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Clinical improvement after liver transplantation for type I familial amyloid polyneuropathy

1995; Oxford University Press; Volume: 82; Issue: 6 Linguagem: Inglês

10.1002/bjs.1800820634

1365-2168

P Parrilla, P. Ramı́rez, F.S Bueno, R Robles, F Acosta, Manuel Miras, J.A. Pons, Franc Andreu, Miguel Munar-Qués,

Trace Elements in Health

Abstract Long-term results of 13 liver transplantations in patients with a previous diagnosis of type I familial amyloid polyneuropathy (FAP) are presented. The diagnosis of type I FAP was based on the presence of a biochemical marker in the plasma (TTR-Met-30 in 11 patients, TTR-Ala-71 in two). Maximum follow-up is 28 months and the survival rate stands at 11 of 13 patients. Two patients died from sepsis at 2 and 6 months. TTR disappeared from plasma in all cases. Neurological status improved in all eight patients undergoing transplantation more than 6 months previously, although electromyographic studies showed a slight improvement only in the six with follow-up of more than 1 year. All 13 patients showed a hyperdynamic haemodynamic pattern with a high incidence (four patients) of the use of venovenous bypass due to haemodynamic intolerance. Two patients also received transplants by the ‘piggy-back’ technique. In conclusion, liver transplantation may be useful in the treatment of certain patients with FAP to halt and improve the neurological consequences of the disease.