6533b7d3fe1ef96bd1260c01

RESEARCH PRODUCT

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

Caterina De CarolisMarco CicardiN. PriorTeresa CaballeroAnette BygumHenriette FarkasJ. GooiMichael M. FrankLaurence BouilletJanne BjökanderKrystina ObtulowitzKonrad BorkHilary LonghurstAnne GompelChristina FagerbergTom BowenRoberto PerriconeInmaculada Martinez-saguerErik Waage Nielsen

subject

Complement C1 Inactivator ProteinsAbortionCardiovascularEcallantidechemistry.chemical_compoundDelivery Obstetric; Complement C1 Inactivator Proteins; Humans; Infant Newborn; Breast Neoplasms; Genetic Counseling; Pregnancy; Lactation; Genital Diseases Female; Infant; Contraception; Hereditary Angioedema Types I and II; Menstruation; Pregnancy Complications Cardiovascular; Chemoprevention; Prenatal Diagnosis; Menopause; FemalePregnancyIcatibantPrenatal DiagnosisImmunology and AllergyfertilityHereditary Angioedema Types I and IItreatmentObstetricsVaginal deliveryMenstruationContraceptioncontraceptionGenital DiseasesHereditary angioedemaFemalepregnancyMenopausedeliverymedicine.symptomComplement C1 Inhibitor ProteinDeliverymedicine.drugmedicine.medical_specialtyPregnancy Complications CardiovascularImmunologyBreast NeoplasmsGenetic CounselingIntrauterine deviceChemopreventionbreast cancermedicineHumansLactationGynecologyPregnancygenetic counselingAngioedemabusiness.industryangioedemaInfant NewbornInfantObstetricDelivery ObstetricNewbornmedicine.diseasehereditary angioedemaPregnancy ComplicationsSettore MED/16 - ReumatologiachemistryC1 inhibitor deficiencybusinessGenital Diseases Female

description

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.

yearjournalcountryeditionlanguage
2012-02-01Journal of Allergy and Clinical Immunology
https://doi.org/10.1016/j.jaci.2011.11.025