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KMID : 0191120140290030441
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Journal of Korean Medical Science
2014 Volume.29 No. 3 p.441 ~ p.444
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Living Donor Liver Transplantation for an Infant with Osteogenesis Imperfecta and Intrahepatic Cholestasis: Report of a Case
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Choi Young-Rok
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
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Abstract
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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
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KEYWORD
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Osteogenesis Imperfecta, Liver Transplantation, Cholestatic Liver Disease, Malignant Hyperthermia
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