Abstract

BackgroundIt has been demonstrated in many studies that quality of life can be improved after liver transplantation in patients with liver disease. Nevertherless quality of life improvement in specific groups of transplantated patients such as those with Familial Amyloid Polineuropathy hasn't yet been explored. The present study aimed to compare the change in quality of life following liver transplantation between patients with Familial Amyloid Polineuropathy (FAP) and patients with liver disease.ResultsPatient's mental quality of life showed an improvement in all liver disease patients, and a worsening in FAP patients, resulting in a significant difference between the two groups. Regarding physical quality of life, although a similar improvement was seen in both groups, FAP patients had significantly less improvement than the sub-group of decompensated liver disease (Child-Pugh B and C).ConclusionIt is concluded that liver transplantation has a less beneficial impact in FAP patient's physical quality of life, probably because they are not so much disabled by their disease at the moment of liver transplantation. The lesser improvement in mental quality of life of FAP patients may be due to their particular psychological profile and greater expectations towards transplantation.

Highlights

  • It has been demonstrated in many studies that quality of life can be improved after liver transplantation in patients with liver disease and that this improvement is present 6 months after transplantation [1,2,3,4]

  • The amyloid protein in type 1 Familial Amyloid Polineuropathy (FAP) of Portuguese, Swedish and Japanese origin is the variant of transthyretin 8TTR, in which methionine is a substitute for valine at position 30 (TTR Met)

  • The fact that liver transplantation had a less beneficial impact in mental quality of FAP patients is difficult to explain. It cannot be explained by the paucity of symptoms that FAP patients have before transplantation, since

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Summary

Introduction

It has been demonstrated in many studies that quality of life can be improved after liver transplantation in patients with liver disease. Nevertherless quality of life improvement in specific groups of transplantated patients such as those with Familial Amyloid Polineuropathy hasn't yet been explored. The present study aimed to compare the change in quality of life following liver transplantation between patients with Familial Amyloid Polineuropathy (FAP) and patients with liver disease. The amyloid protein in type 1 FAP of Portuguese, Swedish and Japanese origin is the variant of transthyretin 8TTR, in which methionine is a substitute for valine at position 30 (TTR Met). More than 90% of this TTR Met 30 is produced by the liver and the rest by choroid plexus.

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Conclusion

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