Bradykinin metabolism and drug-induced angioedema

S Smolinska, D Antolín-Amérigo… - International Journal of …, 2023 - mdpi.com
Bradykinin (BK) metabolism and its receptors play a central role in drug-induced
angioedema (AE) without urticaria through increased vascular permeability. Many …

CRISPR-Cas9 In Vivo Gene Editing of KLKB1 for Hereditary Angioedema

HJ Longhurst, K Lindsay, RS Petersen… - … England Journal of …, 2024 - Mass Medical Soc
Background Hereditary angioedema is a rare genetic disease that leads to severe and
unpredictable swelling attacks. NTLA-2002 is an in vivo gene-editing therapy based on …

The inhibition of serine proteases by Serpins is augmented by negatively charged heparin: A concise review of some clinically relevant interactions

ED Chan, PT King, X Bai, AM Schoffstall… - International Journal of …, 2024 - mdpi.com
Serine proteases are members of a large family of hydrolytic enzymes in which a particular
serine residue in the active site performs an essential role as a nucleophile, which is …

Efficacy and safety of garadacimab, a factor XIIa inhibitor for hereditary angioedema prevention (VANGUARD): a global, multicentre, randomised, double-blind …

TJ Craig, A Reshef, HH Li, JS Jacobs, JA Bernstein… - The Lancet, 2023 - thelancet.com
Background Hereditary angioedema is a rare and potentially life-threatening genetic
disease that is associated with kallikrein–kinin system dysregulation. Garadacimab …

Inhibition of prekallikrein for hereditary angioedema

LM Fijen, MA Riedl, L Bordone… - … England Journal of …, 2022 - Mass Medical Soc
Background Hereditary angioedema is characterized by recurrent and unpredictable
swellings that are disabling and potentially fatal. Selective inhibition of plasma prekallikrein …

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 …

Urticaria and angioedema: understanding complex pathomechanisms to facilitate patient communication, disease management, and future treatment

GN Konstantinou, MA Riedl, P Valent, I Podder… - The Journal of Allergy …, 2023 - Elsevier
Chronic spontaneous urticaria (CSU) is primarily a T2-dominant disease with a complex
genetic background. Skin mast cell activation can be induced not only via the IgE-FcεRI axis …

Why does it take so long for rare disease patients to get an accurate diagnosis?—A qualitative investigation of patient experiences of hereditary angioedema

M Isono, M Kokado, K Kato - PLoS One, 2022 - journals.plos.org
Introduction Many patients with rare diseases experience a diagnostic delay. Although
several quantitative studies have been reported, few studies have used a qualitative …

Epidemiology, management, and treatment access of hereditary angioedema in the Asia Pacific region: outcomes from an international survey

PH Li, R Pawankar, BYH Thong, JS Fok… - The Journal of Allergy …, 2023 - Elsevier
Background Hereditary angioedema (HAE) is a rare genetic disease with significant
morbidity and mortality for which early diagnosis and effective therapy are critical. Many Asia …

Sensitivity to change and minimal clinically important difference of the angioedema control test

LM Fijen, C Vera, T Buttgereit… - Clinical and …, 2023 - Wiley Online Library
Abstract Background The Angioedema Control Test (AECT) is a patient‐reported outcome
measure developed and validated for the assessment of disease control in patients with …