{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T01:48:24Z","timestamp":1762998504744},"reference-count":43,"publisher":"FapUNIFESP (SciELO)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arq. Gastroenterol."],"published-print":{"date-parts":[[2003,6]]},"abstract":"<jats:p>RACIONAL: A polipose adenomatosa familiar \u00e9 doen\u00e7a heredit\u00e1ria de car\u00e1ter autoss\u00f4mico dominante, que freq\u00fcentemente se associa a numerosas manifesta\u00e7\u00f5es extracol\u00f4nicas. OBJETIVOS: Relatar a incid\u00eancia de manifesta\u00e7\u00f5es extracol\u00f4nicas em nosso meio e analisar seu impacto na evolu\u00e7\u00e3o da doen\u00e7a. PACIENTES E M\u00c9TODOS: Revis\u00e3o dos prontu\u00e1rios de pacientes com polipose adenomatosa familiar tratados no per\u00edodo de 1977 a 2001, relatando as manifesta\u00e7\u00f5es extracol\u00f4nicas associadas e suas complica\u00e7\u00f5es. RESULTADOS: Dos 59 pacientes com polipose adenomatosa familiar, 23 (38,9%) apresentaram alguma manifesta\u00e7\u00e3o extracol\u00f4nica por ocasi\u00e3o do diagn\u00f3stico ou no seguimento. Foram registradas 37 diferentes manifesta\u00e7\u00f5es (1,6 por paciente). As mais comuns foram osteomas e altera\u00e7\u00f5es na pigmenta\u00e7\u00e3o da retina, diagnosticadas em 25% e 20% dos pacientes pesquisados, respectivamente. Outras manifesta\u00e7\u00f5es extracol\u00f4nicas achadas foram adenomas do trato digestivo superior, cistos epiderm\u00f3ides, tumores desm\u00f3ides (sete cada), c\u00e2ncer g\u00e1strico (tr\u00eas) e c\u00e2ncer de tire\u00f3ide (dois). Complica\u00e7\u00f5es importantes diretamente relacionadas aos tumores desm\u00f3ides foram reportadas em seis pacientes, sendo obstru\u00e7\u00e3o intestinal em quatro e hidronefrose em dois. Registraram-se \u00f3bitos em dois pacientes (28,5%). CONCLUS\u00d5ES: A incid\u00eancia de manifesta\u00e7\u00f5es extracol\u00f4nicas \u00e9 alta (40%), podendo afetar a evolu\u00e7\u00e3o da doen\u00e7a e a qualidade de vida dos pacientes. Por esses motivos, torna-se de fundamental import\u00e2ncia a pesquisa, a preven\u00e7\u00e3o e o tratamento adequado dessas manifesta\u00e7\u00f5es na polipose adenomatosa familiar.<\/jats:p>","DOI":"10.1590\/s0004-28032003000200006","type":"journal-article","created":{"date-parts":[[2005,4,15]],"date-time":"2005-04-15T14:20:45Z","timestamp":1113574845000},"page":"92-98","source":"Crossref","is-referenced-by-count":16,"title":["Manifesta\u00e7\u00f5es extracol\u00f4nicas da polipose adenomatosa familiar: incid\u00eancia e impacto na evolu\u00e7\u00e3o da doen\u00e7a"],"prefix":"10.1590","volume":"40","author":[{"given":"F\u00e1bio Guilherme","family":"Campos","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angelita","family":"Habr-Gama","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Desid\u00e9rio Roberto","family":"Kiss","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F\u00e1bio C\u00e9sar","family":"Atu\u00ed","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"F\u00e1bio","family":"Katayama","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joaquim","family":"Gama-Rodrigues","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"530","reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1097\/00008469-199301000-00011","article-title":"Congenital hypertrophy of retinal pigment epithelium (CHRPE) as a marker for familial adenomatous polyposis (FAP)","volume":"2","author":"Bertario L","year":"1993","journal-title":"Eur J Cancer 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