Hereditary angioedema education in otolaryngology residencies: survey of program directors

Patel, Vijay A.; Siddique, Laila; Stahl, Lauren E.; Carr, Michele M.

doi:10.1002/alr.22116

Cited by 4 publications

(4 citation statements)

References 32 publications

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“…HAE with normal C1‐INH, previously referred to as Type III HAE, has normal C1‐INH levels and function as well as normal C4 levels. This type of HAE has been associated with coagulation factor XII (FXII), plasminogen, and angiopoietin‐1 gene mutations 2,4,10 . In HAE associated with FXII gene mutations, the onset of clinical symptoms typically begins in adulthood and predominantly affects females.…”

Section: Methodsmentioning

confidence: 99%

Clinical manifestations of hereditary angioedema and a systematic review of treatment options

Rosi‐Schumacher

Shah

Craig

et al. 2021

Laryngoscope Investig Oto

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Objective This study systematically reviews the existing literature on the management of hereditary angioedema (HAE) and provides an update on the clinical presentation and specific therapies. Methods A literature search of PubMed and Embase databases was conducted from start of the database to February 2021. Inclusion criteria included relevant systematic reviews, randomized control clinical trials, prospective and retrospective cohort studies, and outcomes research published in English and available in full‐text. Out of 310 candidate articles, a total of 55 articles were included in our study. Results The most common genetic form of HAE in up to 85% of cases is caused by low levels of C1 esterase inhibitor (C1‐INH) protein, leading to a bradykinin‐mediated increase in vascular permeability. During an attack of HAE, abortive treatment with C1‐INH replacement is most commonly described, however, icatibant, ecallantide, or fresh frozen plasma are also used. Long‐term prophylaxis in the form of C1‐INH replacement (subcutaneous or intravenous), monoclonal antibodies targeting plasma kallikrein, attenuated androgens, and transexemic acid should be considered for those who suffer from frequent, severe attacks. Conclusion Progressively distal involvement of the upper airway, especially the larynx, has been shown to pose an increased risk of asphyxiation and death in the acute presentation of HAE. Evaluation by an otolaryngologist is often sought during the emergent clinical management of HAE; therefore, it is prudent that the consulting physician is well‐versed in the prompt recognition, triage of patients, and appropriate treatment modalities. Level of Evidence 1A.

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Section: Methodsmentioning

confidence: 99%

Clinical manifestations of hereditary angioedema and a systematic review of treatment options

Rosi‐Schumacher

Shah

Craig

et al. 2021

Laryngoscope Investig Oto

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“…Although otolaryngologists are often required to treat laryngeal angioedema, there is evidence to suggest that they may be inadequately prepared. A survey of program directors ( n = 34) of otolaryngology residencies in the US found that <50% felt their training program provided adequate education and exposure to HAE, even though 97% thought such knowledge was important for their residents to have 40 . Awareness of HAE by otolaryngologists is vital, considering the risk of suffocation due to laryngeal edema, which can be fatal if symptoms are not alleviated or airway patency is not quickly restored.…”

Section: Hae and Otolaryngologymentioning

confidence: 99%

“…A survey of program directors ( n = 34) of otolaryngology residencies in the US found that <50% felt their training program provided adequate education and exposure to HAE, even though 97% thought such knowledge was important for their residents to have. 40 Awareness of HAE by otolaryngologists is vital, considering the risk of suffocation due to laryngeal edema, which can be fatal if symptoms are not alleviated or airway patency is not quickly restored. As such, the mortality rate of undiagnosed patients with HAE during a laryngeal attack is high (29.4%).…”

Section: Hae and Otolaryngologymentioning

confidence: 99%

Could it be hereditary angioedema?—Perspectives from different medical specialties

Magerl,

Sala‐Cunill,

Weber‐Chrysochoou

et al. 2023

Clinical & Translational All

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Hereditary angioedema (HAE) is a rare autosomal dominant disease, with patients often suffering with associated symptoms for many years before receiving a correct diagnosis. The symptoms greatly impact a patient's quality of life (QoL) and include excruciating abdominal pain and angioedema of the skin and submucosa. Angioedema of the larynx represents a significant mortality risk in undiagnosed patients, and a large proportion of patients with HAE receive incorrect diagnoses and undergo unnecessary surgery. HAE‐specific treatments can control and prevent acute life‐threatening episodes, in addition to improving QoL, emphasizing the value of early diagnosis for patients. Diagnostic delay may be due to a lack of HAE awareness by healthcare professionals and the similarity of HAE symptoms with those of more common conditions, complicating differential diagnosis. The multifaceted nature of the condition may result in visits to one of many different medical settings, for example: the Emergency Room, pediatrics, general practice, otolaryngology, gastroenterology, and dermatology. Therefore, it is crucial that physicians in multiple healthcare specialties are aware of the disease to ensure that patients with HAE receive a timely diagnosis. Using patient cases from various medical specialties, this review highlights the necessity for cross‐specialty awareness of HAE and outlines the essential information for the various healthcare professionals that may encounter a patient with HAE symptoms, in order to effectively treat and/or diagnose HAE.

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“…report a comprehensive review of dose‐response studies for subcutaneous immunotherapy and discuss recommended doses as compared to current otolaryngic allergy practice. Finally, in the allergy education arena, Patel et al . describe their assessment of otolaryngology resident education on hereditary angioedema due to C1‐esterase inhibitor deficiency.…”

mentioning

confidence: 99%

A focus on quality

Wise¹

2018

Int Forum Allergy Rhinol

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Hereditary angioedema education in otolaryngology residencies: survey of program directors

Cited by 4 publications

References 32 publications

Clinical manifestations of hereditary angioedema and a systematic review of treatment options

Clinical manifestations of hereditary angioedema and a systematic review of treatment options

Could it be hereditary angioedema?—Perspectives from different medical specialties

A focus on quality

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