Liver transplantation in transthyretin familial amyloid polyneuropathy: first report from Argentina.
This is the first report from Argentina of liver transplantation in patients with transthyretin related familial amyloidotic polyneuropathy. The aims of the study were to analyze the clinical characteristics of this new focus and evaluate the postoperative complications and long term follow up. Five of ten patients evaluated underwent liver transplantation. During the waiting period the polyneuropathy disability score in each patient progressed one or two stages. Pretransplant modified body mass index was 723. The procedure was done with full size grafts in four cases and a split right graft in one. All patients presented postoperative complications related to disease: severe edema of the legs, recurrent choledochal lithiasis, postoperative hernia, necrotizing fasciitis and ischemic rectosigmoidal perforation. Assessment of three patients after 20 months of transplantation showed improvement in somatic and mental symptoms. No improvement was noted in cardiac denervation and gastric stasis. Liver transplantation is a rational therapeutic option for transthyretin familial amyloidotic polyneuropathy in Argentina and should be indicated in earlier stages of the symptomatic disease to reduce the postoperative morbidity and mortality. Family studies and follow up of asymptomatic carriers will define the epidemiological behavior in this country and facilitate early therapeutic intervention.
Lendoire
J
J
Department of Liver Transplantation-Hospital Dr. Cosme Argerich, Buenos Aires, Argentina. lendoire@sat.org.ar
Trigo
P
P
Aziz
H
H
Cueto
G
G
Ando
Y
Y
Ohlsson
P I
PI
Suhr
O B
OB
Imventarza
O
O
D000328:Adult; D017772:Amyloid Neuropathies / Q000401:mortality / Q000628:therapy*; D001118:Argentina; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D009154:Mutation; D011228:Prealbumin / Q000235:genetics*
D011228:Prealbumin