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

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 t...

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Detalles Bibliográficos
Autores principales: Steinbrück, Klaus, Enne, Marcelo, Fernandes, Reinaldo, Martinho, Jose M., Pacheco-Moreira, Lúcio F.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: International Scholarly Research Network 2011
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3195350/
https://www.ncbi.nlm.nih.gov/pubmed/22084740
http://dx.doi.org/10.5402/2011/106487
Descripción
Sumario: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.