title: Liver mitochondrial function in familial amyloidotic polyneuropathy.
abstract: The objective of the present study was to analyze hepatic mitochondrial function in patients with familial amyloidotic polyneuropathy (FAP) undergoing cadaveric donor orthotopic liver transplantation. From February 2005 to May 2007, eight patients with FAP, ranging in age from 34 to 41 years and with Model for End-Stage Liver Disease scores ranging from 24 to 29. Underwent orthotopic transplantation using a liver from a deceased donor by the piggyback method. Immediately before beginning the recipient hepatectomy in a patient with FAP, a biopsy was obtained for analysis of mitochondrial function (FAP group). The control group consisted of 15 patients undergoing hepatic surgery to treat small tumors of the liver. Mitochondrial respiration was determined on the basis of oxygen consumption by energized mitochondria using a polarographic method. The membrane potential of the mitochondria was determined spectrofluorometrically. Data were analyzed statistically by the Mann-Whitney test, with the level of significance set at 5%. State 3 and 4 values, respiratory control ratio, and membrane potential were 47 +/- 8 versus 28 +/- 10 natoms O/min/mg protein (P < .05); 14 +/- 3 vs 17 +/- 7 nat.O/min/mg.prot.mit. (P > .05); 3.6 +/- .5 vs 1.7 +/- 0.7 (P < .05); and 135 +/- 5.2 vs 135 +/- 6 mV (P > .05) for control versus FAP patients, respectively, demonstrating a decreased energy status of the liver in FAP.
authors.affiliation: Special Liver Transplantation Unit, Departments of Surgery and Anatomy, Faculty of Medicine of Ribeirào Preto, University of São Paulo, São Paulo, Brazil. orlando@fmrp.usp.br
authors.forename: O
authors.initials: O
authors.lastname: Castro e Silva
authors.forename: A K
authors.initials: AK
authors.lastname: Sankarankutty
authors.forename: M E J
authors.initials: ME
authors.lastname: Souza
authors.forename: M A N C
authors.initials: MA
authors.lastname: Picinato
authors.forename: C F
authors.initials: CF
authors.lastname: Fina
authors.forename: M C
authors.initials: MC
authors.lastname: Jordani
authors.forename: E D
authors.initials: ED
authors.lastname: Mente
authors.forename: D
authors.initials: D
authors.lastname: Cagnolatti
authors.forename: A C
authors.initials: AC
authors.lastname: Teixeira
authors.forename: F F
authors.initials: FF
authors.lastname: Souza
authors.forename: A L C
authors.initials: AL
authors.lastname: Martinelli
authors.forename: L Z
authors.initials: LZ
authors.lastname: Rondon
mesh_terms: D000328:Adult; D028227:Amyloid Neuropathies, Familial / Q000378:metabolism* / Q000601:surgery*; D005260:Female; D006498:Hepatectomy; D006801:Humans; D016031:Liver Transplantation; D008297:Male; D008564:Membrane Potentials; D008930:Mitochondria, Liver / Q000378:metabolism*; D010101:Oxygen Consumption