**Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.**
Ilias KOUNIS
Affiliations
1) AP-HP Hôpital Paul-Brousse, Centre Hépato-Biliaire, 94800, Villejuif, France
2) Inserm, Université Paris-Saclay, UMR-S 1193, 94800, Villejuif, France
3) Université Paris-Saclay, Inserm, Physiopathogénèse et traitement des maladies du Foie, 94805, Villejuif
4) France FHU Hepatinov, 94800, Villejuif, France

**Main Body of the Abstract**
Background and aims: Familial amyloid polyneuropathy (FAP) is an autosomal dominant multisystemic fatal disorder. Since more than 90% of TTR is produced within the liver, it is expected that liver transplantation (LT) will stop disease progression and that variant TTR will clear from the serum. 
Methods: This is a retrospective monocentric study including adult patients transplanted for FAP between 1997 and 2022. The research employs Cox models to identify predictive factors for the progression of neuropathy symptoms after LT and for survival.
Results: A total of 257 ATTRv-PN patients underwent LT, with a median follow-up of 12.4 [3.8, 20.1] years. 56% of donors carried Val MET 30 mutations. Symptom outcomes post-LT revealed improvement in 3.5%, stability in 48.6%, and worsening in 47.9%. At 5 and 10 years, exacerbation of neuropathic symptoms was observed in 33.3% and 51.7%, respectively, with prevalent symptoms including sensibility loss (47.5%), neuropathic pain (38.2%), walking difficulties (39.3%), diarrhea (47.2%), and pacemaker use (42%). Survival rates were 90.6% at 5 years and 77.3% at 10 years. Univariate analysis identified age at LT (p<0.001), AST and ALT at 5 years post-LT (p=0.03 and 0.01), and the use of steroids (p<0.001) and tacrolimus (p<0.001) one year after LT as significant predictors of disease progression. Cox model results highlighted a notable negative association between disease's progression post-LT (HR=11.961, p=0.001) and survival. 
Conclusion: Despite symptom progression in half of the patients post-LT, it is associated with poor survival. Early detection and alternative treatments post-LT are crucial for improved outcomes.



**Author Listing - Lead/ Submitting Author (we will be communicating with this individual): Name, Institution, City and State where the work was done. Co-authors will be added in a separate section.**
Ilias KOUNIS
## Affiliations
1) AP-HP Hôpital Paul-Brousse, Centre Hépato-Biliaire, 94800, Villejuif, France
2) Inserm, Université Paris-Saclay, UMR-S 1193, 94800, Villejuif, France
3) Université Paris-Saclay, Inserm, Physiopathogénèse et traitement des maladies du Foie, 94805, Villejuif
4) France FHU Hepatinov, 94800, Villejuif, France

**Main Body of the Abstract**
**Background and aims:** Familial amyloid polyneuropathy (FAP) is an autosomal dominant multisystemic fatal disorder. Since more than 90% of TTR is produced within the liver, it is expected that liver transplantation (LT) will stop disease progression and that variant TTR will clear from the serum. 
**Methods:** This is** **a retrospective monocentric study including adult patients transplanted for FAP between 1997 and 2022. The research employs Cox models to identify predictive factors for the progression of neuropathy symptoms after LT and for survival.
**Results:** A total of 257 ATTRv-PN patients underwent LT, with a median follow-up of 12.4 [3.8, 20.1] years. 56% of donors carried Val MET 30 mutations. Symptom outcomes post-LT revealed improvement in 3.5%, stability in 48.6%, and worsening in 47.9%. At 5 and 10 years, exacerbation of neuropathic symptoms was observed in 33.3% and 51.7%, respectively, with prevalent symptoms including sensibility loss (47.5%), neuropathic pain (38.2%), walking difficulties (39.3%), diarrhea (47.2%), and pacemaker use (42%). Survival rates were 90.6% at 5 years and 77.3% at 10 years. Univariate analysis identified age at LT (p<0.001), AST and ALT at 5 years post-LT (p=0.03 and 0.01), and the use of steroids (p<0.001) and tacrolimus (p<0.001) one year after LT as significant predictors of disease progression. Cox model results highlighted a notable negative association between disease's progression post-LT (HR=11.961, p=0.001) and survival. 
**Conclusion:** Despite symptom progression in half of the patients post-LT, it is associated with poor survival. Early detection and alternative treatments post-LT are crucial for improved outcomes.



FAP-LIFE: Familial Amyloid Polyneuropathy and Long-term Impact Following Liver Transplantation: Evaluating Factors PB149 (#145)

We invite you to attend the XIX International Symposium on Amyloidosis to be held in Rochester, MN.

This symposium is organized as a hybrid meeting (with the focus on in-person) held from Sunday, May 26 through Thursday, May 30, 2024.

Together with the ISA Board and members of Mayo Clinic, a very attractive scientific hybrid program is being planned. The program will cover a variety of types of systemic amyloidosis: ATTR (hereditary and wild-type), AL (light-chain), AA and less frequent forms. There will be key lectures, specific symposia including those company sponsored, oral presentations and poster sessions of accepted abstracts.

COURSE DESCRIPTION
This program will provide a comprehensive review of state-of-the-art education in systemic amyloidosis: basic science, pathophysiology, diagnosis, current and emerging treatments. Scientific presentations will include competitive oral and poster abstracts. The rapid evolution of scientific discovery and expanding treatment options has created tremendous interest in the field and this meeting will fill and important gap in educating scientists and clinicians.