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Fig. 5 | Orphanet Journal of Rare Diseases

Fig. 5

From: Diagnosis and management of transthyretin familial amyloid polyneuropathy in Japan: red-flag symptom clusters and treatment algorithm

Fig. 5

Treatment algorithm specific to ATTR-FAP Val30Met in Japan. aMost late-onset ATTR-FAP patients show progression of the disease even after LT. However, our experience in Japan and evidence in literature suggest a good outcome after LT in some of the late-onset patients; especially, late-onset females showed significantly improved survival after LT than their male counterparts (p = 0.02, Okamoto S, et al. 2009 [108]; hazard ratio 1.57 [male vs female, p = 0.014], Ericzon BG, et al. 2015 [33]). bEven in the late-onset cases, LT sometimes show good outcome in females (Ericzon BG, et al. 2015 [33]). The outcome of LT is comparably good in neuropathic type of non-Val30Met ATTR-FAP patients from our experience in Kumamoto University Hospital and Shinshu University Hospital in Japan (unpublished observations). LT liver transplantation, ATTR-FAP transthyretin familial amyloid neuropathy, Val30Met replacement of valine with methionine at position 30 in the TTR gene

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