Wijnsma Kioa L, Schijvens Anne M, Rossen John W A, Kooistra-Smid A M D Mirjam, Schreuder Michiel F, van de Kar Nicole C A J
Department of Paediatric Nephrology, Radboud University Medical Center, Amalia Children's Hospital, PO Box 9101, 6500 HB, Nijmegen, The Netherlands.
Department of Medical Microbiology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9713 GZ, Groningen, The Netherlands.
Pediatr Nephrol. 2017 Jul;32(7):1263-1268. doi: 10.1007/s00467-017-3642-3. Epub 2017 Mar 25.
Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in children, with the majority of cases caused by an infection with Shiga toxin-producing Escherichia coli (STEC). Whereas O157 is still the predominant STEC serotype, non-O157 serotypes are increasingly associated with STEC-HUS. However, little is known about this emerging and highly diverse group of non-O157 serotypes. With supportive therapy, STEC-HUS is often self-limiting, with occurrence of chronic sequelae in just a small proportion of patients.
CASE DIAGNOSIS/TREATMENT: In this case report, we describe a 16-month-old boy with a highly severe and atypical presentation of STEC-HUS. Despite the presentation with multi-organ failure and extensive involvement of central nervous system due to extensive thrombotic microangiopathy (suggestive of atypical HUS), fecal diagnostics revealed an infection with the rare serotype: shiga toxin 2d-producing STEC O80:H2.
This report underlines the importance of STEC diagnostic tests in all children with HUS, including those with an atypical presentation, and emphasizes the importance of molecular and serotyping assays to estimate the virulence of an STEC strain.
溶血尿毒综合征（HUS）是儿童急性肾衰竭最常见的病因之一，大多数病例由产志贺毒素大肠杆菌（STEC）感染引起。虽然O157仍然是主要的STEC血清型，但非O157血清型与STEC-HUS的关联日益增加。然而，对于这一新兴且高度多样化的非O157血清型群体，人们知之甚少。通过支持性治疗，STEC-HUS通常具有自限性，仅有一小部分患者会出现慢性后遗症。
病例诊断/治疗：在本病例报告中，我们描述了一名16个月大的男孩，他患有高度严重且非典型的STEC-HUS。尽管因广泛血栓性微血管病出现多器官功能衰竭和中枢神经系统广泛受累（提示非典型HUS），粪便诊断显示感染了罕见血清型：产志贺毒素2d的STEC O80:H2。
本报告强调了对所有HUS患儿进行STEC诊断检测的重要性，包括那些非典型表现的患儿，并强调了分子和血清分型检测对于评估STEC菌株毒力的重要性。
