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

Yasuhiro Shirahata, Nobuhiro Ohkohchi, Naoki Kawagishi, Masaru Syouji, Sigeki Tsukamoto, Satoru Sekiguchi, Nozomisa Koyamada, Sinichi Oikawa, Susumu Satomi

研究成果: Article査読

32 被引用数 (Scopus)

抄録

Familial hypercholesterolemia is a rare inherited disease with an incidence of approximately one per million. Severe hypercholesterolemia is observed from the time of birth onwards. It is associated with severe atherosclerosis in childhood, leading to death from myocardial infarction before the age of 20 years. Liver transplantation is the only effective treatment for this disease. We experienced the case of an infant aged 2 years 5 months who had homozygous familial hypercholesterolemia and who received a liver graft from his father, who had familial heterozygous hypercholesterolemia. The pre-operative plasma cholesterol level was > 800 mg/dl. After liver transplantation, the recipient's cholesterol level decreased to 250 mg/dl after we administered the HMG-CoA reductase inhibitor. At present, 6 months after transplantation, the patient is doing well and free from a special diet. We can thus conclude that the combination therapy of liver transplantation from a donor with heterozygous familial hypercholesterolemia on cholesterol-lowering drugs is an effective therapy for a patient with the homozygous type of hypercholesterolemia.

本文言語English
ページ(範囲)276-279
ページ数4
ジャーナルTransplant International
16
4
DOI
出版ステータスPublished - 2003 4月 1
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ASJC Scopus subject areas

  • 移植

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