Ten Years of Experience with Liver Transplantation for Familial Amyloid Polyneuropathy in Japan: Outcomes of Living Donor Liver Transplantations

Takei, Yo‐ichi; Ikeda, Shu‐ichi; Ikegami, Toshihiko; Hashikura, Yasuhiko; Miyagawa, Shinichi; Ando, Yumiko

doi:10.2169/internalmedicine.44.1151

Cited by 44 publications

(23 citation statements)

References 37 publications

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“…Although marked prolongation of survival can be now expected in patients with successful operations [19,20], a significant difference in survival was reported between patients FAP receiving transplants at early and advanced stages of the disease [6]. Thus, early operation is recommended to improve patients' post-transplant quality of life [21].…”

Section: Discussionmentioning

confidence: 99%

Ten-year follow-up of peripheral nerve function in patients with familial amyloid polyneuropathy after liver transplantation

et al. 2008

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Section: Discussionmentioning

confidence: 99%

Ten-year follow-up of peripheral nerve function in patients with familial amyloid polyneuropathy after liver transplantation

et al. 2008

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“…In addition to the Japanese culture and nationality, an insufficient psychological support system after predictive testing is considered to be an important reason for the low ratio of predictive test usage in Japan. In contrast to HD and SCD, most (74.3%) clients interested in FAP actually underwent predictive testing in Japan, suggesting that the availability of disease-modifying therapy promoted the usage of testing [13][14][15] .…”

Section: Discussionmentioning

confidence: 99%

Follow-up nationwide survey on predictive genetic testing for late-onset hereditary neurological diseases in Japan

et al. 2013

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A follow-up nationwide survey on predictive genetic testing for late-onset neurological diseases in Japan was conducted. A questionnaire was sent to 89 institutional members of the Japan's National Liaison Council for Clinical Sections of Medical Genetics, and was returned by 60 (67.4%). A total of 301 clients with an interest in predictive testing were accumulated from April 2006 to March 2011. The greatest interest was shown for spinocerebellar degeneration (SCD, n=110), followed by myotonic dystrophy type 1 (DM1, n=69), Huntington's disease (HD, n=52), and familial amyloid polyneuropathy (FAP, n=35). The ratios of clients who actually underwent predictive testing were: SCD, 21.8%; DM1, 39.1%; HD, 26.9%; and FAP, 74.3%, indicating that predictive testing was conducted very cautiously for untreatable neurological diseases in Japan. Clinical geneticists were predominantly involved in genetic counseling, whereas the participation of non-medical doctor (non-MD) staff, including nurses, clinical psychologists, and genetic counselors, was not common.Lack of non-MD counseling staff was one of the most serious issues in conducting predictive testing, which has not been improved since the previous survey performed in 2006. Institutional arrangements, such as revision of medical insurance system regarding genetic testing and counseling, might be necessary to resolve this issue.

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“…Hepatic artery thrombosis was a frequent complication in living donor liver transplantation for FAP patients compared with other severe hepatic diseases at our hospital: we have performed liver transplantation in 25 FAP patients over the last 11 years [24], and five patients have experienced hepatic artery thrombosis. On the other hand, seven out of 197 recipients have also been reported with this complication [25].…”

Section: Discussionmentioning

confidence: 99%

Value of renal biopsy in the prognosis of liver transplantation in familial amyloid polyneuropathy ATTR Val30Met patients

Oguchi

Takei

Ikeda

2006

Amyloid

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Liver transplantation for familial amyloid polyneuropathy (FAP) patients has been carried out worldwide and the outcomes seem to be promising. To clarify the severity of amyloid deposits on visceral organs, we evaluated the histopathological findings of biopsied renal and sural nerve specimens in 13 FAP patients with ATTR Val30Met by quantitative analysis, and compared them with the outcome of transplantation. Renal dysfunction with proteinuria seemed to correlate with the degree of amyloid deposits in glomeruli, not with that in medullary tissues. The severity of renal amyloid deposition did not consistently parallel that of myelinated nerve fiber loss in sural nerve. Three patients with proteinuria and severe amyloid deposits in glomeruli were considered to be unsuitable for transplantation. Ten patients underwent living donor liver transplantation and three resulted in unfavorable outcomes. These three had heavy amyloid deposits on renal tissues, especially in glomerular areas, but the severity of myelinated nerve fiber loss in their sural nerves was very similar to that in patients who made a good recovery. The prognosis after operation might be closely related to the severity of amyloid deposits in renal glomeruli. Renal biopsy is, therefore, recommended when determining the indications and contraindications for liver transplantation in FAP patients, although this biopsy is not routinely required.

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Ten Years of Experience with Liver Transplantation for Familial Amyloid Polyneuropathy in Japan: Outcomes of Living Donor Liver Transplantations

Cited by 44 publications

References 37 publications

Ten-year follow-up of peripheral nerve function in patients with familial amyloid polyneuropathy after liver transplantation

Ten-year follow-up of peripheral nerve function in patients with familial amyloid polyneuropathy after liver transplantation

Follow-up nationwide survey on predictive genetic testing for late-onset hereditary neurological diseases in Japan

Value of renal biopsy in the prognosis of liver transplantation in familial amyloid polyneuropathy ATTR Val30Met patients

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