Publications by authors named "Stephen D Betschel"

Angioedema.

Allergy Asthma Clin Immunol

December 2024

Angioedema can occur in the absence of urticaria and can be broadly divided into three main categories: mast cell-mediated (e.g., histamine), non-mast-cell-mediated (bradykinin-induced) and idiopathic angioedema.

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Background: Hereditary angioedema (HAE) is characterized by debilitating attacks of tissue swelling in various locations. While guidelines recommend the importance of early on-demand treatment, recent data indicate that many patients delay or do not treat their attacks.

Objective: This survey aimed to investigate patient behavior and evaluate the key factors that drive on-demand treatment decision-making, as reported by those living with HAE.

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Article Synopsis
  • Angioedema (AE) is a condition characterized by localized swelling in the skin or mucous membranes and can be hereditary or acquired, making its classification complex due to various underlying mechanisms and taxonomies.
  • The DANCE initiative, involving 91 experts from 35 countries, aimed to create a unified consensus on the definition, acronyms, and classification of AE through an extensive online discussion and voting process over 16 months.
  • The resulting DANCE classification introduces five types of AE, standardizes terminology, and is designed to enhance research and patient care while complementing existing clinical guidelines without replacing them.
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Hereditary angioedema (HAE) is a rare disease characterized by sudden and often unprovoked episodes of swelling that can be potentially life-threatening when it involves the upper airway. The treatment options for both acute episodes of HAE and LTP, used to minimize the frequency and severity of angioedema attacks, were limited historically to very few options, had considerable side effects, and/or had considerable burden of treatment. Fortunately, through the elucidation of the pathophysiology of HAE, the development of newer targeted therapies has been possible both for acute therapy and long-term prophylaxis and even more are on the horizon.

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Background: The rates of suspected allergic reactions to the first dose of the coronavirus disease 2019 (COVID-19) mRNA vaccines have been reported to be as high as 2%, with an anaphylaxis incidence up to 2.5 per 10,000 individuals. Anaphylaxis in response to the first dose may be considered a contraindication to administration of the second dose, even though the second dose is necessary for optimal protection against severe disease.

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Importance: Hereditary angioedema is a rare disease of potentially life-threatening attacks of angioedema that can affect patients of all ages, including women of childbearing age. Pregnancy can affect the course of the disease and the choice of treatment used. It is important for the care providers to recognize this disease and understand its mechanism in order to provide appropriate care for the patients.

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Background: Hereditary angioedema (HAE) is a rare autosomal dominant disease resulting in recurring episodes of swelling, leading to considerable patient morbidity and mortality. Lanadelumab is a plasma kallikrein inhibitor that is approved as 1st line therapy in Canada for long term prophylaxis of HAE attacks.

Objective: To describe our clinical findings from a case series of adult patients with HAE type 1/2 who have been initiated on lanadelumab.

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The objective of this study was to perform a systematic review of the literature on vaccine responsiveness in patients who have received anti-CD20 therapy. PubMed and EMBASE were searched up to 4 January 2021 to identify studies of vaccine immunogenicity in patients treated with anti-CD20 therapy, including patients with hematologic malignancy or autoimmune disease. The primary outcomes were seroprotection (SP), seroconversion (SC), and/or seroresponse rates for each type of vaccine reported.

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Background: Gastrointestinal (GI) symptoms are common among patients with common variable immunodeficiency disorder (CVID) yet remain poorly understood.

Aims: The aim of this study was to characterize the demographic, clinical, endoscopic and histologic features of patients with CVID and GI symptoms.

Methods: We conducted a retrospective observational study of all patients with CVID at a large Canadian tertiary care centre between January 2000 and May 2018.

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Urticaria (hives) is a common disorder that often presents with angioedema (swelling that occurs beneath the skin). It is generally classified as acute or chronic. Second-generation, non-sedating, non-impairing histamine type 1 (H1)-receptor antihistamines represent the mainstay of therapy for both acute and chronic urticaria.

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Purpose: Patients with common variable immune deficiency and X-linked agammaglobulinemia are unable to produce their own antibodies thus leading to a higher incidence of recurrent infections, particularly those involving the sinuses and lungs. Treatment with intravenous immunoglobulin therapy aims to reduce the incidence of infections; however, as serum IgG approaches its trough during the third and fourth week after infusion, we hypothesized that the rate of infection would be higher during this time period.

Methods: Patients with a diagnosis of either common variable immunodeficiency (CVID) or X-linked agammaglobulinemia (XLA) treated with intravenous immunoglobulin (IVIg) were analyzed in a prospective cohort study.

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The clinical evaluation of angioedema is reliant on obtaining a thorough patient and family history with an assessment of risk factors and presenting symptoms unique to each subtype. It is important to distinguish between angioedema with and without urticaria as a primary step in the evaluation; thereafter, laboratory parameters and investigations allow for subsequent stratification. There is a significant disease burden associated with angioedema and thus it is essential for health care practitioners to establish a prompt and accurate diagnosis, and a comprehensive care plan that addresses the patient's physical and mental well-being alike.

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In Canada, intravenous immune globulin (IVIg) products are licensed for six disease indications, however it has been demonstrated that patients with a number of other conditions also benefit from IVIg. Here we report the routine clinical use of Octagam(®) 10 % across three Canadian institutions. A total of 135 patients were treated with Octagam(®), for conditions represented by five distinct indication groups.

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Introduction: Occupational allergy to rodents among laboratory animal workers is common. Most patients generally experience allergic symptoms after the first few years of work. Associated symptoms are usually mild, such as rhinoconjunctivits, urticaria, and asthma.

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Background: Switching primary/secondary immunodeficiency (PID/SID) patients from intravenous immunoglobulin (IVIg) to home-based subcutaneous immunoglobulin (SCIg) therapy reduces nurse time. A nurse shortage in Canada provides an important context to estimate the net economic benefit, the number of patients needed to switch to SCIg to recoup one full-time equivalent (FTE), and potential population-wide savings of reduced nurse time to a payer.

Methods: The net economic benefit was estimated by multiplying the hourly compensation for nurses in Canada by the hours required for each administration route.

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The first documented confirmed case of an imported fire ant causing anaphylaxis in Canada is herein reported. In a patient with anaphylaxis to ants a physician in Canada should be aware that an allergic reaction to fire ant is a possibility.

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