• J. Korean Med. Sci. · Mar 2014

    Case Reports

    Living donor liver transplantation for an infant with osteogenesis imperfecta and intrahepatic cholestasis: report of a case.

    • YoungRok Choi, Nam-Joon Yi, Jae Sung Ko, Jung Min Ko, Ung Sik Jin, Hee Soo Kim, Kook Hyun Lee, Tae-Joon Cho, Suk-Won Suh, Tae Yoo, Kwang-Woong Lee, and Kyung-Suk Suh.
    • Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.
    • J. Korean Med. Sci. 2014 Mar 1; 29 (3): 441-4.

    AbstractOsteogenesis 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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