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

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 eve...

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Veröffentlicht in:	Journal of allergy and clinical immunology 2012-02, Vol.129 (2), p.308-320
Hauptverfasser:	Caballero, Teresa, MD, PhD, Farkas, Henriette, MD, PhD, DSc, Bouillet, Laurence, MD, PhD, Bowen, Tom, MD, Gompel, Anne, MD, PhD, Fagerberg, Christina, MD, Bjökander, Janne, MD, Bork, Konrad, MD, Bygum, Anette, MD, Cicardi, Marco, MD, de Carolis, Caterina, MD, Frank, Michael, MD, Gooi, Jimmy H.C., MD, Longhurst, Hilary, MD, Martínez-Saguer, Inmaculada, MD, Nielsen, Erik Waage, MD, Obtulowitz, Krystina, MD, Perricone, Roberto, MD, Prior, Nieves, MD
Format:	Artikel
Sprache:	eng
Schlagworte:	Allergic diseases Allergy and Immunology Amino acids Androgens Angioedema Biological and medical sciences breast cancer Breast Neoplasms - complications C1 inhibitor deficiency Chemoprevention Complement C1 Inactivator Proteins - deficiency Complement C1 Inhibitor Protein Contraception delivery Delivery, Obstetric Drug dosages Family medical history FDA approval Female fertility Fundamental and applied biological sciences. Psychology Fundamental immunology Genetic Counseling Genital Diseases, Female - complications Gynecology. Andrology. Obstetrics hereditary angioedema Hereditary Angioedema Types I and II - complications Hereditary Angioedema Types I and II - diagnosis Hereditary Angioedema Types I and II - drug therapy Hereditary Angioedema Types I and II - genetics Humans Immunopathology Infant Infant, Newborn Lactation Mammary gland diseases Medical sciences Menopause Menstruation Obstetrics Plasma Pregnancy Pregnancy Complications, Cardiovascular - diagnosis Pregnancy Complications, Cardiovascular - drug therapy Prenatal Diagnosis Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis Skin allergic diseases. Stinging insect allergies Task forces treatment Tumors Womens health
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Zusammenfassung:	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.
ISSN:	0091-6749 1097-6825
DOI:	10.1016/j.jaci.2011.11.025