angioedema (HAE) prophylaxis. In the APeX-2 trial, berotralstat …

[HTML][HTML] Measurement and interpretation of Salmonella typhi Vi IgG antibodies for the assessment of adaptive immunity

AR Parker, C Bradley, S Harding… - Journal of …, 2018 - Elsevier
Response to polysaccharide vaccination can be an invaluable tool for assessing
functionality of the adaptive immune system. Measurement of antibodies raised in response …