International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency

Caballero, Teresa; Farkas, Henriette; Bouillet, Laurence; Bowen, Tom; Gompel, Anne; Fagerberg, Christina; Bjökander, Janne; Bork, Konrad; Bygum, Anette; Cicardi, Marco; Carolis, Caterina De; Frank, Michael M.; Gooi, Jimmy; Longhurst, Hilary; Martinez‐Saguer, Inmaculada; Nielsen, Erik Waage; Obtulowitz, Krystina; Perricone, Roberto; Prior, Nieves

doi:10.1016/j.jaci.2011.11.025

Cited by 216 publications

(434 citation statements)

References 153 publications

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“…If the family would consider pregnancy termination with the diagnosis of an affected fetus and varying with local ethical restrictions, then prenatal diagnosis of C1‐INH‐HAE may be achieved by chorionic villous sampling or amniocentesis 63. C1‐INH‐HAE has a highly variable disease severity within and between families with poor correlation between gene defect and clinical severity.…”

Section: Resultsmentioning

confidence: 99%

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International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Farkas

Martinez‐Saguer²,

Bork

et al. 2016

Allergy

177

308

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BackgroundThe consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1‐INH‐HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients. We intended to produce consensus recommendations for the diagnosis and management of pediatric patients with C1‐INH‐HAE.MethodsDuring an expert panel meeting that took place during the 9th C1 Inhibitor Deficiency Workshop in Budapest, 2015 (www.haenet.hu), pediatric data were presented and discussed and a consensus was developed by voting.ResultsThe symptoms of C1‐INH‐HAE often present in childhood. Differential diagnosis can be difficult as abdominal pain is common in pediatric C1‐INH‐HAE, but also commonly occurs in the general pediatric population. The early onset of symptoms may predict a more severe subsequent course of the disease. Before the age of 1 year, C1‐INH levels may be lower than in adults; therefore, it is advisable to confirm the diagnosis after the age of one year. All neonates/infants with an affected C1‐INH‐HAE family member should be screened for C1‐INH deficiency. Pediatric patients should always carry a C1‐INH‐HAE information card and medicine for emergency use. The regulatory approval status of the drugs for prophylaxis and for acute treatment is different in each country. Plasma‐derived C1‐INH, recombinant C1‐INH, and ecallantide are the only agents licensed for the acute treatment of pediatric patients. Clinical trials are underway with additional drugs. It is recommended to follow up patients in an HAE comprehensive care center.ConclusionsThe pediatric‐focused international consensus for the diagnosis and management of C1‐INH‐HAE patients was created.

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Section: Resultsmentioning

confidence: 99%

“…Preselection of unaffected embryos for implantation is under consideration in some jurisdictions 63.…”

Section: Resultsmentioning

confidence: 99%

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Farkas

Martinez‐Saguer²,

Bork

et al. 2016

Allergy

177

308

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“…Given the recent increase in the number of therapeutic options for HAE patients as well as new guidelines and consensus documents [14,[19][20][21], this audit aimed to provide more detailed information on UK patients and practice to help inform planning decisions and raise awareness of this condition.…”

Section: Introductionmentioning

confidence: 99%

A UK national audit of hereditary and acquired angioedema

Jolles

Williams

Carne

et al. 2013

Clinical and Experimental Immunology

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SummaryHereditary angioedema (HAE) and acquired angioedema (AAE) are rare lifethreatening conditions caused by deficiency of C1 inhibitor (C1INH). Both are characterized by recurrent unpredictable episodes of mucosal swelling involving three main areas: the skin, gastrointestinal tract and larynx. Swelling in the gastrointestinal tract results in abdominal pain and vomiting, while swelling in the larynx may be fatal. There are limited UK data on these patients to help improve practice and understand more clearly the burden of disease. An audit tool was designed, informed by the published UK consensus document and clinical practice, and sent to clinicians involved in the care of HAE patients through a number of national organizations. Data sets on 376 patients were received from 14 centres in England, Scotland and Wales. There were 55 deaths from HAE in 33 families, emphasizing the potentially lethal nature of this disease. These data also show that there is a significant diagnostic delay of on average 10 years for type I HAE, 18 years for type II HAE and 5 years for AAE. For HAE the average annual frequency of swellings per patient affecting the periphery was eight, abdomen 5 and airway 0·5, with wide individual variation. The impact on quality of life was rated as moderate or severe by 37% of adult patients. The audit has helped to define the burden of disease in the UK and has aided planning new treatments for UK patients.

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“…There is no evidence to suggest that the risk of an attack during forceps or vacuum-assisted vaginal deliveries is greater than during spontaneous deliveries. However, the international consensus and practical guidelines according to Caballero et al recommend administration of hC1-INH as soon as possible [3,5]. In our case, there were no problems during our vacuum extraction delivery.…”

Section: Discussionmentioning

confidence: 57%

“…Martinez-Saguer et al reported 22 cases of type I HAE and recommended the use of prophylactic administration of hC1-INH before labor [3]. However, according to a report on the international consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency [4], it is recommended that patients with HAE deliver in a hospital that provides quick access to consultants in obstetrics, anesthesiology, perinatology, and hC1-INH.…”

Section: Discussionmentioning

confidence: 99%

A Case of Pregnancy Complicated with Type 1 Hereditary Angioedema (HAE 1)

Ushijima¹,

Sasaki²,

Horiuchi³

et al. 2017

J Clin Case Rep

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Hereditary angioedema (HAE) is an inherited autosomal dominant trait and symptoms of HAE include sudden onset of edema in various organs, such as subcutaneous edema, laryngeal edema and gastrointestinal tracts mucosa. We have experienced a case of a pregnant woman diagnosed with HAE and successfully managed her pregnancy. The patient was 37 years old, gravida 1, primiparous. She had experienced two attacks of angioedema during pregnancy but her delivery course was not affected by her HAE. The frequency of attacks during pregnancy had decreased compared with before and after pregnancy. Treatment using human C1-inactivator concentrate was as effective for attack suppression during pregnancy as it had been in her non-pregnant state.

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International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency

Cited by 216 publications

References 153 publications

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

A UK national audit of hereditary and acquired angioedema

A Case of Pregnancy Complicated with Type 1 Hereditary Angioedema (HAE 1)

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