Anesthetic management of living donor liver transplantation for complement factor H deficiency hemolytic uremic syndrome: a case report
Korean Journal of Anesthesiology 2014³â 66±Ç 6È£ p.481 ~ p.485
¹Ú¼®Èñ(Park Suk-Hee) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Anesthesiology and Pain Medicine
±è°©¼ö(Kim Gaab-Soo) - Sungkyunkwan University School of Medicine Samsung Medical Center Department of Anesthesiology and Pain Medicine
Abstract
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.
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Complement factor H, Hemolytic-uremic syndrome, Liver transplantation
KMID :
0356920140660060481
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