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Titolo:
ENDOSCOPIC AND PATHOLOGICAL MANIFESTATIONS OF THE GASTROINTESTINAL-TRACT IN FAMILIAL AMYLOIDOTIC POLYNEUROPATHY TYPE-I (MET30)
Autore:
YOSHIMATSU SI; ANDO Y; TERAZAKI H; SAKASHITA N; TADA S; YAMASHITA T; SUGA M; UCHINO M; ANDO M;
Indirizzi:
UMEA UNIV HOSP,DEPT MED S-90185 UMEA SWEDEN KUMAMOTO UNIV,SCH MED,DEPT INTERNAL MED 1 KUMAMOTO 860 JAPAN KUMAMOTO UNIV,SCH MED,DEPT PATHOL 2 KUMAMOTO 860 JAPAN KUMAMOTO UNIV,SCH MED,DEPT NEUROL KUMAMOTO 860 JAPAN SAISEIKAI CENT HOSP,DEPT GASTROENTEROL KUMAMOTO JAPAN
Titolo Testata:
Journal of internal medicine
fascicolo: 1, volume: 243, anno: 1998,
pagine: 65 - 72
SICI:
0954-6820(1998)243:1<65:EAPMOT>2.0.ZU;2-3
Fonte:
ISI
Lingua:
ENG
Soggetto:
SMALL-INTESTINE; SYSTEMIC AMYLOIDOSIS; HAPLOTYPE ANALYSIS; CHEMICAL TYPES; JAPANESE TYPE; TRANSTHYRETIN; VARIANT; FEATURES; IDENTIFICATION; DEPOSITION;
Keywords:
FAP; AL AMYLOIDOSIS; SECONDARY AMYLOIDOSIS; AMYLOID; TRANSTHYRETIN; GASTROINTESTINAL TRACT;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
35
Recensione:
Indirizzi per estratti:
Citazione:
S.I. Yoshimatsu et al., "ENDOSCOPIC AND PATHOLOGICAL MANIFESTATIONS OF THE GASTROINTESTINAL-TRACT IN FAMILIAL AMYLOIDOTIC POLYNEUROPATHY TYPE-I (MET30)", Journal of internal medicine, 243(1), 1998, pp. 65-72

Abstract

Objectives. To evaluate the characteristic changes in the gastrointestinal tract in familial amyloidotic polyneuropathy (FAP) (Met30), bothfibre gastroscopy and colonoscopy studies were performed in FAP (Met30) patients. Microscopic changes were also examined in autopsied and biopsied materials from patients with FAP, and compared with data from autopsied samples from patients with AL amyloidosis, and secondary amyloidosis patients. Design. Endoscopic and histopathological study. Setting. Kumamoto University Hospital, Kumamoto, Japan. Subjects. Nine patients with FAP (Met30) underwent fibre gastroscopy and colonoscopy. Six autopsied and 23 biopsied gastrointestinal samples from FAP patients, four from autopsied amyloidosis (including two myeloma associated form), and two from autopsied secondary amyloidosis patients were examined for histopathological study. Main outcome measures. Fibre gastroscopy and colonoscopy were employed for macroscopic study. Congo red andH-E staining were performed for histopathological study. Macroscopic changes in the gastrointestinal tract and microscopic differences in the amyloid distribution pattern were compared between the different types of amyloidosis. Results. Fibre gastroscopy and colonoscopy for nine FAP patients revealed that four showed a fine granular appearance inthe duodenum, three showed lack of lustre, and two showed mucosal friability in the gastrointestinal tract; however, no macroscopic abnormality was observed in four other FAP patients. Histopathological examination of tissue from FAP patients revealed that, although a small amount of amyloid was recognized in the submucosa perivascular layer, a significant amount of amyloid was seen in and around the nerves of the gastrointestinal tract, but very little in Auerbach's nerve plexus. In total, the amount of deposited amyloid in the tissues was small compared with that in other types of systemic amyloidosis, such as AL and secondary amyloidosis. Conclusion. These results suggest that the major reason why FAP patients show such severe gastrointestinal symptoms, compared with other types of systemic amyloidosis, may be because of thedeposition of a significant amount of amyloid in the nerves in the gastrointestinal tract.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 28/09/20 alle ore 13:51:26