Living donor liver transplantation for homozygous familial hypercholesterolemia from a donor with heterozygous hypercholesterolemia

Yasuhiro Shirahata, Naoki Kawagishi, Satoru Sekiguchi, Nobuhiro Ohkohchi, Susumu Satomi

Research output: Contribution to journalArticlepeer-review

Abstract

Homozygous familial hypercholesterolemia is a rare inherited disease occurring approximately 1 per 1 million people. Severe hyper-cholesterolemia is observed from birth, associated with severe pediatric athero-sclerosis, leading to death due to myocardial infarction before the age of 20 years. Liver transplantation is the only effective treatment. We report a 2-year 5-month-old infant with homozygous familial hyper-cholesterolemia who underwent a liver graft from his father, who had the same disease of heterotype. Preoperative plasma cholesterol was > 800 mg/dl. After liver transplantation, the recipient's cholesterol level decreased to 250-300 mg/dl using the HMG-CoA reductase inhibitor. At 43 months after transplantation, the patient is doing well and eating freely. The combination of liver transplantation from a donor with heterozygous familial hypercholesterolemia and cholesterol-lowering drugs is effective in treating such patients.

Original languageEnglish
Pages (from-to)57-62
Number of pages6
JournalJapanese Journal of Gastroenterological Surgery
Volume38
Issue number1
DOIs
Publication statusPublished - 2005

Keywords

  • Donor with heterozygous familial hypercholesterolemia
  • Homozygous familial hypercholesterolemia
  • Living donor liver transplantation

ASJC Scopus subject areas

  • Surgery
  • Gastroenterology

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