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Titolo:
Scavenger treatment of free radical injury in familial amyloidotic polyneuropathy: a study on Swedish transplanted and non-transplanted patients
Autore:
Suhr, OB; Lang, K; Wikstrom, L; Anan, I; Ando, Y; El-Salhy, M; Holmgren, G; Tashima, K;
Indirizzi:
Umea Univ Hosp, Dept Med, S-90185 Umea, Sweden Umea Univ Hosp Umea Sweden S-90185 Hosp, Dept Med, S-90185 Umea, Sweden Pitea Hosp, Dept Med, Pitea, Sweden Pitea Hosp Pitea SwedenPitea Hosp, Dept Med, Pitea, Sweden Skelleftea Hosp, Dept Med, Skelleftea, Sweden Skelleftea Hosp SkellefteaSweden ea Hosp, Dept Med, Skelleftea, Sweden Umea Univ Hosp, Dept Clin Genet, S-90185 Umea, Sweden Umea Univ Hosp Umea Sweden S-90185 Dept Clin Genet, S-90185 Umea, Sweden
Titolo Testata:
SCANDINAVIAN JOURNAL OF CLINICAL & LABORATORY INVESTIGATION
fascicolo: 1, volume: 61, anno: 2001,
pagine: 11 - 18
SICI:
0036-5513(200102)61:1<11:STOFRI>2.0.ZU;2-O
Fonte:
ISI
Lingua:
ENG
Soggetto:
HEREDITARY TRANSTHYRETIN AMYLOIDOSIS; INDUCED OXIDATIVE STRESS; LIVER-TRANSPLANTATION; ALZHEIMERS-DISEASE; VITAMIN-E; CYCLOSPORINE NEPHROTOXICITY; SYNAPTOSOMAL MEMBRANES; THIOL CONJUGATION; FOLLOW-UP; NEUROTOXICITY;
Keywords:
drug therapy scavengers; immunohistochemistry; inborn errors of metabolism; liver transplantation; neuropathic amyloidosis; oxidative stress; transthyretin;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
35
Recensione:
Indirizzi per estratti:
Indirizzo: Suhr, OB Umea Univ Hosp, Dept Med, S-90185 Umea, Sweden Umea Univ Hosp Umea Sweden S-90185 pt Med, S-90185 Umea, Sweden
Citazione:
O.B. Suhr et al., "Scavenger treatment of free radical injury in familial amyloidotic polyneuropathy: a study on Swedish transplanted and non-transplanted patients", SC J CL INV, 61(1), 2001, pp. 11-18

Abstract

Since oxidative stress has been implicated in amyloid diseases, a study ofscavenger treatment of hereditary transthyretin amyloidosis was undertakenon 23 familial amyloidotic polyneuropathy (FAP) patients. Nine patients had undergone a liver transplantation for the disease. Twenty patients completed the 6-month study period of scavenger treatment (vitamin C, 1 g, three times daily, vitamin E, 0.1 g, three times daily and acetylcysteine, 0.2 g three times daily). They were evaluated clinically and by immunohistochemical measurement of hydroxynonenal (HNE), a product of lipid peroxidation, inbiopsy specimens. For non-transplanted patients, no improvement was found for HNE in relation to the amyloid content in biopsy specimens, whereas a tendency to a decreased amount was noted for transplanted patients. Clinically, no differences were found for non-transplanted patients, but an increased nutritional status, measured by a modified body mass index (mBMI) was noted for transplanted patients. In summary, scavenger treatment with the drugs and doses used in the present study appears to be unable to decrease lipid peroxidation in amyloid-rich tissue in non-transplanted FAP patients. For transplanted patients, lipid peroxidation tended to decrease, and the nutritional status measured by mBMI improved, even though the findings may be explained by liver transplantation alone, scavenger treatment may facilitate recovery after transplantation.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 29/03/20 alle ore 12:19:39