Department of Pediatrics, Medical University of Innsbruck, Innsbruck, Austria.
Research Institute for Developmental Medicine, Johannes Kepler University, Linz, Austria.
Pediatr Nephrol. 2024 Aug;39(8):2459-2465. doi: 10.1007/s00467-024-06355-z. Epub 2024 Apr 9.
Hemolytic uremic syndrome (HUS) is an important cause of acute kidney injury in children. HUS is known as an acute disease followed by complete recovery, but patients may present with kidney abnormalities after long periods of time. This study evaluates the long-term outcome of Shiga toxin-producing Escherichia coli-associated HUS (STEC-HUS) in pediatric patients, 10 years after the acute phase of disease to identify risk factors for long-term sequelae.
Over a 6-year period, 619 patients under 18 years of age with HUS (490 STEC-positive, 79%) were registered in Austria and Germany. Long-term follow-up data of 138 STEC-HUS-patients were available after 10 years for analysis.
A total of 66% (n = 91, 95% CI 0.57-0.73) of patients fully recovered showing no sequelae after 10 years. An additional 34% (n = 47, 95% CI 0.27-0.43) presented either with decreased glomerular filtration rate (24%), proteinuria (23%), hypertension (17%), or neurological symptoms (3%). Thirty had sequelae 1 year after STEC-HUS, and the rest presented abnormalities unprecedented at the 2-year (n = 2), 3-year (n = 3), 5-year (n = 3), or 10-year (n = 9) follow-up. A total of 17 patients (36.2%) without kidney abnormalities at the 1-year follow-up presented with either proteinuria, hypertension, or decreased eGFR in subsequent follow-up visits. Patients needing extracorporeal treatments during the acute phase were at higher risk of presenting symptoms after 10 years (p < 0.05).
Patients with STEC-HUS should undergo regular follow-up, for a minimum of 10 years following their index presentation, due to the risk of long-term sequelae of their disease. An initial critical illness, marked by need of kidney replacement therapy or plasma treatment may help predict poor long-term outcome.
溶血尿毒综合征（HUS）是儿童急性肾损伤的重要病因。HUS 通常被认为是一种在完全恢复后即结束的急性疾病，但患者在疾病的很长一段时间后可能会出现肾脏异常。本研究评估了志贺毒素产生型大肠杆菌相关性 HUS（STEC-HUS）在儿科患者中的长期预后，对疾病急性发作后 10 年的患者进行了分析，以确定长期后遗症的风险因素。
在 6 年的时间里，奥地利和德国共登记了 619 名年龄在 18 岁以下的 HUS 患者（490 名 STEC 阳性，79%）。10 年后，有 138 名 STEC-HUS 患者可获得长期随访数据进行分析。
66%（n=91，95%CI 0.57-0.73）的患者完全恢复，10 年后无后遗症。另外 34%（n=47，95%CI 0.27-0.43）的患者出现肾小球滤过率降低（24%）、蛋白尿（23%）、高血压（17%）或神经系统症状（3%）。30 例患者在 STEC-HUS 后 1 年出现后遗症，其余患者在 2 年（n=2）、3 年（n=3）、5 年（n=3）或 10 年（n=9）随访时出现异常。17 名在 1 年随访时无肾脏异常的患者在随后的随访中出现蛋白尿、高血压或肾小球滤过率降低。急性期间需要体外治疗的患者在 10 年后出现症状的风险更高（p<0.05）。
由于 STEC-HUS 疾病存在长期后遗症的风险，患者应在发病后至少 10 年进行定期随访。初始危重症，表现为需要肾脏替代治疗或血浆治疗，可能有助于预测不良的长期预后。
