Hilary Longhurst
A5089073407- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Hemophilia Treatment and Research
- Autoimmune Bullous Skin Diseases
- Immunodeficiency and Autoimmune Disorders
- Urticaria and Related Conditions
- Vitamin K Research Studies
- Mast cells and histamine
- Peptidase Inhibition and Analysis
- Blood disorders and treatments
- Blood Coagulation and Thrombosis Mechanisms
- Chronic Lymphocytic Leukemia Research
- Enzyme function and inhibition
- Immune Cell Function and Interaction
- COVID-19 Clinical Research Studies
- PI3K/AKT/mTOR signaling in cancer
- SARS-CoV-2 and COVID-19 Research
- Chronic Myeloid Leukemia Treatments
- Mycobacterium research and diagnosis
- Cystic Fibrosis Research Advances
- Parvovirus B19 Infection Studies
- Platelet Disorders and Treatments
- Beetle Biology and Toxicology Studies
- Pharmaceutical studies and practices
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- HIV Research and Treatment
University of Auckland
2021-2025
Auckland City Hospital
2021-2025
Cambridge University Hospitals NHS Foundation Trust
2018-2024
University Hospital Frankfurt
2024
Charité - Universitätsmedizin Berlin
2024
Fraunhofer Institute for Translational Medicine and Pharmacology
2024
Goethe University Frankfurt
2024
Centre National de la Recherche Scientifique
2024
Centre Hospitalier Universitaire de Lille
2024
Centre Hospitalier Universitaire de Grenoble
2016-2024
We published the Canadian 2003 International Consensus Algorithm for Diagnosis, Therapy, and Management of Hereditary Angioedema (HAE; C1 inhibitor [C1-INH] deficiency) updated this as angioedema: a current state-of-the-art review: Hungarian 2007 Angioedema. To update Therapy (circa 2010). The Network (CHAEN)/Réseau Canadien d'angioédème héréditaire (RCAH) http://www.haecanada.com cosponsors University Calgary Society Allergy Clinical Immunology (with an unrestricted educational grant from...
Abstract Hereditary Angioedema ( HAE ) is a rare and disabling disease. Early diagnosis appropriate therapy are essential. This update revision of the global guideline for provides up‐to‐date consensus recommendations management . In development this guideline, an international expert panel reviewed existing evidence developed 20 that were discussed, finalized consented during conference in June 2016 Vienna. The final version incorporates contributions board reviewers endorsing societies....
BackgroundActivated phosphoinositide 3-kinase δ syndrome (APDS) is a recently described combined immunodeficiency resulting from gain-of-function mutations in PIK3CD, the gene encoding catalytic subunit of (PI3Kδ).ObjectiveWe sought to review clinical, immunologic, histopathologic, and radiologic features APDS large genetically defined international cohort.MethodsWe applied clinical questionnaire performed medical notes, radiology, histopathology, laboratory investigations 53 patients with...
To cite this article: Cicardi M, Bork K, Caballero T, Craig Li HH, Longhurst H, Reshef A, Zuraw B on behalf of HAWK ( H ereditary A ngioedema International W or k ing Group). Evidence‐based recommendations for the therapeutic management angioedema owing to hereditary C1 inhibitor deficiency: consensus report an Working Group. Allergy 2012; 67 : 147–157. Abstract Angioedema deficiency (HAE) is a rare, life‐threatening, disabling disease. In last 2 years, results well‐designed and controlled...
Hereditary Angioedema (HAE) is a rare disease and for this reason proper diagnosis appropriate therapy are often unknown or not available physicians other health care providers. For we convened group of specialists that focus upon HAE from around the world to develop only consensus on management HAE, but also provide evidence based grades, strength classification consensus. Since both grading were adhered document meets criteria as guideline. The outcome guideline improve patients with...
Hereditary angioedema (HAE) is a rare and disabling disease for which early diagnosis effective therapy are critical. This revision update of the global WAO/EAACI guideline on management HAE provides up-to-date guidance HAE. For this guideline, an international panel experts reviewed existing evidence, developed 28 recommendations, established consensus by online DELPHI process. The goal these recommendations to help physicians their patients in making rational decisions with deficient C1...
Current treatments for long-term prophylaxis in hereditary angioedema have limitations.To assess the efficacy of lanadelumab, a fully human monoclonal antibody that selectively inhibits active plasma kallikrein, preventing attacks.Phase 3, randomized, double-blind, parallel-group, placebo-controlled trial conducted at 41 sites Canada, Europe, Jordan, and United States. Patients were randomized between March 2016, September 9, 2016; last day follow-up was April 13, 2017. Randomization 2:1...
Hereditary angioedema is a disabling, potentially fatal condition caused by deficiency (type I) or dysfunction II) of the C1 inhibitor protein. In phase 2 trial, use CSL830, nanofiltered preparation that suitable for subcutaneous injection, resulted in functional levels activity would be expected to provide effective prophylaxis attacks.We conducted an international, prospective, multicenter, randomized, double-blind, placebo-controlled, dose-ranging, 3 trial evaluate efficacy and safety...
Hereditary angioedema is a rare genetic disease that leads to severe and unpredictable swelling attacks. NTLA-2002 an in vivo gene-editing therapy based on clustered regularly interspaced short palindromic repeats (CRISPR)-CRISPR-associated protein 9. targets the gene encoding kallikrein B1 (
We evaluated a patient with disseminated Mycobacterium tuberculosis and chelonae infection, of which he died. He also developed autoimmune (type I) diabetes primary hypothyroidism. His serum contained high titer immunoglobulin G autoantibody to interferon-γ (IFN-γ) capable blocking in vitro responses this cytokine by peripheral blood mononuclear cells from normal donors. These results suggest that autoantibodies IFN-γ can induce susceptibility mycobacterial may be refractory chemotherapy.
Hereditary angioedema (C1 inhibitor deficiency, HAE) is associated with intermittent swellings which are disabling and may be fatal. Effective treatments available these most useful when given early in the course of swelling. The requirement to attend a medical facility for parenteral treatment results delays. Home therapy offers possibility earlier better symptom control, enabling patients live more healthy, productive lives. This paper examines evidence patient-controlled home acute...
Activated Phosphoinositide 3-kinase (PI3K) δ Syndrome (APDS), caused by autosomal dominant mutations in PIK3CD (APDS1) or PIK3R1 (APDS2), is a heterogeneous primary immunodeficiency. While initial cohort-descriptions summarized the spectrum of clinical and immunological manifestations, questions about long-term disease evolution response to therapy remain. The prospective European Society for Immunodeficiencies (ESID)-APDS registry aims characterize course, identify outcome predictors...
Background Attacks of hereditary angioedema (HAE) are unpredictable and, if affecting the upper airway, can be lethal. Icatibant is used for physician- or patient self-administered symptomatic treatment HAE attacks in adults. Its mode action includes disruption bradykinin pathway via blockade B2 receptor. Early believed to shorten attack duration and prevent severe outcomes; however, evidence support these benefits lacking. Objective To examine impact timing icatibant administration on...
Background: Rituximab, a chimeric monoclonal antibody against CD20, is increasingly used in the treatment of B-cell lymphomas and autoimmune conditions. Transient peripheral depletion expected following rituximab therapy. Although initial clinical trials did not show significant hypogammaglobulinaemia, reports this are now appearing literature. Methods: We performed retrospective review patients previously treated with that were referred to Clinical Immunology symptomatic or severe...
Hereditary angioedema (HAE) is a rare, debilitating, and potentially life-threatening disease characterized by recurrent edema attacks. Important advances in HAE treatment have been made, including the development of new therapies for treating or preventing Nevertheless, still frequently misdiagnosed inappropriately treated, exposing patients with laryngeal attacks to risk asphyxiation.The Icatibant Outcome Survey (IOS) an international, observational study that documents clinical outcome...