The natural history of hereditary angioedema and the impact of treatment with human C1-inhibitor concentrate during pregnancy: a long-term survey

Czaller, Ibolya; Visy, Beáta; Csuka, Dorottya; Füst, George; Tóth, Ferenc

doi:10.1016/j.ejogrb.2010.05.008

Cited by 111 publications

(240 citation statements)

References 22 publications

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“…Our study is the largest to date and can be considered representative of the approach to and management of pregnancies and deliveries in C1INH-HAE patients in Spain in recent decades. Mild aggravation of C1INH-HAE symptoms was experienced in 59.2% of the pregnancies in our series; this figure is similar to previous reports [18,19]. The mild worsening detected is more likely to be attributable to changes associated with pregnancy rather than to discontinuation of HAE treatment, as most patients were not receiving regular treatment before they became pregnant.…”

Section: Resultssupporting

confidence: 80%

Management of Pregnancy and Delivery in Patients With Hereditary Angioedema Due to C1 Inhibitor Deficiency

González-Quevedo

Larco

Marcos

et al. 2016

J Investig Allergol Clin Immunol

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Background and Objective: There is little information on pregnancy and delivery in patients with hereditary angioedema due to C1 inhibitor deficiency (C1INH-HAE). The aim of this study was to describe the effect of pregnancy and deliveries on symptoms of C1INH-HAE and review the need for and safety of treatments available during the study period. Methods: Retrospective review using a purpose-designed questionnaire of 61 C1INH-HAE patients from 5 hospitals specialized in the management of HAE in Spain. The outcomes measured were number of pregnancies, changes in symptoms during pregnancy and delivery, mode of delivery, type of anesthesia during delivery, treatments received, and tolerance of treatments. Results: We reviewed 125 full-term pregnancies (89 without a prior diagnosis of C1INH-HAE), 14 miscarriages, and 4 induced abortions. Patients reported an increased frequency of C1INH-HAE symptoms in 59.2% of pregnancies (74/125) and the presence of symptoms throughout pregnancy in 40% (50/125). Prophylactic C1INH-HAE therapy was used during 9 (7.2%) of the 125 pregnancies. Nine patients-in 11 pregnancies (8.8 %)-received treatment for acute attacks. Most deliveries (n=110, 88%) were vaginal. A cesarean section was necessary in 15 cases (12%). Short-term prophylaxis with pdhC1INH was administered before 14 deliveries (11.2 %); 111 deliveries (88.8 %) were performed without premedication and were well tolerated. Anesthesia was used in 51 deliveries (40.8%). Conclusions: Pregnancy has a variable influence on the clinical expression of C1INH-HAE. Attacks tend to occur more frequently but not to increase in severity. Vaginal delivery was mostly well tolerated. pdhC1INH prophylaxis should be administered prior to cesarean delivery and is also recommended before vaginal delivery if there are additional risk factors. pdhC1INH should always be available in the delivery room.

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Section: Resultssupporting

confidence: 80%

Management of Pregnancy and Delivery in Patients With Hereditary Angioedema Due to C1 Inhibitor Deficiency

González-Quevedo

Larco

Marcos

et al. 2016

J Investig Allergol Clin Immunol

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“…Ten patients did not receive prophylaxis and one of them experienced mild symptoms during delivery and was treated with a plasma-derived C1-inh concentrate. After vaginal delivery without prophylaxis, a few patients developed mild local edema [63]. Similar observations were also reported by other authors [64,65].…”

Section: Hae In Pregnancysupporting

confidence: 79%

“…Although the frequency and severity of episodes can increase in some patients, others may not have any symptoms. Patients who have had severe or more frequent episodes during this pregnancy or a previous pregnancy or have additional risk factors are recommended to have a vaginal delivery with a prophylactic plasma-derived C1-inh concentrate before delivery [63]. In addition, plasma-derived C1-inh should be accessible during delivery and hospitalization [63].…”

Section: Hae In Pregnancymentioning

confidence: 99%

Hereditary Angioedema

Gelincik¹,

Demir²

2017

A Comprehensive Review of Urticaria and Angioedema

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Hereditary angioedema (HAE) is an autosomal dominantly inherited orphan disease manifested by recurrent unpredictable nonpiting and nonpruritic swelling atacks without urticarial plaques. HAE is caused by a deiciency of the C1 esterase inhibitor (C1-inh) or decreased function of C1-inh. Type 1 HAE, the most common form, occurs due to C1-inh deiciency and is seen with low-serum C1-inh levels. In type 2 HAE, the function of C1-inh is impaired, and in HAE with normal C1-inh serum levels, the function of C1-inh is normal. HAE episodes can afect various sites in the body such as the larynx, face, extremities, gastrointestinal tract, and urogenital area. Acute episodes can be treated with C1-inh concentrates, a kallikrein inhibitor, called ecallantide and bradykinin B2 receptor antagonist, icatibant. Depending on the frequency, severity, and location of the episodes, long-term prophylaxis regimens with plasma-derived C1-inh concentrates, antiibrinolytics, or 17α-alkylated androgens can be used. C1-inh concentrates or 17α-alkylated androgens should be administered before dental procedures and minor or major surgical interventions to provide short-term prophylaxis. In conclusion, HAE is a rare life-threatening disease of which clinical presentation is highly variable and early accurate diagnosis signiicantly prevents mortality and morbidity.

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“…The presence of a fetus with C1‐INH‐HAE may affect the number of maternal attacks 25, 26. The nature of C1‐INH transport across the placental barrier is unclear, but likely requires active transport.…”

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

176

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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The natural history of hereditary angioedema and the impact of treatment with human C1-inhibitor concentrate during pregnancy: a long-term survey

Cited by 111 publications

References 22 publications

Management of Pregnancy and Delivery in Patients With Hereditary Angioedema Due to C1 Inhibitor Deficiency

Management of Pregnancy and Delivery in Patients With Hereditary Angioedema Due to C1 Inhibitor Deficiency

Hereditary Angioedema

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

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