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J Korean Med Sci. 2014 Mar;29(3):441-4. doi: 10.3346/jkms.2014.29.3.441. Epub 2014 Feb 27.

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

Author information

  • 1Department of Surgery, Seoul National University College of Medicine, Seoul, Korea.
  • 2Department of Pediatrics and Adolescent Medicine, Seoul National University College of Medicine, Seoul, Korea.
  • 3Department of Plastic and Reconstructive Surgery, Seoul National University College of Medicine, Seoul, Korea.
  • 4Department of Anesthesiology and Pain Medicine, Seoul National University College of Medicine, Seoul, Korea.
  • 5Department of Orthopaedic Surgery, Seoul National University College of Medicine, Seoul, Korea.

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.

KEYWORDS:

Cholestatic Liver Disease; Liver Transplantation; Malignant Hyperthermia; Osteogenesis Imperfecta

PMID:
24616597
[PubMed - indexed for MEDLINE]
PMCID:
PMC3945143
Free PMC Article
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