Department of Anesthesiology and Pain Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
Korean J Anesthesiol. 2014 Jun;66(6):481-5. doi: 10.4097/kjae.2014.66.6.481. Epub 2014 Jun 26.
We experienced a living donor liver transplantation for a 26-month-old girl with complement factor H deficiency. Complement factor H is a plasma protein that regulates the activity of the complement pathway. Complement overactivity induced by complement factor H deficiency is associated with atypical hemolytic uremic syndrome. Liver transplantation can be the proper treatment for this condition. During the liver transplantation of these patients, prevention of the complement overactivation is necessary. Minimizing complement activation, through the use of modalities such as plasma exchange before the surgery and transfusion of fresh frozen plasma throughout the entire perioperative period, may be the key for successful liver transplantation in these patients.
我们为一名 26 个月大的补体因子 H 缺乏症女孩进行了活体供肝移植。补体因子 H 是一种调节补体途径活性的血浆蛋白。补体因子 H 缺乏引起的补体过度激活与非典型溶血尿毒综合征有关。肝移植是治疗这种疾病的合适方法。在这些患者的肝移植过程中,需要预防补体过度激活。通过在手术前使用血浆置换等方法,以及在整个围手术期输注新鲜冷冻血浆,最大限度地减少补体激活,可能是这些患者成功进行肝移植的关键。