Living Donor Liver Transplantation for Caroli's Disease: A Report of Two Cases.

ISRN surgery Pub Date : 2011-01-01 Epub Date: 2011-04-27 DOI:10.5402/2011/106487
Klaus Steinbrück, Marcelo Enne, Reinaldo Fernandes, Jose M Martinho, Lúcio F Pacheco-Moreira
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引用次数: 4

Abstract

Caroli's disease (CD) is a rare autosomal recessive disorder characterized by intrahepatic cystic dilatation of the bile ducts. Patients with bilobar or progressive disease may require orthotopic liver transplantation (OLT). In the MELD era, living donor liver transplantation (LDLT) raised as the ultimate treatment option for these patients, once their MELD score is usually low. Herein, we describe 2 cases of patients (a 2-year-old girl and a 19-year-old teenager) that successfully underwent LDLT as a treatment for diffuse CD. The good postoperative courses of the two cases indicate that LDLT is a feasible option in the treatment of this disorder, even in complicated or early age patients.

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活体肝移植治疗卡罗里病2例报告
卡罗里氏病(CD)是一种罕见的常染色体隐性遗传病,以肝内胆管囊性扩张为特征。双叶或进行性疾病的患者可能需要原位肝移植(OLT)。在MELD时代,当这些患者的MELD评分通常较低时,活体供肝移植(LDLT)被作为最终的治疗选择。在本文中,我们描述了2例患者(一名2岁的女孩和一名19岁的青少年)成功接受LDLT作为弥漫性CD的治疗。这两例患者良好的术后过程表明LDLT是治疗这种疾病的可行选择,即使是复杂或早期患者。
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