Management of Children With Hereditary Angioedema Due to C1 Inhibitor Deficiency

Frank, Michael M.; Zuraw, Bruce L.; Banerji, Aleena; Bernstein, Jonathan A.; Craig, Timothy J.; Busse, Paula J.; Christiansen, Sandra C.; Davis-Lorton, Mark; Li, H Henry; Lumry, William R.; Riedl, Marc A.

doi:10.1542/peds.2016-0575

Cited by 51 publications

(81 citation statements)

References 57 publications

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“…It has been observed in 42%‐58% of cases and is often mistaken for urticaria. Misdiagnosis of prodromal erythema marginatum can lead to incorrect or insufficient treatment …”

Section: Management Of Hae‐1/2 In Childrenmentioning

confidence: 99%

“…In 2017, icatibant was licensed for the use in children or adolescents in some countries. Clinical trials to investigate the efficacy and safety of rhC1‐INH and icatibant in children are ongoing …”

Section: Management Of Hae‐1/2 In Childrenmentioning

confidence: 99%

“…Unfavorable effects on behavior are possible. Reduction in ultimate body height may occur owing to the premature closure of epiphyseal growth plates …”

Section: Management Of Hae‐1/2 In Childrenmentioning

confidence: 99%

See 2 more Smart Citations

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

Maurer

Magerl

Ansotegui

et al. 2018

Allergy

467

974

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Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up‐to‐date consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1‐inhibitor (type 1) and HAE with dysfunctional C1‐inhibitor (type 2). The key clinical questions covered by these recommendations are: (1) How should HAE‐1/2 be defined and classified?, (2) How should HAE‐1/2 be diagnosed?, (3) Should HAE‐1/2 patients receive prophylactic and/or on‐demand treatment and what treatment options should be used?, (4) Should HAE‐1/2 management be different for special HAE‐1/2 patient groups such as pregnant/lactating women or children?, and (5) Should HAE‐1/2 management incorporate self‐administration of therapies and patient support measures?

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“…It has been observed in 42%‐58% of cases and is often mistaken for urticaria. Misdiagnosis of prodromal erythema marginatum can lead to incorrect or insufficient treatment …”

Section: Management Of Hae‐1/2 In Childrenmentioning

confidence: 99%

Section: Management Of Hae‐1/2 In Childrenmentioning

confidence: 99%

See 1 more Smart Citation

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

Maurer

Magerl

Ansotegui

et al. 2018

Allergy

467

974

View full text Add to dashboard Cite

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“…Pediatric patients may often be treated with HAE-specific medications in an “off label” approach with limited controlled data to guide these decisions. 5 Data from uncontrolled trials support the use of C1INH concentrate (Cinryze) and tranexamic acid for prophylaxis, and icatibant for on demand treatment in children. Data from uncontrolled trials suggests the efficacy of danazol prophylaxis for children but use is not recommended given the numerous potentially serious adverse effects.…”

Section: Recent Developments In Hae Therapymentioning

confidence: 99%

Emerging Therapies in Hereditary Angioedema

Meng

Riedl

2017

Immunology and Allergy Clinics of North America

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SYNOPSIS Remarkable progress has been made in the treatment of bradykinin mediated angioedema with the advent of multiple new angioedema specific therapies over the last decade, largely studied in patients with hereditary angioedema (HAE) due to C1 esterase inhibitor (C1-INH) deficiency. While affected individuals previously had little treatment available, patients in many parts of the world now have effective medications available for prophylaxis and treatment of acute angioedema attacks. Despite these advances, patients are still faced with challenges of a burdensome disease that can lead to debilitating and dangerous angioedema episodes associated with significant costs for individuals and society. The burden of treatment must also be addressed with regard to medication administration difficulties, treatment complications, and adverse side effects. Multiple new therapies with novel mechanisms of action are currently being investigated and may offer potential solutions to these challenges faced by patients and physicians. We herein review the emerging therapeutic options for the treatment of HAE.

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“…[50]. Berinert ® , at 20 U/kg, has less risk of containing an infectious agent, is approved by the Food and Drug Administration (FDA) in children and is preferable when available [51][52][53].…”

Section: Us Consensus For the Management Of Children With C1-inh-haementioning

confidence: 99%

Short‐Term Prophylaxis in Odontostomatological, Maxillofacial and ENT Procedures in Patients with Hereditary Angioedema Due to C1‐Inhibitor Deficiency

Palomo¹,

Caballero²

2017

A Comprehensive Review of Urticaria and Angioedema

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Oestrogens, trauma, infections or stress has been described as triggers for angioedema (AE) attacks in patients with hereditary angioedema due to C1-inhibitor deficiency (C1-INH-HAE). Microtrauma can precipitate the onset of acute AE attacks, and thus, dental-oral procedures carry a high risk of triggering them and also an increased risk of death from asphyxiation due to the AE location. In the past, without proper specific treatment, the overall mortality after dental surgery in patients with C1-INH-HAE was up to 30-40%. Some dental-oral, medical and/or surgical procedures are susceptible to receive "short-term prophylaxis" (STP) in order to reduce the risk of AE. We describe the published case reports of dental-oral, maxillofacial and ear, nose and throat (ENT) procedures in patients with C1-INH-HAE. Different consensus algorithms and clinical guidelines have been published for managing dental-oral, maxillofacial and otolaryngological procedures (DOMFOPs) and will be reviewed below. Based on the clinical experience of the Department of Allergology of the University Hospital La Paz (Madrid) and the University General Hospital Nuestra Señora del Prado (Talavera de la Reina), these algorithms have been updated and modified. We advise to classify procedures according to the risk of producing AE as minor, intermediate and major risks.

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Management of Children With Hereditary Angioedema Due to C1 Inhibitor Deficiency

Cited by 51 publications

References 57 publications

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

The international WAO/EAACI guideline for the management of hereditary angioedema—The 2017 revision and update

Emerging Therapies in Hereditary Angioedema

Short‐Term Prophylaxis in Odontostomatological, Maxillofacial and ENT Procedures in Patients with Hereditary Angioedema Due to C1‐Inhibitor Deficiency

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