title: 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.
authors.affiliation: Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: YoungRok
authors.identifier: 0000-0003-2408-7086
authors.initials: Y
authors.lastname: Choi
authors.affiliation: Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Nam-Joon
authors.identifier: 0000-0002-5467-425X
authors.initials: NJ
authors.lastname: Yi
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Jae Sung
authors.identifier: 0000-0002-3064-2974
authors.initials: JS
authors.lastname: Ko
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Jung Min
authors.identifier: 0000-0002-0407-7828
authors.initials: JM
authors.lastname: Ko
authors.affiliation: Department of Plastic and Reconstructive Surgery, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Ung Sik
authors.identifier: 0000-0001-5096-6207
authors.initials: US
authors.lastname: Jin
authors.affiliation: Department of Anesthesiology and Pain Medicine, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Hee Soo
authors.initials: HS
authors.lastname: Kim
authors.affiliation: Department of Anesthesiology and Pain Medicine, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Kook Hyun
authors.initials: KH
authors.lastname: Lee
authors.affiliation: Department of Orthopaedic Surgery, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Tae-Joon
authors.identifier: 0000-0001-8514-377X
authors.initials: TJ
authors.lastname: Cho
authors.affiliation: Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Suk-Won
authors.identifier: 0000-0001-6111-9601
authors.initials: SW
authors.lastname: Suh
authors.affiliation: Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Tae
authors.identifier: 0000-0003-3834-7125
authors.initials: T
authors.lastname: Yoo
authors.affiliation: Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Kwang-Woong
authors.identifier: 0000-0001-6412-1926
authors.initials: KW
authors.lastname: Lee
authors.affiliation: Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Kyung-Suk
authors.initials: KS
authors.lastname: Suh
mesh_terms: D015519:Bone Density; D050071:Bone Density Conservation Agents / Q000627:therapeutic use; D002780:Cholestasis, Intrahepatic / Q000175:diagnosis*; D004164:Diphosphonates / Q000627:therapeutic use; D050723:Fractures, Bone / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D019520:Living Donors; D008297:Male; D010013:Osteogenesis Imperfecta / Q000150:complications / Q000601:surgery*; D000077268:Pamidronate; D011859:Radiography
chemical_list: D050071:Bone Density Conservation Agents; D004164:Diphosphonates; D000077268:Pamidronate
keywords: Cholestatic Liver Disease; Liver Transplantation; Malignant Hyperthermia; Osteogenesis Imperfecta