Matthies J, Hünseler C, Ehren R, Volland R, Körber F, Hoppe B, Weber L T, Habbig S
Division of Pediatric Nephrology, University Children's and Adolescent's Hospital, University Hospital of Cologne, Germany.
Division of Pediatric Gastroenterology, University Children's and Adolescent's Hospital of Cologne, Germany.
Klin Padiatr. 2016 Jul;228(4):181-8. doi: 10.1055/s-0042-108444. Epub 2016 Jun 13.
Shigatoxin-associated haemolytic uremic syndrome (STEC-HUS) is the most frequent cause of acute kidney injury in children worldwide. Extrarenal manifestations are the main determinants for both, short- and long-term prognosis of patients with STEC-HUS.
46 patients treated over the last 10 years for STEC-HUS in a single center.
This retrospective study analysed the incidence and outcome of extrarenal manifestations in our cohort of children with STEC-HUS. Risk factors for extrarenal involvement and adverse outcome were assessed by detailed chart review.
Eleven extrarenal manifestations occurred in 9/46 patients comprising 8 neurological, 2 gastro-intestinal, and 1 cardiovascular complication. One patient died from cerebral bleeding. Liver transplantation was required in a girl 18 months after HUS due to secondary sclerosing cholangitis. PATIENTS with extrarenal manifestations were significantly younger and presented with higher leucocyte counts and higher alanine aminotransferase levels at admission. Renal replacement therapy was necessary for a longer period than in patients without extrarenal complications.
Extrarenal manifestations occurred in about 20% of our patients with STEC-HUS. The identification of risk-factors will help to provide a better management of these patients which might also include novel treatment strategies like complement inhibition.
志贺毒素相关溶血尿毒综合征（STEC-HUS）是全球儿童急性肾损伤最常见的病因。肾外表现是STEC-HUS患者短期和长期预后的主要决定因素。
过去10年在单一中心接受治疗的46例STEC-HUS患儿。
这项回顾性研究分析了我们的STEC-HUS患儿队列中肾外表现的发生率和结局。通过详细的病历审查评估肾外受累和不良结局的危险因素。
9/46例患者出现11种肾外表现，包括8例神经系统并发症、2例胃肠道并发症和1例心血管并发症。1例患者死于脑出血。1名女孩在患溶血尿毒综合征18个月后因继发性硬化性胆管炎需要进行肝移植。出现肾外表现的患者年龄显著更小，入院时白细胞计数和丙氨酸转氨酶水平更高。与无肾外并发症的患者相比，需要进行更长时间的肾脏替代治疗。
我们的STEC-HUS患者中约20%出现了肾外表现。识别危险因素将有助于更好地管理这些患者，这可能还包括补体抑制等新的治疗策略。
