Living Donor Liver Transplantation for an Infant with Osteogenesis Imperfecta and Intrahepatic Cholestasis: Report of a Case.
10.3346/jkms.2014.29.3.441
- Author:
Youngrok CHOI
1
;
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
;
Kyung Suk SUH
Author Information
1. Department of Surgery, Seoul National University College of Medicine, Seoul, Korea. gsleenj@hanmail.net
- Publication Type:Case Reports
- Keywords:
Osteogenesis Imperfecta;
Liver Transplantation;
Cholestatic Liver Disease;
Malignant Hyperthermia
- MeSH:
Bone Density;
Bone Density Conservation Agents/therapeutic use;
Cholestasis, Intrahepatic/*diagnosis;
Diphosphonates/therapeutic use;
Fractures, Bone/drug therapy/etiology/radiography;
Humans;
Infant;
*Liver Transplantation;
Living Donors;
Male;
Osteogenesis Imperfecta/complications/*surgery
- From:Journal of Korean Medical Science
2014;29(3):441-444
- CountryRepublic of Korea
- Language:English
-
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.
