Bertholet-Thomas A, Ranchin B, King L-A, Bacchetta J, Belot A, Gillet Y, Collardeau-Frachon S, Cochat P
Inserm U820, centre de référence des maladies rénales rares « néphrogones », hôpital Femme-Mère-Enfant, hospices civils de Lyon, université Claude-Bernard Lyon 1, 59, boulevard Pinel, 69677 Bron cedex, France.
Arch Pediatr. 2011 Jul;18(7):823-30. doi: 10.1016/j.arcped.2011.04.001. Epub 2011 May 14.
Haemolytic uremic syndrome (HUS) is characterized by thrombotic microangiopathy with acute renal failure, haemolytic anaemia with schizocytes and thrombocytopenia. Typical forms (D(+) HUS) are caused by gastrointestinal infection with Escherichia coli species producing verotoxines (or Shiga toxins, STEC). It is estimated that 5-8 % of infected individuals will develop HUS following STEC infection. E. coli O157:H7 is the most commonly involved serotype and can lead to D(+) HUS in 15 % of young infected children. Vehicles of STEC transmission are contaminated food (ground beef, unpasteurised dairy products, unwashed and uncooked fruit and vegetables), person-to-person transmission and contact with farm animals with STEC. After an average incubation period of 3 to 8 days, patients develop painful bloody diarrhoea followed by systemic toxinemia. This may lead to thrombotic microangiopathy with endothelial damage and activation of local thrombosis. Since 1996, the Institut de Veille Sanitaire (InVS) centralises all notified French cases of D(+) HUS in children less than 15 years of age and investigates cases regrouped by time and place for the presence of STEC risk factors. The average annual incidence ranges between 0.6 and one for 100 000 children younger than 15 years and with a peak at 1 year of age. Fifty-one percent of HUS occur between June and September. Patients with a suspicion of STEC infection or bloody diarrhoea should not receive antibiotics, antimotility agents, narcotics and non-steroidal anti-inflammatory drugs. Maintenance optimal hydration provides nephroprotection. The management of HUS remains supportive. Dialysis was required for 46 % of HUS cases in 2009. For similar indication, peritoneal dialysis has to be a first choice treatment. Neurological injury is the most frequent non-renal complication and the first cause of death. Early initiation of plasmapheresis might improve the prognosis. Overall mortality rate ranges between 1 and 5 %. One third of patients suffer from long-term renal morbidity such as proteinuria, arterial hypertension and decrease of glomerular filtration rate. The longer the duration of anuria, the greater the risk of sequellae. Any patient with a history of HUS needs a long-term renal follow-up.
溶血尿毒综合征（HUS）的特征为血栓性微血管病伴急性肾衰竭、伴有裂体细胞的溶血性贫血和血小板减少。典型形式（D(+) HUS）由感染产生志贺毒素（或志贺样毒素，STEC）的大肠杆菌引起的胃肠道感染所致。据估计，5-8%的受感染个体在感染STEC后会发展为HUS。大肠杆菌O157:H7是最常涉及的血清型，可导致15%的受感染幼儿发生D(+) HUS。STEC的传播媒介为受污染的食物（碎牛肉、未经巴氏消毒的乳制品、未清洗和未煮熟的水果及蔬菜）、人传人传播以及与感染STEC的农场动物接触。平均潜伏期为3至8天后，患者会出现疼痛性血性腹泻，随后出现全身毒素血症。这可能导致伴有内皮损伤和局部血栓形成激活的血栓性微血管病。自1996年以来，法国卫生监测研究所（InVS）集中收集了所有15岁以下儿童D(+) HUS的报告病例，并对按时间和地点分组的病例进行调查，以确定是否存在STEC危险因素。15岁以下儿童的年平均发病率在每10万人中为0.6至1例，1岁时达到峰值。51%的HUS病例发生在6月至9月。疑似STEC感染或血性腹泻的患者不应使用抗生素、止泻剂、麻醉剂和非甾体抗炎药。维持最佳水合状态可提供肾脏保护。HUS的治疗仍以支持治疗为主。2009年，46%的HUS病例需要进行透析。对于类似指征，腹膜透析应作为首选治疗方法。神经损伤是最常见的非肾脏并发症和首要死亡原因。早期开始血浆置换可能会改善预后。总体死亡率在1%至5%之间。三分之一的患者患有长期肾脏疾病，如蛋白尿、动脉高血压和肾小球滤过率下降。无尿持续时间越长，后遗症风险越大。任何有HUS病史的患者都需要长期的肾脏随访。
