Síndrome urémico hemolítico atípico en el embarazo

作者: Ángel Augusto Pérez-Calatayud , Jesús Carlos Briones-Garduño , Mercedes del Pilar Álvarez-Goris , Ricardo Sánchez Zamora , Angélica A. Torres Aguilar

DOI: 10.1016/J.CIRCIR.2016.02.001

关键词:

摘要: Atypical haemolytic uraemic syndrome is one of the main variants thrombotic microangiopathy, and characterized by excessive complement activation in microvasculature. It also characterised clinical triad; non-immune anaemia, thrombocytopenia, acute renal failure. In addition, 60% patients have mutations genes encoding regulators (factor H, factor I, membrane cofactor proteins, thrombomodulin), activators B C3), as well autoantibodies against H. Multiple factors are required for disease to manifest itself, including a trigger gene with adequate penetration. Being differential diagnoses preeclampsia- eclampsia HELLP means that clinician must be familiar due its high mortality, which can modified early diagnosis comprehensive treatment.

参考文章(48)
David Kavanagh, Tim H. Goodship, Anna Richards, Atypical Hemolytic Uremic Syndrome Seminars in Nephrology. ,vol. 33, pp. 508- 530 ,(2013) , 10.1016/J.SEMNEPHROL.2013.08.003
Andreas Kourouklaris, Kyriakos Ioannou, Ioannis Athanasiou, Alexia Panagidou, Kiproulla Demetriou, Michalis Zavros, Postpartum thrombotic microangiopathy revealed as atypical hemolytic uremic syndrome successfully treated with eculizumab: a case report Journal of Medical Case Reports. ,vol. 8, pp. 307- 307 ,(2014) , 10.1186/1752-1947-8-307
Fadi Fakhouri, Lubka Roumenina, François Provot, Marion Sallée, Sophie Caillard, Lionel Couzi, Marie Essig, David Ribes, Marie-Agnès Dragon-Durey, Frank Bridoux, Eric Rondeau, Veronique Frémeaux-Bacchi, Pregnancy-Associated Hemolytic Uremic Syndrome Revisited in the Era of Complement Gene Mutations Journal of The American Society of Nephrology. ,vol. 21, pp. 859- 867 ,(2010) , 10.1681/ASN.2009070706
Chantal Loirat, Véronique Frémeaux-Bacchi, Atypical hemolytic uremic syndrome Orphanet Journal of Rare Diseases. ,vol. 6, pp. 60- 60 ,(2011) , 10.1186/1750-1172-6-60
Cines Db, McCrae Kr, Thrombotic microangiopathy during pregnancy. Seminars in Hematology. ,vol. 34, pp. 148- 158 ,(1997)
Fremeaux-Bacchi, Loirat C, Fakhouri F, [Hemolytic-uremic syndrome: what is the mechanism?]. La Revue du praticien. ,vol. 58, pp. 2093- ,(2008)
Dezra White, Ethan Natelson, Recurrent thrombotic thrombocytopenic purpura in early pregnancy: effect of uterine evacuation. Obstetrics & Gynecology. ,vol. 66, ,(1985)
Gregor Verhoef, Hans E Johnsen, Francesca Gaia Rossi, Antonio Salar, Lisa Hamilton, W. Marieke Schoonen, Beatriz Pujol, Ulrich Duhrsen, An Observational Study of Anaemia Management in Patients with NHL Receiving CHOP-14 or -21 (With or Without Rituximab) Blood. ,vol. 112, pp. 4948- 4948 ,(2008) , 10.1182/BLOOD.V112.11.4948.4948