Pregnancy and C1 esterase inhibitor deficiency: a successful outcome

Nathani, Fatima; Sullivan, Helen; Churchill, David

doi:10.1007/s00404-006-0183-6

Cited by 25 publications

(22 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…There have been many isolated reports of pregnant C1INH-HAE patients who experienced no edema attacks after a vaginal or cesarean delivery with prophylactic pdhC1INH treatment [23][24][25][26][27][28]. There have also been reports of 2 cesarean deliveries [29] and 2 vaginal deliveries [30] that were well tolerated without prophylactic therapy.…”

Section: Discussionmentioning

confidence: 99%

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

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionmentioning

confidence: 99%

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

View full text Add to dashboard Cite

show abstract

“…Androgens prophylaxis is contraindicated and antifibrinolitic agents should be used with caution 3 . Even though C1INH concentrate is generally accepted as an effective therapeutic option, the best outcome often requires prophylactic administration 4 . Therefore pregnancy is often challenging and requires multidisciplinary approach during peripartum and postpartum period to ensure a good maternal and fetal outcome.…”

Section: Discussionmentioning

confidence: 99%

Post partum flare in a patient with hereditary angioedema

Amarasekara¹,

Uppal

2012

Sri Lanka J Obstet & Gynae

View full text Add to dashboard Cite

Hereditary angioedema (HAE) is a rare autosomal dominant genetic disorder due to dysfunctional or deficiency of C1 esterase inhibitor (C1INH). This leads to episodic subcutaneous and submucosal oedema involving the upper respiratory and gastrointestinal tracts which can be life threatening. Stress and trauma are known to trigger angioedema attack 1 . As a result of hormonal changes in pregnancy some women may experience increase angioedema attacks. This is a case report of a 34 year old woman who developed life threatening post partum complications with a background history of HAE.

show abstract

“…Postpartum, some patients suffer a higher rate of edema episodes,23,42,52,53 mainly abdominal attacks 42. Caring for and close monitoring of the mother in the postnatal period is recommended for at least 72 hours after giving birth 10,54,55…”

Section: Managementmentioning

confidence: 99%

Management of hereditary angioedema in pregnant women: a review

Caballero¹,

Canabal²,

Rivero-Paparoni³

et al. 2014

IJWH

View full text Add to dashboard Cite

Three types of hereditary angioedema (HAE) have been described: two are due to C1 inhibitor (C1-INH) deficiency (C1-INH-HAE types I and II) and one is characterized by normal C1-INH (nC1-INH-HAE). The management of pregnancy in patients with HAE is often a clinical challenge owing to potential worsening of the disease in relation to the physiological increase in estrogens and the limited treatment options. This review addresses the potential influence of pregnancy on the clinical severity of hereditary angioedema and the management of this disease during pregnancy with currently available treatments.

show abstract

Pregnancy and C1 esterase inhibitor deficiency: a successful outcome

Cited by 25 publications

References 17 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

Post partum flare in a patient with hereditary angioedema

Management of hereditary angioedema in pregnant women: a review

Contact Info

Product

Resources

About