Geramita Matthew A, Hofer Johannes, Cooper James, Moritz Michael L
Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA.
Department Zahn Mund und Kieferheilkunde und Mund Kiefer und Gesichtschirurgie, Medizinische Universitat Innsbruck, Innsbruck, Tirol, Austria.
BMJ Case Rep. 2017 Aug 30;2017:bcr-2017-221043. doi: 10.1136/bcr-2017-221043.
Shiga toxin-producing -associated haemolytic uraemic syndrome (STEC-HUS) is characterised by haemolytic anaemia, thrombocytopenia and acute kidney injury. Von Willebrand Factor (vWF) is an important mediator of normal thrombi formation and indirect evidence suggests that vWF may play an important role in Shiga toxin-induced thrombi formation. Clinical evidence supporting the role of vWF in STEC-HUS is lacking. A 10-year-old girl with type 1 von Willebrand Disease (vWD) had a mild case of STEC-HUS, with nadir haemoglobin 7.3 g/dL and platelet count 105×10 cells/L and peak serum creatinine 0.56 mg/L and lactate dehydrogenase 741 U/L. This is the first report of STEC-HUS in a patient with vWD. We speculate that the quantitative deficiency of vWF associated with type 1 vWD may have attenuated the course of disease by reducing platelet aggregation, complement activation and thrombi formation. This case adds to a growing literature supporting a link between vWF and STEC-HUS.
产志贺毒素相关溶血尿毒综合征（STEC-HUS）的特征为溶血性贫血、血小板减少和急性肾损伤。血管性血友病因子（vWF）是正常血栓形成的重要介质，间接证据表明vWF可能在志贺毒素诱导的血栓形成中起重要作用。缺乏支持vWF在STEC-HUS中作用的临床证据。一名患有1型血管性血友病（vWD）的10岁女孩患了轻度STEC-HUS，最低血红蛋白为7.3g/dL，血小板计数为105×10⁹细胞/L，血清肌酐峰值为0.56mg/L，乳酸脱氢酶为741U/L。这是vWD患者发生STEC-HUS的首例报告。我们推测，与1型vWD相关的vWF定量缺乏可能通过减少血小板聚集、补体激活和血栓形成而减轻了病程。该病例为支持vWF与STEC-HUS之间联系的不断增加的文献增添了内容。
