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Korean Academy of Medical Sciences, Journal of Korean Medical Science, 3(29), p. 441, 2014

DOI: 10.3346/jkms.2014.29.3.441

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

Journal article published in 2014 by YoungRok Choi ORCID, Nam-Joon Yi ORCID, Jae Sung Ko ORCID, Jung Min Ko ORCID, Ung Sik Jin ORCID, Hee Soo Kim, Kook Hyun Lee, Tae-Joon Cho ORCID, Suk-Won Suh ORCID, Tae Yoo ORCID, Kwang-Woong Lee ORCID, Kyung-Suk Suh
This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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Abstract

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.