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补体因子 H 缺乏性溶血尿毒综合征活体肝移植的麻醉管理：病例报告。

Anesthetic management of living donor liver transplantation for complement factor H deficiency hemolytic uremic syndrome: a case report.

机构信息

Department of Anesthesiology and Pain Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

出版信息

Korean J Anesthesiol. 2014 Jun;66(6):481-5. doi: 10.4097/kjae.2014.66.6.481. Epub 2014 Jun 26.

DOI:10.4097/kjae.2014.66.6.481
PMID:25006375
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4085272/
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.

摘要

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b475/4085272/b70bdf0ee726/kjae-66-481-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b475/4085272/b70bdf0ee726/kjae-66-481-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b475/4085272/b70bdf0ee726/kjae-66-481-g001.jpg

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引用本文的文献

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2
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Pediatr Nephrol. 2016 Jan;31(1):15-39. doi: 10.1007/s00467-015-3076-8. Epub 2015 Apr 11.

本文引用的文献

1
Liver-kidney transplantation to cure atypical hemolytic uremic syndrome.肝肾移植治疗非典型溶血性尿毒症综合征。
J Am Soc Nephrol. 2009 May;20(5):940-9. doi: 10.1681/ASN.2008080906. Epub 2008 Dec 17.
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Successful split liver-kidney transplant for factor H associated hemolytic uremic syndrome.成功进行针对因子H相关性溶血尿毒综合征的肝肾联合移植
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Successful liver-kidney transplantation in two children with aHUS caused by a mutation in complement factor H.
两名因补体因子H突变导致溶血尿毒综合征的儿童成功接受肝肾移植。
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Epidemiology, clinical presentation, and pathophysiology of atypical and recurrent hemolytic uremic syndrome.非典型和复发性溶血尿毒综合征的流行病学、临床表现及病理生理学
Semin Thromb Hemost. 2006 Mar;32(2):113-20. doi: 10.1055/s-2006-939767.
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Hemolytic uremic syndrome: a fatal outcome after kidney and liver transplantation performed to correct factor h gene mutation.溶血性尿毒症综合征：为纠正因子H基因突变而进行肾和肝移植后的致命结局。
Am J Transplant. 2005 May;5(5):1146-50. doi: 10.1111/j.1600-6143.2005.00783.x.
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Attempted treatment of factor H deficiency by liver transplantation.尝试通过肝移植治疗H因子缺乏症。
Pediatr Nephrol. 2004 Apr;19(4):454-8. doi: 10.1007/s00467-003-1371-2. Epub 2004 Feb 24.
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Complement membrane attack complex and hemodynamic changes during human orthotopic liver transplantation.人类原位肝移植过程中的补体膜攻击复合物与血流动力学变化
Liver Transpl. 2004 Feb;10(2):273-8. doi: 10.1002/lt.20061.
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Combined kidney and liver transplantation for familial haemolytic uraemic syndrome.家族性溶血性尿毒症综合征的肝肾联合移植
Lancet. 2002 May 11;359(9318):1671-2. doi: 10.1016/S0140-6736(02)08560-4.
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Successful plasma therapy in hemolytic uremic syndrome with factor H deficiency.在伴有补体因子H缺乏的溶血性尿毒症综合征中成功应用血浆疗法。
Pediatr Nephrol. 2001 Jul;16(7):554-6. doi: 10.1007/s004670100609.