Department of Pediatric Nephrology and Transplantation, New Children's Hospital, University of Helsinki and Helsinki University Hospital|, P.O. Box 347, 00029 HUS, Helsinki, Finland.
Department of Health Security, Finnish Institute for Health and Welfare (THL), Helsinki, Finland.
Pediatr Nephrol. 2020 Sep;35(9):1749-1759. doi: 10.1007/s00467-020-04560-0. Epub 2020 Apr 22.
Hemolytic uremic syndrome (HUS) is a multisystemic disease. In a nationwide study, we characterized the incidence, clinical course, and prognosis of HUS caused by Shiga toxin (Stx)-producing Escherichia coli (STEC) strains with emphasis on risk factors, disease severity, and long-term outcome.
The data on pediatric HUS patients from 2000 to 2016 were collected from the medical records. STEC isolates from fecal cultures of HUS and non-HUS patients were collected from the same time period and characterized by whole genome sequencing analysis.
Fifty-eight out of 262 culture-positive cases developed verified (n = 58, 22%) STEC-HUS. Another 29 cases had probable STEC-HUS, the annual incidence of STEC-HUS being 0.5 per 100,000 children. Eleven different serogroups were detected, O157 being the most common (n = 37, 66%). Age under 3 years (OR 2.4), stx2 (OR 9.7), and stx2a (OR 16.6) were found to be risk factors for HUS. Fifty-five patients (63%) needed dialysis. Twenty-nine patients (33%) developed major neurological symptoms. Complete renal recovery was observed in 57 patients after a median 4.0 years of follow-up. Age under 3 years, leukocyte count over 20 × 10/L, and need for dialysis were predictive factors for poor renal outcome.
Age under 3 years, stx2, and stx2a were risk factors for HUS in STEC-positive children. However, serogroup or stx types did not predict the renal outcome or major CNS symptoms.
溶血性尿毒症综合征（HUS）是一种多系统疾病。在一项全国性研究中，我们对产志贺毒素（Stx）大肠杆菌（STEC）菌株引起的 HUS 的发病率、临床病程和预后进行了特征描述，重点关注了危险因素、疾病严重程度和长期结局。
我们从病历中收集了 2000 年至 2016 年期间儿科 HUS 患者的数据。同时从 HUS 和非 HUS 患者的粪便培养中收集 STEC 分离株，并通过全基因组测序分析进行特征描述。
在 262 例培养阳性病例中，有 58 例（22%）发展为确诊（n=58，22%）STEC-HUS。另有 29 例为可能的 STEC-HUS，STEC-HUS 的年发病率为每 10 万儿童 0.5 例。共检测到 11 种不同的血清群，O157 最为常见（n=37，66%）。年龄小于 3 岁（OR 2.4）、stx2（OR 9.7）和 stx2a（OR 16.6）被认为是 HUS 的危险因素。55 例患者（63%）需要透析。29 例患者（33%）出现主要神经症状。57 例患者在中位随访 4.0 年后完全恢复肾功能。年龄小于 3 岁、白细胞计数超过 20×10/L 和需要透析是肾功能不良的预测因素。
年龄小于 3 岁、stx2 和 stx2a 是 STEC 阳性儿童发生 HUS 的危险因素。然而，血清群或 stx 类型不能预测肾脏结局或主要 CNS 症状。
