Clinical improvement after liver transplantation for type I familial amyloid polyneuropathy.
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.
Parrilla
P
P
Department of Surgery, Virgen Arrixaca Hospital, Murcia, Spain.
Ramirez
P
P
Bueno
F S
FS
Robles
R
R
Acosta
F
F
Miras
M
M
Pons
J A
JA
Andreu
F L
FL
Munar-Ques
M
M
D000328:Adult; D000682:Amyloid / Q000032:analysis*; D017772:Amyloid Neuropathies / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D006439:Hemodynamics; D006801:Humans; D008099:Liver / Q000473:pathology; D016031:Liver Transplantation; D008875:Middle Aged; D011228:Prealbumin / Q000032:analysis*; D011379:Prognosis; D013497:Sural Nerve / Q000473:pathology; D016896:Treatment Outcome
D000682:Amyloid; D011228:Prealbumin; C041120:amyloid prealbumin