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药物疹伴嗜酸性粒细胞增多和全身症状综合征导致急性肝衰竭患儿的肝移植

Liver transplantation in a child with acute liver failure resulting from drug rash with eosinophilia and systemic symptoms syndrome.

作者信息

Song Seung Min, Cho Min Sung, Oh Seak Hee, Kim Kyung Mo, Park Young Seo, Kim Dae Yeon, Lee Sung Gyu

机构信息

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

出版信息

Korean J Pediatr. 2013 May;56(5):224-6. doi: 10.3345/kjp.2013.56.5.224. Epub 2013 May 28.

Abstract

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is characterized by a severe idiosyncratic reaction including rash and fever, often with associated hepatitis, arthralgias, lymph node enlargement, or hematologic abnormalities. The mortality rate is approximately 10%, primarily owing to liver failure with massive or multiple disseminated focal necrosis. Here, we report a case of a 14-year-old girl treated with vancomycin because of a wound infection by methicillin-resistant Staphylococcus aureus, who presented with non-specific symptoms, which progressed to acute liver failure, displaying the hallmarks of DRESS syndrome. With the presence of aggravated hepatic encephalopathy and azotemia, the patient was refractory to medical treatments, she received a living-donor liver transplantation, and a cure was achieved without any sign of recurrence. Vancomycin can be a cause of DRESS syndrome. A high index of suspicion and rapid diagnosis are necessary not to miss this potentially lethal disease.

摘要

药物性皮疹伴嗜酸性粒细胞增多和全身症状(DRESS)综合征的特征是严重的特异性反应,包括皮疹和发热,常伴有肝炎、关节痛、淋巴结肿大或血液学异常。死亡率约为10%,主要是由于肝衰竭伴大片或多发性弥漫性局灶性坏死。在此,我们报告一例14岁女孩,因耐甲氧西林金黄色葡萄球菌伤口感染接受万古霉素治疗,最初出现非特异性症状,随后进展为急性肝衰竭,表现出DRESS综合征的特征。由于出现严重肝性脑病和氮质血症,该患者对药物治疗无效,接受了活体供肝移植,最终治愈且无复发迹象。万古霉素可能是DRESS综合征的病因。高度怀疑并快速诊断对于不遗漏这种潜在致命疾病很有必要。

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