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Titolo:
Bannayan-Riley-Ruvalcaba syndrome: Spectrum of intestinal pathology including juvenile polyps
Autore:
Lowichik, A; White, FV; Timmons, CF; Weinberg, AG; Gunasekaran, TS; Nathan, K; Coffin, CM;
Indirizzi:
Univ Utah, Sch Med, Div Pediat Pathol, Salt Lake City, UT 84113 USA Univ Utah Salt Lake City UT USA 84113 athol, Salt Lake City, UT 84113 USA Primary Childrens Med Ctr, Dept Pathol, Salt Lake City, UT 84113 USA Primary Childrens Med Ctr Salt Lake City UT USA 84113 City, UT 84113 USA Washington Univ, Sch Med, Lauren V Ackerman Div Surg Pathol, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 Surg Pathol, St Louis, MO 63110 USA Childrens Med Ctr, Dept Pathol, Dallas, TX 75235 USA Childrens Med Ctr Dallas TX USA 75235 , Dept Pathol, Dallas, TX 75235 USA Univ Texas, SW Med Ctr, Dallas, TX 75235 USA Univ Texas Dallas TX USA 75235 iv Texas, SW Med Ctr, Dallas, TX 75235 USA Lutheran Gen Childrens Hosp, Div Pediat Gastroenterol, Pk Ridge, IL 60068 USA Lutheran Gen Childrens Hosp Pk Ridge IL USA 60068 Pk Ridge, IL 60068 USA Loyola Med Ctr, Maywood, IL 60153 USA Loyola Med Ctr Maywood IL USA 60153Loyola Med Ctr, Maywood, IL 60153 USA
Titolo Testata:
PEDIATRIC AND DEVELOPMENTAL PATHOLOGY
fascicolo: 2, volume: 3, anno: 2000,
pagine: 155 - 161
SICI:
1093-5266(200003/04)3:2<155:BSSOIP>2.0.ZU;2-C
Fonte:
ISI
Lingua:
ENG
Soggetto:
HEREDITARY GASTROINTESTINAL POLYPOSIS; COWDEN-DISEASE; LOCALIZATION; MUTATIONS; PTEN; GENE;
Keywords:
Bannayan-Riley-Ruvalcaba syndrome; juvenile polyposis; ectopic ganglion cells; atypical polyps;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
27
Recensione:
Indirizzi per estratti:
Indirizzo: Lowichik, A Univ Utah, Sch Med, Div Pediat Pathol, 100 N Med Dr, Salt LakeCity, UT 84113 USA Univ Utah 100 N Med Dr Salt Lake City UT USA 84113 T 84113 USA
Citazione:
A. Lowichik et al., "Bannayan-Riley-Ruvalcaba syndrome: Spectrum of intestinal pathology including juvenile polyps", PEDIATR D P, 3(2), 2000, pp. 155-161

Abstract

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a disorder that includes juvenile polyposis as part of its pathologic spectrum, and it recently has been shown to share phenotypic and genotypic features with Cowden's disease. In existing literature, descriptions of intestinal pathology in patients with ERRS are relatively sparse and occasionally erroneous. We describe the intestinal pathology in multiple specimens from three children with ERRS. Examination of gastrointestinal biopsies from these children revealed predominantly colonic and rectal polyps with the histology of juvenile polyps. Additionally, two cases with clusters of ectopic ganglion cells within the laminapropria, one in a colonic polyp and one in a duodenal biopsy, and an atypical polyp were observed. Bannayan-Riley-Ruvalcaba syndrome should be included in the list of differential diagnostic considerations when a child or young adult presents with a juvenile polyp, particularly if unusual histologic features such as atypical polyps or ectopic ganglion cells are encountered.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 19/09/20 alle ore 15:25:04