Adult Angioedema Research Articles

Berotralstat (Orladeyo)


 CADTH reimbursement reviews are comprehensive assessments of the clinical effectiveness and cost-effectiveness, as well as patient and clinician perspectives, of a drug or drug class.
 The assessments inform non-binding recommendations that help guide the reimbursement decisions of Canada's federal, provincial, and territorial governments, with the exception of Quebec.
 This review assesses berotralstat (Orladeyo), 150 mg oral capsule.
 Indication: For routine prevention of episodes of hereditary angioedema in adults and pediatric patients aged 12 years and older.

Physician adjudication of angioedema diagnosis codes in a population of patients with heart failure prescribed angiotensin-converting enzyme inhibitor therapy.

Our objective was to calculate the positive predictive value (PPV) of the ICD-9 diagnosis code for angioedema when physicians adjudicate the events by electronic health record review. Our secondary objective was to evaluate the inter-rater reliability of physician adjudication. Patients from the Cardiovascular Research Network previously diagnosed with heart failure who were started on angiotensin-converting enzyme inhibitors (ACEI) during the study period (July 1, 2006 through September 30, 2015) were included. A team of two physicians per participating site adjudicated possible events using electronic health records for all patients coded for angioedema for a total of five sites. The PPV was calculated as the number of physician-adjudicated cases divided by all cases with the diagnosis code of angioedema (ICD-9-CM code 995.1) meeting the inclusion criteria. The inter-rater reliability of physician teams, or kappa statistic, was also calculated. There were 38 061 adults with heart failure initiating ACEI in the study (21 489 patient-years). Of 114 coded events that were adjudicated by physicians, 98 angioedema events were confirmed for a PPV of 86% (95% CI: 80%, 92%). The kappa statistic based on physician inter-rater reliability was 0.65 (95% CI: 0.47, 0.82). ICD-9 diagnosis code of 995.1 (angioneurotic edema, not elsewhere classified) is highly predictive of angioedema in adults with heart failure exposed to ACEI.

Penoscrotal angioedema in an 8-year-old boy following insect bites

Angioedema in children displays a varied etiology and clinical manifestations, unlike adult angioedema. As opposed to angioedema in adults, pediatric angioedema majorly results from food, insect bites, and drugs. Reactions to insect bites, both allergic and toxic, are frequently encountered in pediatric medical practice but rarely seen in urological practice. Here, we present a case of penoscrotal angioedema resulting from an insect bite in the affected region. This case report emphasizes the need to consider penoscrotal angioedema as an important differential diagnosis of genital swelling, as early diagnosis may obviate fatal complications.

Reactions induced by angiotensin-converting enzyme inhibitors – etiopathogenic, clinical and therapeutic aspects

Angiotensin-converting enzyme inhibitors (ACEI) have been largely used in clinical practice for more than 30 years, mainly for treating arterial hypertension and heart failure. They have been proven to reduce cardiovascular risk, by reducing mortality and morbidity after miocardial infarction and stroke . Besides their well known positive effects, treatment with ACEI may induce some adverse reactions, such as chronic cough and angioedema. Intolerance to ACEI is due to increasing levels of bradikinin in plasma and lungs, secondary to blocking the conversion of angiotensin II to angiotensin I. Cough induced or agravated by ACEI is quite frequently encountered in clinical practice and generally underestimated. Angioedema associated with ACEI therapy has a low incidence, but because of the large number of patients treated with this class of medication, ACEI is the most common cause of angioedema in adults who presented to hospitals or emergency departments. Angioedema may occur any time during the ACEI therapy, usually in the first year, but late onset is possible, leading to delayed or confused diagnosis. The reported incidence is higher in women, African-Americans and patients with previous drug allergies. Diagnosis of ACEI induced angioedema may be difficult because of the late onset, clinical picture similar with histamine-induced angioedema and concomitant medication. Bradikinin- mediated angioedema does not respond to standard therapy with antihistamines, corticosteroids and adrenaline, usually used for treating allergic reactions. Recent studies support the usefulness of subcutaneous icatibant, a selective bradikin B2 receptor antagonist.

Icatibant as acute treatment for hereditary angioedema in adults

ABSTRACTIntroduction: Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is a rare disease, characterized by recurrent, unpredictable episodes of cutaneous and/or mucosal edema. Bradykinin, released by the activation of the contact system, binds to bradykinin B2 receptors on the endothelial cell surface to enhance vascular permeaility, which leads to angioedema.Areas covered: C1-INH-HAE therapy is aimed at the inhibition of bradykinin release, as well as at the blockage of its effects mediated by its receptor. Three controlled trials, three open-label extensions, and two open-label studies, and a prospective, observational study have confirmed the safety and efficacy of the bradykinin B2 receptor antagonist, icatibant administered as acute treatment for HAE attacks in adult patients with C1-INH-HAE.Expert commentary: The ready-to-use, pre-filled syringes of icatibant can be self-administered easily, effectively, safely and, importantly, conviently. – This has resulted in patients being able to quickly treat an attack and realize a dramatic change for the better in their lives.

Angioneurotic edema: report of two cases

Pediatric angioedema exhibits a different cause and clinical manifestations than does adult angioedema. Unlike angioedema in adults, pediatric angioedema is caused mostly due to food, followed by insect bites, infection and antibiotics. Reactions to insect stings, both allergic and toxic, are commonly seen in medical pediatric practice but uncommonly encountered by pediatric dentists. Here we present two cases of angioedema involving the face mainly in children who presented insect bite in the affected region. Treatment and case resolution are described.

Pediatric Angioedema

To clarify the cause, clinical course, and management of children with angioedema. Retrospective review. Urban tertiary care hospital for children. Consecutive sample of all children hospitalized from January 1, 1987, to December 31, 1997, with the diagnosis of angioedema. Complete records permitting analysis were available for 10 patients. Sex, age, site, symptoms at initial examination, cause, therapeutic management, and clinical outcome. Seven boys and 3 girls, a mean age of 7.7 years, had angioedema of the head or neck, most often facial (8/10 [80%]). Manifesting symptoms, in addition to swelling, were tenderness or pain in 4 children (40%), dyspnea in 3 (30%), dysphagia (including drooling and spitting) in 3 (30%), and hoarseness in 1 (10%). Angioedema was due to food in 4 children (40%), insect bites in 3 (30%), infection in 2 (20%), and an antibiotic in 1 (10%). Treatment was pharmacological in all cases. No child required intubation or tracheotomy. Care in the intensive care unit was necessary for 1 child (10%). Pediatric angioedema exhibits a different cause and clinical manifestations than does adult angioedema. Prompt diagnosis and early treatment with an intravenous corticosteroid, an antihistamine, and/or epinephrine lead to rapid resolution and may, in appropriately staffed settings, avoid the need for care in the intensive care unit or airway intervention. Management algorithms based on adult experience must be modified to account for the milder pediatric manifestations of this immunologic disease.