{"id":188,"date":"2018-11-29T14:50:56","date_gmt":"2018-11-29T12:50:56","guid":{"rendered":"https:\/\/www.barnaclinic.com\/blog\/cirugia-del-pancreas\/ca\/?p=188"},"modified":"2018-11-29T14:50:56","modified_gmt":"2018-11-29T12:50:56","slug":"fibrosis-quistica-pancrees","status":"publish","type":"post","link":"https:\/\/www.barnaclinic.com\/blog\/cirugia-del-pancreas\/ca\/fibrosis-quistica-pancrees\/","title":{"rendered":"La fibrosi qu\u00edstica: Qu\u00e8 \u00e9s, com afecta al p\u00e0ncrees i com tractar-la"},"content":{"rendered":"

La\u00a0fibrosi qu\u00edstica<\/strong>\u00a0(FQ, tamb\u00e9 anomenada\u00a0mucoviscidosi)<\/strong>\u00a0\u00e9s una malaltia que, hist\u00f2ricament, es definia com la pres\u00e8ncia de moc esp\u00e8s i de\u00a0fibrosi en\u00a0el p\u00e0ncrees<\/strong>\u00a0i \/ o en el pulm\u00f3.<\/strong><\/span>\u00a0La\u00a0fibrosi qu\u00edstica<\/strong>\u00a0generalment s’iniciava en edats molt primerenques (inf\u00e0ncia), comportava una insufici\u00e8ncia respirat\u00f2ria progressiva i t\u00e9 molt mal pron\u00f2stic per al malalt ja que pocs arribaven l’edat adulta.<\/span>\u00a0Avui en dia, el diagn\u00f2stic i el tractament de la\u00a0fibrosi qu\u00edstica<\/strong>\u00a0han evolucionat molt, de tal manera que l’esperan\u00e7a de vida dels malalts ha augmentat de forma molt notable, i pot apreciar altres manifestacions cl\u00edniques que pr\u00e8viament eren desconegudes.<\/span><\/span><\/p>\n

FIBROSI QU\u00cdSTICA O MALALTIES ASSOCIADES A MUTACIONS DEL GEN CFTR?<\/span><\/h2>\n

La\u00a0fibrosi qu\u00edstica<\/strong>\u00a0es produeix com a conseq\u00fc\u00e8ncia de la pres\u00e8ncia en l’ADN del pacient d’una o diverses mutacions en les dues c\u00f2pies del\u00a0gen CFTR<\/a><\/strong>\u00a0(una provinent de la mare i una altra del pare).\u00a0<\/span>El gen CFTR t\u00e9 com a finalitat produir una prote\u00efna que facilitar\u00e0 l’intercanvi de diversos ions, com el clor, entre l’interior i l’exterior de la c\u00e8l\u00b7lula.<\/span>\u00a0A dia d’avui\u00a0es coneixen m\u00e9s de 1000 mutacions diferents d’aquest gen,<\/strong>\u00a0i el tipus de mutaci\u00f3 espec\u00edfica determinar\u00e0 les conseq\u00fc\u00e8ncies cl\u00edniques.<\/span>\u00a0Com a factor com\u00fa, aquestes mutacions comportaran un augment del gruix de les secrecions que, depenent on estiguin, produiran un determinat quadre cl\u00ednic.<\/span>\u00a0Aix\u00ed, en augmentar l’esperan\u00e7a de vida, sabem que\u00a0la fibrosi qu\u00edstica pot afectar, a m\u00e9s dels pulmons o\u00a0el p\u00e0ncrees\u00a0, a la pr\u00e0ctica totalitat de l’aparell digestiu,<\/strong>\u00a0ocasionant quadres d’obstrucci\u00f3 intestinal,\u00a0o l’aparell reproductor mascul\u00ed,\u00a0<\/strong>produint esterilitat.<\/span>\u00a0\u00c9s m\u00e9s, s’ha detectat mutacions en una de les dues c\u00f2pies de l’ADN que poden estar relacionades amb alteracions digestives, biliars o pancre\u00e0tiques que no encaixen amb la forma cl\u00e0ssica de FQ;<\/span>\u00a0per tant,\u00a0seria m\u00e9s exacte parlar de malalties associades a mutacions del gen CFTR.<\/strong><\/span><\/p>\n

\n

\"dr-santiago-sanchez-Cabus\"<\/h4>\n

“La fibrosi qu\u00edstica pot afectar, a m\u00e9s dels pulmons o\u00a0el p\u00e0ncrees\u00a0, a la pr\u00e0ctica totalitat de l’aparell digestiu<\/strong>\u00a0o l’aparell reproductor mascul\u00ed”<\/strong><\/span><\/h4>\n<\/blockquote>\n

COM AFECTA LA FIBROSI QU\u00cdSTICA AL P\u00c0NCREES?<\/span><\/h2>\n

La forma cl\u00e0ssica de fibrosi qu\u00edstica afecta el p\u00e0ncrees produint una p\u00e8rdua de la funci\u00f3 pancre\u00e0tica,<\/strong>\u00a0tant exocrina com, en algunes ocasions, endocrina.<\/span>\u00a0En l’exploraci\u00f3 radiol\u00f2gica d’un pacient amb FQ s’apreciar\u00e0 una atr\u00f2fia molt important de la gl\u00e0ndula pancre\u00e0tica, amb la pres\u00e8ncia de\u00a0m\u00faltiples quists\u00a0(important\u00edssim no confondre amb la pres\u00e8ncia d’altres tipus de\u00a0quists pancre\u00e0tics amb capacitat de degeneraci\u00f3<\/strong>) o fins i tot la substituci\u00f3 del teixit pancre\u00e0tic per una acumulaci\u00f3 de l\u00edquid muc\u00f3s.<\/span>\u00a0El tractament de la “fibrosi qu\u00edstica del p\u00e0ncrees” ha d’estar centrat en suplir la\u00a0funci\u00f3 que el p\u00e0ncrees ha perdut\u00a0mitjan\u00e7ant enzims pancre\u00e0tics i, si \u00e9s necess\u00e0ria, l’administraci\u00f3 d’insulina.<\/strong><\/span><\/p>\n

\n

\"dr-santiago-sanchez-Cabus\"<\/h4>\n

“La forma cl\u00e0ssica de fibrosi qu\u00edstica afecta el p\u00e0ncrees produint una p\u00e8rdua de la funci\u00f3 pancre\u00e0tica,<\/strong>\u00a0tant exocrina com, en algunes ocasions, endocrina”<\/span><\/h4>\n<\/blockquote>\n

ES POT TRACTAR LA “FIBROSI QU\u00cdSTICA DEL P\u00c0NCREES” AMB CIRURGIA?<\/span><\/h2>\n

\u00d2bviament, per l’anteriorment exposat,\u00a0la cirurgia no t\u00e9 un paper en el tractament de la fibrosi qu\u00edstica<\/strong>\u00a0com a tal, i especialment en el p\u00e0ncrees;<\/span>\u00a0no obstant aix\u00f2, \u00e9s possible que alguns pacients amb FQ acabin precisant en algun moment un tractament quir\u00fargic.<\/span>\u00a0Tot i aix\u00ed,\u00a0generalment es deu a la pres\u00e8ncia d’una obstrucci\u00f3 intestinal.<\/span>\u00a0Per tant,\u00a0la cirurgia ha de reservar-se \u00fanicament per al tractament de les complicacions.<\/strong><\/span><\/p>\n

\n

\"cita-sanchez-cabus-cirugia-del-pancreas\"<\/a><\/p>\n","protected":false},"excerpt":{"rendered":"

La\u00a0fibrosi qu\u00edstica\u00a0(FQ, tamb\u00e9 anomenada\u00a0mucoviscidosi)\u00a0\u00e9s una malaltia que, hist\u00f2ricament, es definia com la pres\u00e8ncia de moc esp\u00e8s i de\u00a0fibrosi en\u00a0el p\u00e0ncrees\u00a0i \/ o en el pulm\u00f3.\u00a0La\u00a0fibrosi qu\u00edstica\u00a0generalment s’iniciava en edats molt primerenques (inf\u00e0ncia), comportava una insufici\u00e8ncia respirat\u00f2ria progressiva i t\u00e9 molt mal pron\u00f2stic per al malalt ja que pocs arribaven l’edat adulta.\u00a0Avui en dia, el diagn\u00f2stic […]<\/p>\n","protected":false},"author":2,"featured_media":193,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[44],"tags":[61,54,60,57],"yoast_head":"\nLa fibrosi qu\u00edstica: Qu\u00e8 \u00e9s, com afecta al p\u00e0ncrees i com tractar-la - Blog de Cirug\u00eda del P\u00e1ncreas<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.barnaclinic.com\/blog\/cirugia-del-pancreas\/ca\/fibrosis-quistica-pancrees\/\" \/>\n<meta property=\"og:locale\" content=\"ca_ES\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"La fibrosi qu\u00edstica: Qu\u00e8 \u00e9s, com afecta al p\u00e0ncrees i com tractar-la - 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