Pérez-Calatayud Ángel Augusto, Briones-Garduño Jesús Carlos, Álvarez-Goris Mercedes Del Pilar, Sánchez Zamora Ricardo, Torres Aguilar Angélica A, Mendoza-Mórales Rosa Elba
Medicina Crítica en Obstetricia, Hospital General de México Dr. Eduardo Liceaga, Ciudad de México, México.
Medicina Crítica en Obstetricia, Hospital General de México Dr. Eduardo Liceaga, Ciudad de México, México.
Cir Cir. 2016 Jul-Aug;84(4):344-9. doi: 10.1016/j.circir.2016.02.001. Epub 2016 Mar 19.
Atypical haemolytic uraemic syndrome is one of the main variants of thrombotic microangiopathy, and is characterized by excessive complement activation in the microvasculature. It is also characterised by the clinical triad; non-immune haemolytic anaemia, thrombocytopenia, and acute renal failure. In addition, 60% of patients have mutations in the genes encoding complement regulators (factor H, factor I, membrane cofactor proteins, and thrombomodulin), activators (factor B and C3), as well as autoantibodies against factor H. Multiple factors are required for the disease to manifest itself, including a trigger and gene mutations with adequate penetration. Being one of the differential diagnoses of preeclampsia- eclampsia and HELLP syndrome means that the clinician must be familiar with the disease due to its high mortality, which can be modified with early diagnosis and comprehensive treatment.
非典型溶血性尿毒症综合征是血栓性微血管病的主要变异型之一,其特征是微血管系统中补体过度激活。它还具有临床三联征的特点,即非免疫性溶血性贫血、血小板减少和急性肾衰竭。此外,60%的患者在编码补体调节因子(H因子、I因子、膜辅助蛋白和血栓调节蛋白)、激活剂(B因子和C3)的基因以及针对H因子的自身抗体中存在突变。该疾病的显现需要多种因素,包括触发因素和具有足够外显率的基因突变。作为子痫前期-子痫和HELLP综合征的鉴别诊断之一,意味着临床医生必须熟悉该疾病,因为其死亡率很高,而早期诊断和综合治疗可以改善这种情况。