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Titolo:
Gastrointestinal dysfunction in familial amyloidotic polyneuropathy (ATTR Val30Met) - comparison of Swedish and Japanese patients
Autore:
Tashima, K; Suhr, OB; Ando, Y; Holmgren, G; Yamashita, T; Obayashi, K; Terazaki, H; Uchino, M;
Indirizzi:
Umea Univ Hosp, Dept Internal Med, S-90185 Umea, Sweden Umea Univ Hosp Umea Sweden S-90185 pt Internal Med, S-90185 Umea, Sweden Kumamoto Univ, Sch Med, Dept Neurol, Kumamoto 8608556, Japan Kumamoto Univ Kumamoto Japan 8608556 ept Neurol, Kumamoto 8608556, Japan Kumamoto Univ, Sch Med, Dept Internal Med 1, Kumamoto 8608556, Japan Kumamoto Univ Kumamoto Japan 8608556 rnal Med 1, Kumamoto 8608556, Japan
Titolo Testata:
AMYLOID-INTERNATIONAL JOURNAL OF EXPERIMENTAL AND CLINICAL INVESTIGATION
fascicolo: 2, volume: 6, anno: 1999,
pagine: 124 - 129
SICI:
1350-6129(199906)6:2<124:GDIFAP>2.0.ZU;2-S
Fonte:
ISI
Lingua:
ENG
Soggetto:
GEOGRAPHICAL-DISTRIBUTION; LIVER-TRANSPLANTATION; FOLLOW-UP; TRANSTHYRETIN; SWEDEN; ONSET; HOMOZYGOSITY; PREVALENCE; SYMPTOMS; SURVIVAL;
Keywords:
amyloidosis; familial; neuropathy; inborn errors of metabolism; mutation; transthyretin; gastrointestinal;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
31
Recensione:
Indirizzi per estratti:
Indirizzo: Tashima, K Umea Univ Hosp, Dept Internal Med, S-90185 Umea, Sweden Umea Univ Hosp Umea Sweden S-90185 Med, S-90185 Umea, Sweden
Citazione:
K. Tashima et al., "Gastrointestinal dysfunction in familial amyloidotic polyneuropathy (ATTR Val30Met) - comparison of Swedish and Japanese patients", AMYLOID, 6(2), 1999, pp. 124-129

Abstract

The aim of the present study was to compare the clinical symptoms of Swedish and Japanese patients with familial amyloidotic polyneuropathy (ATTR Val30Met), especially gastrointestinal disturbances, and to correlate the findings with survival. Seventy-three Swedish and 47 Japanese patients were available for the study. Thirty-two Swedish and 7 Japanese patients had undergone liver transplantation. The mean age at onset was 50 for Swedish and 35 for Japanese patients (P<0.001; non-transplanted patients). Fifty-five percent of Japanese patients had gastrointestinal disturbances from onset, compared to 22% of Swedish patients (P<0.05; whole population). The Swedish patients average survival after the onset was significantly longer than Japanese patients (P<0.05; non-transplanted patients). An early onset of gastrointestinal symptoms was correlated to a decreased survival for Swedish, hut not for Japanese patients. Age at onset was nor correlated to survival neither for Swedish nor for Japanese patients. We conclude that the clinical expressions of familial amyloidotic polyneuropathy ATTR Val30Met are different in Swellish and Japanese patients. The survival after the onset was shorter for Japanese patients, who also had an earlier onset of gastrointestinal disturbances, especially diarrhea than Swedish patients.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 10/07/20 alle ore 03:08:09