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


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.

SUBMITTER: Choi Y 

PROVIDER: S-EPMC3945143 | biostudies-literature | 2014 Mar

REPOSITORIES: biostudies-literature

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

Choi YoungRok Y   Yi Nam-Joon NJ   Ko Jae Sung JS   Ko Jung Min JM   Jin Ung Sik US   Kim Hee Soo HS   Lee Kook Hyun KH   Cho Tae-Joon TJ   Suh Suk-Won SW   Yoo Tae T   Lee Kwang-Woong KW   Suh Kyung-Suk KS  

Journal of Korean medical science 20140227 3


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  ...[more]

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