{"title":"Wiskott-Aldrich综合征:由肝脏、小肠肠系膜和肾脏内的动脉瘤引起的危及生命的出血,需要手术和放射治疗。","authors":"W Loan, K McCune, B Kelly, R Maxwell","doi":"","DOIUrl":null,"url":null,"abstract":"<p><p>Wiskott-Aldrich syndrome (WAS) is a rare, generally X-linked recessive condition, originally described by Wiskott in 1937 as a triad of discharging ears, eczema and thrombocytopoenia. Aldrich included bloody diarrhoea in his report of 1954, with severe immunodeficiency and predisposition to malignancy being recognised subsequently. The incidence currently quoted is approximately 4 per million live male births, although there is some regional variation. We report the case of a long-term survivor who had massive haemorrhage from an intrahepatic aneurysm and, on a separate occasion, the right kidney.</p>","PeriodicalId":76058,"journal":{"name":"Journal of the Royal College of Surgeons of Edinburgh","volume":"45 5","pages":"326-8"},"PeriodicalIF":0.0000,"publicationDate":"2000-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Wiskott-Aldrich syndrome: life-threatening haemorrhage from aneurysms within the liver, small bowel mesentery and kidney, requiring both surgical and radiological intervention.\",\"authors\":\"W Loan, K McCune, B Kelly, R Maxwell\",\"doi\":\"\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Wiskott-Aldrich syndrome (WAS) is a rare, generally X-linked recessive condition, originally described by Wiskott in 1937 as a triad of discharging ears, eczema and thrombocytopoenia. Aldrich included bloody diarrhoea in his report of 1954, with severe immunodeficiency and predisposition to malignancy being recognised subsequently. The incidence currently quoted is approximately 4 per million live male births, although there is some regional variation. We report the case of a long-term survivor who had massive haemorrhage from an intrahepatic aneurysm and, on a separate occasion, the right kidney.</p>\",\"PeriodicalId\":76058,\"journal\":{\"name\":\"Journal of the Royal College of Surgeons of Edinburgh\",\"volume\":\"45 5\",\"pages\":\"326-8\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2000-10-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of the Royal College of Surgeons of Edinburgh\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of the Royal College of Surgeons of Edinburgh","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Wiskott-Aldrich syndrome: life-threatening haemorrhage from aneurysms within the liver, small bowel mesentery and kidney, requiring both surgical and radiological intervention.
Wiskott-Aldrich syndrome (WAS) is a rare, generally X-linked recessive condition, originally described by Wiskott in 1937 as a triad of discharging ears, eczema and thrombocytopoenia. Aldrich included bloody diarrhoea in his report of 1954, with severe immunodeficiency and predisposition to malignancy being recognised subsequently. The incidence currently quoted is approximately 4 per million live male births, although there is some regional variation. We report the case of a long-term survivor who had massive haemorrhage from an intrahepatic aneurysm and, on a separate occasion, the right kidney.