Abstract Details

Title Domino Liver Transplantation (DLT) and De Novo Familial Amyloid Polyneuropathy (FAP): The Portuguese Experience

Topic Peripheral Nerve

Presentation(s) S58 - (-)

Poster/Presentation
Number 004

Objective

Background Domino liver transplantation (DLT) started in 1995, using grafts from patients with familial amyloid polyneuropathy (FAP) as a strategy to increase the number of grafts available to treat patients with liver diseases. Assuming the natural course of FAP, early estimation of disease transfer risk was expected to have a minimum delay of 20 years. After 2005 several papers described clinical cases of de novo FAP developing 5 to 9 years after DLT. Pathologic and neurophysiologic signs of disease in asymptomatic patients were also reported. Portugal has the largest population of domino recipients in the world. In order to better understand the extent and the timing of de novo FAP development we evaluated all receptors transplanted between 1999 and 2005.

Design/Methods 48 patients submitted to domino liver transplant between 1999 and 2005 are presently alive. 47 of them (11 females and 36 males) were observed and had clinical, pathologic (salivary gland biopsy) and electrophysiologic evaluation. One patient is bedridden and not observed.

Results Twenty three (23) patients were clinically symptomatic and 24 were asymptomatic. The symptomatic group started with sensory complaints involving their feet 3 to 10 years after DLT. Twenty one (21) out of 22 salivary gland biopsies showed amyloid deposition. Neurophysiologic evaluation was abnormal in 16 out of 20. In the asymptomatic group 20 out of 22 salivary gland biopsies showed amyloid deposition. Neurophysiologic evaluation was abnormal in 10 out of 18. Three patients were retransplanted to halt FAP progression. Two other patients are in the waiting list for retransplant.

Conclusions Our results, as described by other centres, confirm that de novo amyloidosis in Portuguese domino recipients manifests earlier than expected when DLT was started and occurs in a large percentage of patients.

Authors/Disclosures
PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Aaron Ellenbogen, DO	Dr. Ellenbogen has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for allergan. Dr. Ellenbogen has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for allergan. Dr. Ellenbogen has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Teva. Dr. Ellenbogen has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Supernus. Dr. Ellenbogen has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for ipsen. Dr. Ellenbogen has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Acorda.
	No disclosure on file
	No disclosure on file
	No disclosure on file
Martin E. Sanders, MD	No disclosure on file
Teresa Coelho, MD (Unidate Clinica de Paramoloidose Hospital)	Dr. Coelho has nothing to disclose.

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