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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Main Authors: 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
Format: Online
Language:English
Published: The Korean Academy of Medical Sciences 2014
Online Access:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3945143/
id pubmed-3945143
recordtype oai_dc
spelling pubmed-39451432014-03-10 Living Donor Liver Transplantation for an Infant with Osteogenesis Imperfecta and Intrahepatic Cholestasis: Report of a Case 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 Case Report 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. The Korean Academy of Medical Sciences 2014-03 2014-02-27 /pmc/articles/PMC3945143/ /pubmed/24616597 http://dx.doi.org/10.3346/jkms.2014.29.3.441 Text en © 2014 The Korean Academy of Medical Sciences. http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
repository_type Open Access Journal
institution_category Foreign Institution
institution US National Center for Biotechnology Information
building NCBI PubMed
collection Online Access
language English
format Online
author 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
spellingShingle 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
Living Donor Liver Transplantation for an Infant with Osteogenesis Imperfecta and Intrahepatic Cholestasis: Report of a Case
author_facet 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
author_sort Choi, YoungRok
title Living Donor Liver Transplantation for an Infant with Osteogenesis Imperfecta and Intrahepatic Cholestasis: Report of a Case
title_short Living Donor Liver Transplantation for an Infant with Osteogenesis Imperfecta and Intrahepatic Cholestasis: Report of a Case
title_full Living Donor Liver Transplantation for an Infant with Osteogenesis Imperfecta and Intrahepatic Cholestasis: Report of a Case
title_fullStr Living Donor Liver Transplantation for an Infant with Osteogenesis Imperfecta and Intrahepatic Cholestasis: Report of a Case
title_full_unstemmed Living Donor Liver Transplantation for an Infant with Osteogenesis Imperfecta and Intrahepatic Cholestasis: Report of a Case
title_sort living donor liver transplantation for an infant with osteogenesis imperfecta and intrahepatic cholestasis: report of a case
description 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.
publisher The Korean Academy of Medical Sciences
publishDate 2014
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3945143/
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