Picard C, Burtey S, Bornet C, Curti C, Montana M, Vanelle P
Pharmacie usage intérieur, hôpital Timone, AP-HM, 13005 Marseille, France.
Centre de néphrologie et de transplantation rénale, hôpital de la Conception, AP-HM, 13005 Marseille, France.
Pathol Biol (Paris). 2015 Jun;63(3):136-43. doi: 10.1016/j.patbio.2015.03.001. Epub 2015 Apr 3.
Hemolytic uremic syndrome is a rare disease, frequently responsible for renal insufficiency in children. Recent findings have led to renewed interest in this pathology. The discovery of new gene mutations in the atypical form of HUS and the experimental data suggesting the involvement of the complement pathway in the typical form, open new perspectives for treatment. This review summarizes the current state of knowledge on both typical and atypical hemolytic uremic syndrome pathophysiology and examines new perspectives for treatment.
溶血尿毒综合征是一种罕见疾病，常导致儿童肾功能不全。近期的研究结果引发了对这种病症的新关注。非典型溶血尿毒综合征中新基因突变的发现以及提示补体途径参与典型溶血尿毒综合征的实验数据，为治疗开辟了新前景。本文综述了典型和非典型溶血尿毒综合征病理生理学的当前知识状态，并探讨了新的治疗前景。
