{"title":"血友病A患者复发性肛周脓肿的麻醉处理1例","authors":"Oktom Nurumbetova, Özer Tuç, N. Çekmen","doi":"10.4103/bjoa.bjoa_187_22","DOIUrl":null,"url":null,"abstract":"Hemophilia A is an x-linked recessive disorder characterized by an absent or reduced factor VIII (FVIII). It is a genetic deficiency that causes increased bleeding. It can be challenging for anesthesiologists to discover it in the perioperative management. This case report represents the successful management of a 25-year-old male patient having severe hemophilia A. We managed a 25-year-old man, 89 kg, 180 cm, with a recurring history of perianal abscesses. Previously at 13 years old, he experienced prolonged and excessive bleeding after a minor cut and injury. Following clinical and laboratory investigation, he was diagnosed with severe hemophilia A. Ever since, he used to take antihemophilic factor (AHF) VIII transfusion therapy and revealed severe disease with a positive inhibitor. No other coexisting disease was revealed during history-taking. We managed this case with general anesthesia because of the fact that he was hemophilic. We administered 2000 International Unit (IU) of recombinant Factor VIII (rFVIII) preoperatively as per the hematologist’s recommendation. We continued with 2000 IU of AHF FVIII transfusion after the surgery, but unfortunately, he redeveloped the abscess again. Another careful anesthesia and surgery were then conducted with a satisfactory result. The multidisciplinary team approach was provided to a positive outcome in these patients. Perioperative transfusion of AHF FVIII is essential for patients with hemophilia.","PeriodicalId":8691,"journal":{"name":"Bali Journal of Anesthesiology","volume":"6 1","pages":"243 - 246"},"PeriodicalIF":0.0000,"publicationDate":"2022-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"An anesthetic management of patient with hemophilia A undergoing recurrent perianal abscesses: A case report\",\"authors\":\"Oktom Nurumbetova, Özer Tuç, N. Çekmen\",\"doi\":\"10.4103/bjoa.bjoa_187_22\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Hemophilia A is an x-linked recessive disorder characterized by an absent or reduced factor VIII (FVIII). It is a genetic deficiency that causes increased bleeding. It can be challenging for anesthesiologists to discover it in the perioperative management. This case report represents the successful management of a 25-year-old male patient having severe hemophilia A. We managed a 25-year-old man, 89 kg, 180 cm, with a recurring history of perianal abscesses. Previously at 13 years old, he experienced prolonged and excessive bleeding after a minor cut and injury. Following clinical and laboratory investigation, he was diagnosed with severe hemophilia A. Ever since, he used to take antihemophilic factor (AHF) VIII transfusion therapy and revealed severe disease with a positive inhibitor. No other coexisting disease was revealed during history-taking. We managed this case with general anesthesia because of the fact that he was hemophilic. We administered 2000 International Unit (IU) of recombinant Factor VIII (rFVIII) preoperatively as per the hematologist’s recommendation. We continued with 2000 IU of AHF FVIII transfusion after the surgery, but unfortunately, he redeveloped the abscess again. Another careful anesthesia and surgery were then conducted with a satisfactory result. The multidisciplinary team approach was provided to a positive outcome in these patients. Perioperative transfusion of AHF FVIII is essential for patients with hemophilia.\",\"PeriodicalId\":8691,\"journal\":{\"name\":\"Bali Journal of Anesthesiology\",\"volume\":\"6 1\",\"pages\":\"243 - 246\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2022-10-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Bali Journal of Anesthesiology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/bjoa.bjoa_187_22\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Nursing\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Bali Journal of Anesthesiology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/bjoa.bjoa_187_22","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Nursing","Score":null,"Total":0}
引用次数: 0
摘要
血友病A是一种x连锁隐性疾病,其特征是缺乏或减少因子VIII(FVIII)。这是一种导致出血增加的遗传缺陷。对于麻醉师来说,在围手术期管理中发现它可能是一项挑战。本病例报告成功治疗了一名患有严重血友病a的25岁男性患者 千克,180 厘米,有肛周脓肿复发史。此前,他13岁时,在一次轻微割伤和受伤后,经历了长时间的过度出血。经过临床和实验室调查,他被诊断为严重的血友病A。从那以后,他曾接受抗血友病因子(AHF)VIII输血治疗,并发现患有严重的阳性抑制剂。病史采集期间未发现其他共存疾病。我们在全身麻醉下处理了这个病例,因为他是血友病患者。根据血液学家的建议,我们在术前给予2000国际单位(IU)的重组因子VIII(rFVIII)。手术后,我们继续输注2000 IU AHF FVIII,但不幸的是,他再次出现脓肿。随后进行了另一次仔细的麻醉和手术,结果令人满意。多学科团队方法为这些患者提供了积极的结果。血友病患者围手术期输注AHF FVIII是必不可少的。
An anesthetic management of patient with hemophilia A undergoing recurrent perianal abscesses: A case report
Hemophilia A is an x-linked recessive disorder characterized by an absent or reduced factor VIII (FVIII). It is a genetic deficiency that causes increased bleeding. It can be challenging for anesthesiologists to discover it in the perioperative management. This case report represents the successful management of a 25-year-old male patient having severe hemophilia A. We managed a 25-year-old man, 89 kg, 180 cm, with a recurring history of perianal abscesses. Previously at 13 years old, he experienced prolonged and excessive bleeding after a minor cut and injury. Following clinical and laboratory investigation, he was diagnosed with severe hemophilia A. Ever since, he used to take antihemophilic factor (AHF) VIII transfusion therapy and revealed severe disease with a positive inhibitor. No other coexisting disease was revealed during history-taking. We managed this case with general anesthesia because of the fact that he was hemophilic. We administered 2000 International Unit (IU) of recombinant Factor VIII (rFVIII) preoperatively as per the hematologist’s recommendation. We continued with 2000 IU of AHF FVIII transfusion after the surgery, but unfortunately, he redeveloped the abscess again. Another careful anesthesia and surgery were then conducted with a satisfactory result. The multidisciplinary team approach was provided to a positive outcome in these patients. Perioperative transfusion of AHF FVIII is essential for patients with hemophilia.