Partial-Liver Transplantation To Treat Familial Amyloid Polyneuropathy: Follow-up of 11 Patients

Takei, Yo‐ichi; Ikeda, Shu‐ichi; Hashikura, Yasuhiko; Ikegami, Toshihiko; Kawasaki, Seiji

doi:10.7326/0003-4819-131-8-199910190-00008

Cited by 53 publications

(41 citation statements)

References 15 publications

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“…Since the first success in Sweden in 1990 (57) more than 700 patients had undergone liver transplantation by the end of 2003 (58). In Japan, partial liver transplantation from living donors has been mainly carried out (59,60 (25).…”

Section: Hereditary Amyloidosismentioning

confidence: 99%

Cardiac Amyloidosis: Heterogenous Pathogenic Backgrounds

Ikeda

2004

Intern. Med.

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Cardiac amyloidosis is a fatal disorder which develops on the basis of the different pathologic conditions in systemic amyloidosis: the most common underlying disease is immunoglobulin light chain-derived primary amyloidosis and the next is transthyretin-related hereditary amyloidosis; the latter disorder, typically represented by familial amyloid polyneuropathy, was long regarded as an endemic disease. However, this disorder has now been shown to involve a highly variable clinical picture due to a large number of transthyretin gene mutations, and many patients with diverse ancestors suffer from severe cardiac amyloidosis. Additionally, senile systemic amyloidosis is now noted as a cause of cardiac dysfunction in elderly individuals. Echocardiogram and myocardial technetium-99m-pyrophosphate scintigraphy can provide characteristic findings. Immunohistochemistry on tissue amyloid, biochemical analysis of serum and urine proteins, and DNA sequencing are usually employed to determine the disease-related amyloid fibril protein.Although systemic amyloidosis has become treatable, the prognosis of each patient who received up-to-date and effective, but nevertheless stressful, therapy depends on the severity of cardiac involvement by amyloid deposition.

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Section: Hereditary Amyloidosismentioning

confidence: 99%

Cardiac Amyloidosis: Heterogenous Pathogenic Backgrounds

Ikeda

2004

Intern. Med.

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“…56 Similarly, improvement was also noted in Japanese patients who underwent transplantation within the first 4 years of the disease. 107 Recently, long-term follow-up of Portuguese patients has shown improvement of EMG results in 30% of the patients, 63 but improvement appears to be confined to those who underwent transplantation at an early stage of disease. 108 In a Japanese series of 8 patients, improved neurological score was noted within the initial 6 months after transplantation, but not on subsequent follow-up.…”

Section: Transplantation For Amyloidosismentioning

confidence: 99%

Liver transplantation for hereditary transthyretin amyloidosis

et al. 2000

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“…FAPWTR emphasized that the severity of autonomic symptoms significantly affected the patient's recovery after operation, because many usually appeared in the patients at later stages of FAP (15). However, gastrointestinal autonomic symptoms, including severe episodic nausea, vomiting, and alternating constipation and diarrhea, are often initial symptoms in Japanese patients with this disease (1), and these bowel dysfunctions improved shortly after transplantation in the FAP patients in our series (11,18). Therefore, FAP patients with only autonomic disturbances are considered good candidates for liver transplantation.…”

Section: Discussionmentioning

confidence: 95%

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

et al. 2005

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Object We summarize 10 years of experience with liver transplantation for FAP patients in Japan and review the current opinions regarding this treatment for FAP.Methods and Patients All basic report data on patients at the time of transplantation were registered with the Japanese Liver Transplantation Society (JLTS). Based on the JLST report data, more detailed information on FAP patients was requested from each center.Results Living

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Partial-Liver Transplantation To Treat Familial Amyloid Polyneuropathy: Follow-up of 11 Patients

Abstract: Preoperative clinical severity and duration of illness are associated with outcomes after liver transplantation for FAP.

Cited by 53 publications

References 15 publications

Cardiac Amyloidosis: Heterogenous Pathogenic Backgrounds

Cardiac Amyloidosis: Heterogenous Pathogenic Backgrounds

Liver transplantation for hereditary transthyretin amyloidosis

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

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