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Titolo:
ESOPHAGEAL SMOOTH-MUSCLE DYSFUNCTION IN OCULOPHARYNGEAL MUSCULAR-DYSTROPHY
Autore:
TIOMNY E; KHILKEVIC O; KORCZYN AD; KIMMEL R; HALLAK A; BARON J; BLUMEN S; ASHEROV A; GILAT T;
Indirizzi:
ICHILOV HOSP,DEPT NEUROL,TEL AVIV SOURASKI MED CTR,6 WEIZMANN ST IL-64345 TEL AVIV ISRAEL ICHILOV HOSP,DEPT NEUROL,TEL AVIV SOURASKI MED CTR IL-64345 TEL AVIV ISRAEL ICHILOV HOSP,DEPT GASTROENTEROL IL-64239 TEL AVIV ISRAEL TEL AVIV UNIV,SACKLER FAC MED IL-69978 TEL AVIV ISRAEL
Titolo Testata:
Digestive diseases and sciences
fascicolo: 7, volume: 41, anno: 1996,
pagine: 1350 - 1354
SICI:
0163-2116(1996)41:7<1350:ESDIOM>2.0.ZU;2-1
Fonte:
ISI
Lingua:
ENG
Keywords:
OCULOPHARYNGEAL MUSCULAR DYSTROPHY; ESOPHAGUS; MOTILITY; MANOMETRY; SCINTIGRAPHY; SMOOTH MUSCLE; DYSPHAGIA;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
16
Recensione:
Indirizzi per estratti:
Citazione:
E. Tiomny et al., "ESOPHAGEAL SMOOTH-MUSCLE DYSFUNCTION IN OCULOPHARYNGEAL MUSCULAR-DYSTROPHY", Digestive diseases and sciences, 41(7), 1996, pp. 1350-1354

Abstract

Oculopharyngeal muscular dystrophy (OPMD) is a rare genetic disorder with late-onset progressive myopathy affecting mainly head and neck striated muscles. It is more common in certain ethnic communities. Dysphagia was usually attributed to the malfunction of striated pharyngeal muscles. We studied a group of Bukharan immigrants affected by this disorder (N = 13). Esophageal studies, including endoscopy, manometry, and scintigraphic emptying were performed. Very low pharyngeal pressures were measured. Upper esophageal pressures (UEP) were in the normal range in eight patients, and above normal in three patients. Four also had low lower esophageal sphincter pressure. Esophageal body peristaltic activity was grossly impaired in all. patients: mainly nonpropulsive, simultaneous, retrograde, and failed activity was recorded. Marked retention of isotopic material was demonstrated in all patients studied, usually in the middle and lower parts of the body, ranging from 17 to 100% retention. The dysphagia in OPMD is due not-only to dysfunction of pharyngeal and upper esophageal striated muscle, but also has a significant smooth muscle component.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 01/10/20 alle ore 01:24:49