John Dempster

Status Epilepticus Protocol Variation Across Accredited National Association of Epilepsy Centers Members

Laura K. Lamberta, Melissa M. Asmar, Megan Fredwall, Stephanie M. Ahrens et autres

OBJECTIVES: Status epilepticus (SE) is a neurologic emergency that requires urgent recognition and medical management. SE management remains heterogeneous across centers. METHODS: We analyzed SE treatment protocols from level 3 and level 4 epilepsy centers. Discrete data including stabilization measures, timing of …

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A multi-centre UK-based survey on angioedema secondary to acquired C1 inhibitor deficiency

Hadeil Morsi, Aarnoud Huissoon, Alexandros Grammatikos, Andrew Whyte et autres

BACKGROUND: Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH) is very rare compared to its prototype, hereditary angioedema. An updated characterization of the AAE-C1-INH cohort in the UK is required to inform management. OBJECTIVES: To describe the disease burden of AAE-C1-INH, long-term prophylaxis …

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Lanadelumab for the prevention of hereditary angioedema attacks: A real‐world UK audit

Anthony D. Dorr, Charu Chopra, Tanya Coulter, John Dempster et autres

Appendix S1: Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

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Impact of vaccination on hospitalization and mortality from COVID-19 in patients with primary and secondary immunodeficiency: The United Kingdom experience

Adrian Shields, Susan Tadros, Adam Al‐Hakim, Jeremy Nell et autres

Background: Individuals with primary and secondary immunodeficiency (PID/SID) were shown to be at risk of poor outcomes during the early stages of the SARS-CoV-2 pandemic. SARS-CoV-2 vaccines demonstrate reduced immunogenicity in these patients. Objectives: To understand whether the risk of severe COVID-19 …

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A Multicentre UK-Based Audit on Acquired C1 Inhibitor Deficiency 2021

Hadeil Morsi, Alexandros Grammatikos, Cathal Steele, Catherine Stroud et autres

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Outcomes following SARS-CoV-2 infection in patients with primary and secondary immunodeficiency in the UK

Adrian Shields, Ariharan Anantharachagan, Gururaj Arumugakani, Kenneth F. Baker et autres

In March 2020, the United Kingdom Primary Immunodeficiency Network (UKPIN) established a registry of cases to collate the outcomes of individuals with PID and SID following SARS-CoV-2 infection and treatment. A total of 310 cases of SARS-CoV-2 infection in individuals with PID …

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Outcomes Following SARS-CoV-2 Infection in Patients With Primary and Secondary Immunodeficiency in The United Kingdom

Adrian Shields, Ariharan Anantharachagan, Gururaj Arumugakani, Kenneth F. Baker et autres

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COVID-19 in patients with primary and secondary immunodeficiency: The United Kingdom experience

Adrian Shields, Siobhan O. Burns, Sinisa Savic, Alex Richter et autres

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Whole-genome sequencing of a sporadic primary immunodeficiency cohort

James Thaventhiran, Hana Lango Allen, Oliver S. Burren, William Rae et autres

Primary immunodeficiency (PID) is characterized by recurrent and often life-threatening infections, autoimmunity and cancer, and it poses major diagnostic and therapeutic challenges. Although the most severe forms of PID are identified in early childhood, most patients present in adulthood, typically with no …

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Characterization of the clinical and immunologic phenotype and management of 157 individuals with 56 distinct heterozygous NFKB1 mutations

Manfred Fliegauf, Nils Klammer, Natalie Frede, Michele Proietti et autres

BACKGROUND: An increasing number of NFKB1 variants are being identified in patients with heterogeneous immunologic phenotypes. OBJECTIVE: To characterize the clinical and cellular phenotype as well as the management of patients with heterozygous NFKB1 mutations. METHODS: In a worldwide collaborative effort, we …

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Practicalities of a reduced volume formulation of a C1-INH concentrate for the treatment of hereditary angioedema: real-life experience

John Dempster

Hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency is characterized by recurrent swelling attacks that can be life-threatening if left untreated. Prompt treatment is vital during acute attacks; plasma-derived C1-INH (Berinert ® ) is one treatment currently licensed for the …

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Real-world outcomes in hereditary angioedema: first experience from the Icatibant Outcome Survey in the United Kingdom

Hilary Longhurst, John Dempster, Lorena Lorenzo, Matthew Buckland et autres

BACKGROUND: Hereditary angioedema (HAE) is a potentially life-threatening, bradykinin-mediated disease, often misdiagnosed and under-treated, with long diagnostic delays. There are limited real-world data on best-practice management of HAE in the UK. OBJECTIVES: To characterize the clinical profile, management and outcomes of patients …

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