SERPING1 Variants and C1-INH Biological Function: A Close Relationship With C1-INH-HAE

C Drouet, A López-Lera, A Ghannam… - Frontiers in …, 2022 - frontiersin.org
Hereditary angioedema with C1 Inhibitor deficiency (C1-INH-HAE) is caused by a
constellation of variants of the SERPING1 gene (n= 809; 1,494 pedigrees), accounting for …

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 …

Cold urticaria syndromes: diagnosis and management

VL Diaz, KB Gribbons, K Yazdi-Nejad… - The Journal of Allergy …, 2023 - Elsevier
Cold urticaria is a chronic condition causing episodic symptoms of cold-induced wheals or
angioedema in response to direct or indirect exposure to cold temperatures. Whereas …

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 …

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 …

Pharmacological suppression of the kallikrein kinin system with KVD900: an orally available plasma kallikrein inhibitor for the on‐demand treatment of hereditary …

EJ Duckworth, N Murugesan, L Li… - Clinical & …, 2022 - Wiley Online Library
Background Hereditary angioedema (HAE) is a rare genetic disease that leads to recurrent
episodes of swelling and pain caused by uncontrolled plasma kallikrein (PKa) activity …

Once-daily oral berotralstat for long-term prophylaxis of hereditary angioedema: the open-label extension of the APeX-2 randomized trial

S Kiani-Alikhan, R Gower, T Craig, HJ Wedner… - The Journal of Allergy …, 2024 - Elsevier
Background Berotralstat is a first-line, once-daily oral plasma kallikrein inhibitor approved for
prophylaxis of hereditary angioedema (HAE) attacks in patients 12 years or older. Objective …

Stemming commercial milk formula marketing: now is the time for radical transformation to build resilience for breastfeeding

T Doherty, C Horwood, C Pereira-Kotze, L du Plessis… - The Lancet, 2023 - thelancet.com
One of the striking messages of the Lancet Breastfeeding Series1–3 is that the consumption
of commercial milk formula (CMF) by infants and young children has been normalised. More …

Prioritise research on vaccines for pregnant and breastfeeding women

T Manca, F Baylis, FM Munoz, KA Top - The Lancet, 2022 - thelancet.com
To our knowledge, this trial provides the first clinical evidence that FXIIa inhibition has a
good safety profile in patients and supports the hypothesis that FXIIa inhibition can be an …