title: International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency.
abstract: BACKGROUND
  There are a limited number of publications on the management of gynecologic/obstetric events in female patients with hereditary angioedema caused by C1 inhibitor deficiency (HAE-C1-INH).
  
  
  OBJECTIVE
  We sought to elaborate guidelines for optimizing the management of gynecologic/obstetric events in female patients with HAE-C1-INH.
  
  
  METHODS
  A roundtable discussion took place at the 6th C1 Inhibitor Deficiency Workshop (May 2009, Budapest, Hungary). A review of related literature in English was performed.
  
  
  RESULTS
  Contraception: Estrogens should be avoided. Barrier methods, intrauterine devices, and progestins can be used. Pregnancy: Attenuated androgens are contraindicated and should be discontinued before attempting conception. Plasma-derived human C1 inhibitor concentrate (pdhC1INH) is preferred for acute treatment, short-term prophylaxis, or long-term prophylaxis. Tranexamic acid or virally inactivated fresh frozen plasma can be used for long-term prophylaxis if human plasma-derived C1-INH is not available. No safety data are available on icatibant, ecallantide, or recombinant human C1-INH (rhC1INH). Parturition: Complications during vaginal delivery are rare. Prophylaxis before labor and delivery might not be clinically indicated, but pdhC1INH therapeutic doses (20 U/kg) should be available. Nevertheless, each case should be treated based on HAE-C1-INH symptoms during pregnancy and previous labors. pdhC1INH prophylaxis is advised before forceps or vacuum extraction or cesarean section. Regional anesthesia is preferred to endotracheal intubation. Breast cancer: Attenuated androgens should be avoided. Antiestrogens can worsen angioedema symptoms. In these cases anastrozole might be an alternative. Other issues addressed include special features of HAE-C1-INH treatment in female patients, genetic counseling, infertility, abortion, lactation, menopause treatment, and endometrial cancer.
  
  
  CONCLUSIONS
  A consensus for the management of female patients with HAE-C1-INH is presented.
authors.affiliation: Servicio de Alergia, Hospital La Paz Health Research Institute (IdiPaz), Biomedical Research Network on Rare Diseases-U754 (CIBERER), Madrid, Spain. mteresa.caballero@idipaz.es
authors.forename: Teresa
authors.initials: T
authors.lastname: Caballero
authors.forename: Henriette
authors.initials: H
authors.lastname: Farkas
authors.forename: Laurence
authors.initials: L
authors.lastname: Bouillet
authors.forename: Tom
authors.initials: T
authors.lastname: Bowen
authors.forename: Anne
authors.initials: A
authors.lastname: Gompel
authors.forename: Christina
authors.initials: C
authors.lastname: Fagerberg
authors.forename: Janne
authors.initials: J
authors.lastname: Bjökander
authors.forename: Konrad
authors.initials: K
authors.lastname: Bork
authors.forename: Anette
authors.initials: A
authors.lastname: Bygum
authors.forename: Marco
authors.initials: M
authors.lastname: Cicardi
authors.forename: Caterina
authors.initials: C
authors.lastname: de Carolis
authors.forename: Michael
authors.initials: M
authors.lastname: Frank
authors.forename: Jimmy H C
authors.initials: JH
authors.lastname: Gooi
authors.forename: Hilary
authors.initials: H
authors.lastname: Longhurst
authors.forename: Inmaculada
authors.initials: I
authors.lastname: Martínez-Saguer
authors.forename: Erik Waage
authors.initials: EW
authors.lastname: Nielsen
authors.forename: Krystina
authors.initials: K
authors.lastname: Obtulowitz
authors.forename: Roberto
authors.initials: R
authors.lastname: Perricone
authors.forename: Nieves
authors.initials: N
authors.lastname: Prior
mesh_terms: D001943:Breast Neoplasms / Q000150:complications; D018890:Chemoprevention; D003174:Complement C1 Inactivator Proteins / Q000172:deficiency*; D050718:Complement C1 Inhibitor Protein; D003267:Contraception; D036861:Delivery, Obstetric; D005260:Female; D005817:Genetic Counseling; D005831:Genital Diseases, Female / Q000150:complications; D056829:Hereditary Angioedema Types I and II / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007774:Lactation; D008593:Menopause; D008598:Menstruation; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000175:diagnosis / Q000188:drug therapy; D011296:Prenatal Diagnosis
chemical_list: D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; C469952:SERPING1 protein, human