Hereditary angioedema

Zacek, Lisa

doi:10.1097/01.nurse.0000891944.11247.83

Cited by 2 publications

(1 citation statement)

References 16 publications

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“…Aktas u svom radu opisuje atipičnu prezentaciju HAE sa amnezijom, lošom koncentacijom i glavoboljom [10]. Uvek treba imati na umu da je HAE retka bolest koja lako može dovesti do greške u postavljaju dijagnoze na šta ukazuje i autor Zacek opisujući slučaj pojave edema kod trinaestogodišnjakinje kod koje je došlo do pogoršanja stanja te upućivanja na urgentni tretman u hospitalne uslove [18]. U okviru profilaktičke terapije koriste se atenuisani androgeni i sveža smrznuta plazma, a za lečenje akutnih epizoda, dostupni su plazmatski ili rekombinantni inhibitori C1, koji se dobro podnose i efikasno prekidaju napad, zatim inhibitori bradikininskih B2 receptora, i inhibitori kalikreina.…”

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A case of angioedema of hereditary origin in a rural clinic near Subotica

Gavrilović,

Momirović-Stojković,

Budimski-Soldat

2023

ABC - čas urg medicine

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Introduction: Hereditary angioedema (HAE) is inherited in an autosomal dominant manner. It manifests with bradykinin-mediated swelling due to a deficiency of C1 inhibitor. Managing these patients is challenging during acute edema and the onset of asphyxiation. Transportation to the hospital takes 25 minutes. Objective: To present a patient with HAE for whom specific therapy has only recently become available. Case Presentation: A female patient presented with difficulty breathing, swelling of the lips, and periorbital edema. Status: visibly distressed, talkative, facial and lip swelling, visible mucosa without edema, uvula and pharynx calm. Respiratory movements symmetric, without the use of accessory muscles. Auscultation revealed normal breath sounds, tachycardic heart action, clear tones without murmurs, and no urticaria. Blood pressure 120/70 mmHg, heart rate 100/min., respiratory rate 30/min., SpO2 98%, capillary refill time (CRT) 2 sec. Treatment: infusion, Methylprednisolone 40 mg IV. She was transported with monitoring. During transport, the condition worsened: blood pressure 105/60 mmHg, heart rate 130/min., respiratory rate 40/min., SpO2 94%, CRT 2 sec. Oxygen at 15 L/min. was initiated, an additional IV line was opened, and capnography showed ETCO2 of 36 mmHg. Methylprednisolone was increased to 80 mg. Preparation for difficult intubation was initiated. Parameters did not worsen, and the patient was transferred to the emergency department of the General Hospital Subotica. Conclusion: Since specific therapies, such as human C1 inhibitor concentrate or bradykinin receptor antagonists, have been approved for self-administration at home during acute attacks, the treatment of these patients has been facilitated, minimizing the potential fatal outcomes and morbidity.

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Section: Diskusijaunclassified

A case of angioedema of hereditary origin in a rural clinic near Subotica

Gavrilović,

Momirović-Stojković,

Budimski-Soldat

2023

ABC - čas urg medicine

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An expert panel's review on patients with hereditary angioedema switching from attenuated androgens to oral prophylactic therapy

Lumry,

Bernstein,

et al. 2024

allergy asthma proc

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Background: Hereditary angioedema (HAE) is a rare condition marked by swelling episodes in various body parts, including the extremities, upper airway, face, intestinal tract, and genitals. Long-term prophylaxis (LTP), prescribed to control recurring HAE attacks, is integral to its management. Previously, attenuated androgens (AAs) were the only oral LTP options. However, in 2020, berotralstat, an oral plasma kallikrein inhibitor, was approved in the United States. A 2018 survey of adults with HAE type I or type II showed that almost all the patients who used prophylactic HAE medication preferred oral treatment (98%) and felt that it fit their lifestyle better than injectable treatment (96%). Still, guidelines lack consensus on transitioning patients from AAs to alternative oral prophylactic therapy. Objective: This paper aims to share expert insights and patient feedback on transitioning from AAs to berotralstat, an alternative oral prophylactic therapy, from the perspective of clinicians with extensive experience in treating patients with HAE. Methods: A panel of five HAE specialists convened for a virtual half-day roundtable discussion in April 2023. Results: Discussions about transitioning from AAs to berotralstat were prompted by routine consultations, patient inquiries based on independent research, ineffective current treatment, or worsening AA-related adverse effects. For patients who switched from AAs, the physicians reported that the decision was influenced by the alternative therapy's ability to prevent HAE attacks, its safety, and the once-daily administration schedule. All expert panel members identified fewer AA-related adverse effects; better quality of life; and less severe, shorter, and less frequent HAE attacks as clinical or patient goals they hoped to achieve through the treatment switch. Conclusion: The emergence of new, highly specific LTP drugs for HAE calls for the development of comprehensive recommendations and guidelines for transitioning from AAs to alternative oral prophylactic therapy. The expert panel highlighted key factors to consider during the development of such guidelines.

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Hereditary angioedema

Cited by 2 publications

References 16 publications

A case of angioedema of hereditary origin in a rural clinic near Subotica

A case of angioedema of hereditary origin in a rural clinic near Subotica

An expert panel's review on patients with hereditary angioedema switching from attenuated androgens to oral prophylactic therapy

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