Treatment of a Japanese Patient with Familial Amyloidotic Polyneuropathy with Orthotopic Liver Transplantation

• Published in: Internal Medicine Vol. 33; no. 11; pp. 730 - 732
• Main Authors: ANDO, Yukio, TASHIMA, Kazuhiro, TANAKA, Yoshiya, NAKAZATO, Masamitsu, ERICZON, Bo-Göran, DURAJ, Frans F., SAKASHITA, Naomi, KIMURA, En, ANDO, Eiko, YONEHARA, Toshiro, YAMASHITA, Taro, NAKAMURA, Masaaki, UCHINO, Makoto, ANDO, Masayuki
• Format: Journal Article
• Language: English
• Published: Tokyo The Japanese Society of Internal Medicine 1994; Japanese Society of Internal Medicine
• Subjects: Adult; Amyloid Neuropathies - blood; Amyloid Neuropathies - surgery; Amyloidosis; Biological and medical sciences; Cadaver; Errors of metabolism; heart-beating cadaveric donor; Humans; Japan; Japanese patient; Liver Transplantation; Male; Medical sciences; Metabolic diseases; Prealbumin - analysis; Proteins and glycoproteins; Sweden; Tissue Donors; Transthyretin; Sweden; Japan; Human; Nervous system diseases; Liver; Metabolic diseases; Transplantation; Homotransplantation; Polyneuropathy; Case study; Metabolic disorder; Treatment; Surgery; Amyloidosis; Peripheral nerve disease
• ISSN: 0918-2918; 1349-7235
• DOI: 10.2169/internalmedicine.33.730

A 28-year-old male patient with familial amyloidotic polyneuropathy (FAP) underwent a liver transplantation from a heart-beating cadaveric donor in Sweden. He had suffered from the disease for 2.5 years. It took 5.5 hours to carry out the operation without blood transfusion. After the liver transplantation, serum amyloidgenic variant transthyretin (TTR) levels became extremely low and diarrhea stopped after the 7th day. On day 13, the patient was discharged from the hospital and one month after the transplantation, his general condition remained quite good. This is the first case of a Japanese patient with congenital metabolic disorders as well as FAP to receive a liver transplantation from a heart-beating cadaveric donor. (Internal Medicine 33: 730-732, 1994)