Medical Intensive Care Unit, Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris, Paris, France.
Department of Renal Intensive Care and Transplantation, Hôpital Tenon, Assistance Publique Hôpitaux de Paris, Paris, France.
Crit Care Med. 2018 Sep;46(9):e904-e911. doi: 10.1097/CCM.0000000000003292.
Thrombotic microangiopathy syndromes are a heterogeneous group of severe diseases that often require ICU admission. Prompt initiation of targeted therapies is required for atypical hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, whereas there is no specific consensus therapy for Shiga toxin-associated hemolytic uremic syndrome. We sought to compare the characteristics of Shiga toxin-associated hemolytic uremic syndrome, atypical hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura patients at admission in the ICU to allow early differentiation of Shiga toxin-associated hemolytic uremic syndrome from other thrombotic microangiopathy syndromes and help to tailor initial treatment.
Retrospective cohort study.
Two ICUs part of the French reference center for thrombotic microangiopathy syndromes.
Adult patients presenting with features of thrombotic microangiopathy syndromes. Other causes than Shiga toxin-associated hemolytic uremic syndrome, atypical hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura were excluded.
None.
From September 2003 to January 2017, 236 thrombotic microangiopathy syndrome patients were admitted, including 12 Shiga toxin-associated hemolytic uremic syndrome, 21 atypical hemolytic uremic syndrome, and 91 thrombotic thrombocytopenic purpura. Shiga toxin-associated hemolytic uremic syndrome patients were older than other thrombotic microangiopathy syndromes patients (64 yr [interquartile range, 50-72 yr] vs 42 yr [31-54 yr]; p = 0.007) and presented with more frequent digestive symptoms (92% vs 42%; p < 0.001), especially nonbloody diarrhea and vomiting. Biologically, Shiga toxin-associated hemolytic uremic syndrome patients displayed higher fibrinogen (490 mg/dL [460-540 mg/dL] vs 320 mg/dL [240-410 mg/dL]; p = 0.003) and creatinine levels (2.59 mg/dL [2.12-3.42 mg/dL] vs 1.26 mg/dL [0.61-1.90 mg/dL]; p < 0.001), and less marked anemia (hemoglobin level, 9.7 g/dL [8.7-11.9 g/dL] vs 7.7 g/dL [6.3-9.1 g/dL]; p < 0.001). Forty-two percent (n = 5) required renal replacement therapy, and 83% (n = 10) were treated with plasma exchange before the distinction from other thrombotic microangiopathy syndromes could be made.
Adult Shiga toxin-associated hemolytic uremic syndrome patients are older, present more frequently with digestive symptoms and display higher hemoglobin and fibrinogen levels than other thrombotic microangiopathy syndromes. However, overlap across the three thrombotic microangiopathy syndromes remains substantial, putting forward the need to implement early plasma therapy until thrombotic thrombocytopenic purpura and atypical hemolytic uremic syndrome can be ruled out.
血栓性微血管病综合征是一组严重疾病，常需要入住重症监护病房。对于非典型溶血尿毒综合征和血栓性血小板减少性紫癜，需要及时启动靶向治疗，而对于志贺毒素相关性溶血尿毒综合征则没有特定的共识治疗方法。我们旨在比较志贺毒素相关性溶血尿毒综合征、非典型溶血尿毒综合征和血栓性血小板减少性紫癜患者在入住重症监护病房时的特征，以便早期区分志贺毒素相关性溶血尿毒综合征与其他血栓性微血管病综合征，并有助于制定初始治疗方案。
回顾性队列研究。
两个属于法国血栓性微血管病综合征参考中心的重症监护病房。
表现为血栓性微血管病综合征特征的成年患者。排除了除志贺毒素相关性溶血尿毒综合征、非典型溶血尿毒综合征和血栓性血小板减少性紫癜以外的其他病因。
无。
从 2003 年 9 月至 2017 年 1 月，共有 236 例血栓性微血管病综合征患者入院，包括 12 例志贺毒素相关性溶血尿毒综合征、21 例非典型溶血尿毒综合征和 91 例血栓性血小板减少性紫癜。志贺毒素相关性溶血尿毒综合征患者比其他血栓性微血管病综合征患者年龄更大（64 岁[四分位距，50-72 岁] vs 42 岁[31-54 岁]；p=0.007），且更常出现消化系统症状（92% vs 42%；p<0.001），尤其是无血腹泻和呕吐。在生物学方面，志贺毒素相关性溶血尿毒综合征患者的纤维蛋白原水平更高（490mg/dL[460-540mg/dL] vs 320mg/dL[240-410mg/dL]；p=0.003）和肌酐水平更高（2.59mg/dL[2.12-3.42mg/dL] vs 1.26mg/dL[0.61-1.90mg/dL]；p<0.001），且贫血程度较轻（血红蛋白水平，9.7g/dL[8.7-11.9g/dL] vs 7.7g/dL[6.3-9.1g/dL]；p<0.001）。42%（n=5）需要肾脏替代治疗，在能够明确与其他血栓性微血管病综合征的区别之前，83%（n=10）接受了血浆置换治疗。
成年志贺毒素相关性溶血尿毒综合征患者年龄较大，更常出现消化系统症状，且血红蛋白和纤维蛋白原水平较高，但三种血栓性微血管病综合征之间仍存在明显重叠，这就需要在能够排除血栓性血小板减少性紫癜和非典型溶血尿毒综合征之前，及早实施血浆治疗。
