Living Donor Liver Transplantation for an Infant with Osteogenesis Imperfecta and Intrahepatic Cholestasis: Report of a Case

Osteogenesis imperfecta (OI) is a group of genetic disorders characterized by bone fragility and connective tissue manifestations. We report a successful liver transplantation (LT) in an 8-month-old boy with OI and cholestatic biliary cirrhosis. After 4 cycles of intravenous pamidronate, LT was perf...

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Detalles Bibliográficos
Autores principales: Choi, YoungRok, Yi, Nam-Joon, Ko, Jae Sung, Ko, Jung Min, Jin, Ung Sik, Kim, Hee Soo, Lee, Kook Hyun, Cho, Tae-Joon, Suh, Suk-Won, Yoo, Tae, Lee, Kwang-Woong, Suh, Kyung-Suk
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Academy of Medical Sciences 2014
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3945143/
https://www.ncbi.nlm.nih.gov/pubmed/24616597
http://dx.doi.org/10.3346/jkms.2014.29.3.441
Descripción
Sumario:Osteogenesis imperfecta (OI) is a group of genetic disorders characterized by bone fragility and connective tissue manifestations. We report a successful liver transplantation (LT) in an 8-month-old boy with OI and cholestatic biliary cirrhosis. After 4 cycles of intravenous pamidronate, LT was performed under intravenous anesthesia using a left lateral section from his mother without mechanical retractors. The operation time was 420 min and estimated blood loss was 520 mL requiring one unit of RBC transfusion. He was discharged without surgical complications. Therefore, LT should be considered for patients with end stage liver disease and OI under organic multidisciplinary cooperation. GRAPHICAL ABSTRACT: [Image: see text]

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