PMID- 22197274 OWN - NLM STAT- MEDLINE DCOM- 20120412 LR - 20171116 IS - 1097-6825 (Electronic) IS - 0091-6749 (Linking) VI - 129 IP - 2 DP - 2012 Feb TI - International consensus and practical guidelines on the gynecologic and obstetric management of female patients with hereditary angioedema caused by C1 inhibitor deficiency. PG - 308-20 LID - 10.1016/j.jaci.2011.11.025 [doi] AB - 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. CI - Copyright (c) 2012 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved. FAU - Caballero, Teresa AU - Caballero T AD - Servicio de Alergia, Hospital La Paz Health Research Institute (IdiPaz), Biomedical Research Network on Rare Diseases-U754 (CIBERER), Madrid, Spain. mteresa.caballero@idipaz.es FAU - Farkas, Henriette AU - Farkas H FAU - Bouillet, Laurence AU - Bouillet L FAU - Bowen, Tom AU - Bowen T FAU - Gompel, Anne AU - Gompel A FAU - Fagerberg, Christina AU - Fagerberg C FAU - Bjokander, Janne AU - Bjokander J FAU - Bork, Konrad AU - Bork K FAU - Bygum, Anette AU - Bygum A FAU - Cicardi, Marco AU - Cicardi M FAU - de Carolis, Caterina AU - de Carolis C FAU - Frank, Michael AU - Frank M FAU - Gooi, Jimmy H C AU - Gooi JH FAU - Longhurst, Hilary AU - Longhurst H FAU - Martinez-Saguer, Inmaculada AU - Martinez-Saguer I FAU - Nielsen, Erik Waage AU - Nielsen EW FAU - Obtulowitz, Krystina AU - Obtulowitz K FAU - Perricone, Roberto AU - Perricone R FAU - Prior, Nieves AU - Prior N CN - C-1-INH Deficiency Working Group LA - eng PT - Consensus Development Conference PT - Journal Article PT - Practice Guideline PT - Research Support, Non-U.S. Gov't DEP - 20111224 PL - United States TA - J Allergy Clin Immunol JT - The Journal of allergy and clinical immunology JID - 1275002 RN - 0 (Complement C1 Inactivator Proteins) RN - 0 (Complement C1 Inhibitor Protein) RN - 0 (SERPING1 protein, human) SB - IM MH - Breast Neoplasms/complications MH - Chemoprevention MH - Complement C1 Inactivator Proteins/*deficiency MH - Complement C1 Inhibitor Protein MH - Contraception MH - Delivery, Obstetric MH - Female MH - Genetic Counseling MH - Genital Diseases, Female/complications MH - *Hereditary Angioedema Types I and II/complications/diagnosis/drug therapy/genetics MH - Humans MH - Infant MH - Infant, Newborn MH - Lactation MH - Menopause MH - Menstruation MH - Pregnancy MH - *Pregnancy Complications, Cardiovascular/diagnosis/drug therapy MH - Prenatal Diagnosis EDAT- 2011/12/27 06:00 MHDA- 2012/04/13 06:00 CRDT- 2011/12/27 06:00 PHST- 2011/06/10 00:00 [received] PHST- 2011/10/27 00:00 [revised] PHST- 2011/11/21 00:00 [accepted] PHST- 2011/12/27 06:00 [entrez] PHST- 2011/12/27 06:00 [pubmed] PHST- 2012/04/13 06:00 [medline] AID - S0091-6749(11)01830-6 [pii] AID - 10.1016/j.jaci.2011.11.025 [doi] PST - ppublish SO - J Allergy Clin Immunol. 2012 Feb;129(2):308-20. doi: 10.1016/j.jaci.2011.11.025. Epub 2011 Dec 24.