Mijatovic Dino, Blagaic Ana, Zupan Zeljko
Department of Anesthesiology and Critical Care, University Hospital Rijeka, Rijeka, Croatia.
F1000Res. 2014 Mar 27;3:79. doi: 10.12688/f1000research.2546.2. eCollection 2014.
Hemolytic-uremic syndrome (HUS) is a leading cause of acute renal failure in infants and young children. It is traditionally defined as a triad of acute renal failure, hemolytic anemia and thrombocytopenia that occur within a week after prodromal hemorrhagic enterocolitis. Severe cases can also be presented by acute respiratory distress syndrome (ARDS), toxic megacolon with ileus, pancreatitis, central nervous system (CNS) disorders and multiple organ failure (MOF).
A previously healthy 4-year old Caucasian girl developed acute renal failure, thrombocytopenia and hemolytic anemia following a short episode of abdominal pain and bloody diarrhea. By the end of the first week the diagnosis of the typical HUS was established. During the second week the disease progressed into MOF that included ileus, pancreatitis, hepatitis, coma and ARDS, accompanied by hemodynamic instability and extreme leukocytosis. Nonetheless, the girl made a complete recovery after one month of the disease. She was successfully treated in the intensive care unit and significant improvement was noticed after plasmapheresis and continuous veno-venous hemodialysis.
Early start of plasmapheresis and meticulous supportive treatment in the intensive care unit, including renal placement therapy, may be the therapy of choice in severe cases of HUS presented by MOF. Monitoring of prognostic factors is important for early performance of appropriate diagnostic and therapeutical interventions.
溶血尿毒综合征(HUS)是婴幼儿急性肾衰竭的主要病因。传统上,它被定义为前驱性出血性小肠结肠炎后一周内出现的急性肾衰竭、溶血性贫血和血小板减少三联征。严重病例还可能表现为急性呼吸窘迫综合征(ARDS)、中毒性巨结肠伴肠梗阻、胰腺炎、中枢神经系统(CNS)疾病和多器官功能衰竭(MOF)。
一名此前健康的4岁白人女孩在经历短暂的腹痛和血性腹泻后,出现了急性肾衰竭、血小板减少和溶血性贫血。在第一周结束时,确诊为典型的HUS。在第二周,病情发展为MOF,包括肠梗阻、胰腺炎、肝炎、昏迷和ARDS,伴有血流动力学不稳定和极度白细胞增多。尽管如此,该女孩在患病一个月后完全康复。她在重症监护病房得到了成功治疗,在进行血浆置换和持续静脉-静脉血液透析后,病情有了显著改善。
对于由MOF引起的严重HUS病例,早期开始血浆置换并在重症监护病房进行细致的支持治疗,包括肾脏替代治疗,可能是首选的治疗方法。监测预后因素对于早期进行适当的诊断和治疗干预很重要。