Familial Adenomatous Polyposis
Pfupiso
Familial adenomatous polyposis (FAP) chirwere chisingaperi, chinogara kuri muropa, chinokonzerwa nekusakwana kwe gene ye adenomatous polyposis coli (APC). Vanhu vakawanda vanogara vachinowana gene kubva kuna mubereki. Asi kune 25 kusvika ku30 muzana yevanhu, kuchinja kwemajini kunoitika zvisingatarisirwi. FAP inokonzera tishu yekuwedzera (polyps) kuti ibude mumatumbu makuru (colon) uye rectum. Polyps inogonawo kuitika munzira yepamusoro yegastrointestinal, kunyanya chikamu chepamusoro chechidumbu chako chidiki (duodenum). Kana isina kurapwa, polyps iri mu colon ne rectum zvinogona kuva kenza kana uine makore makumi mana. Vanhu vakawanda vane familial adenomatous polyposis vanopedzisira vachida kuvhiyiwa kuti vabvise ura guru kudzivirira kenza. Polyps iri mu duodenum inogonawo kukura kenza, asi inogona kugadziriswa nekuchengetedza uye kubvisa polyps nguva nenguva. Vamwe vanhu vane chirwere chinenge chisinganyanyi kuoma, chinonzi attenuated familial adenomatous polyposis (AFAP). Vanhu vane AFAP vanowanzove ne polyps shoma dze colon (avhareji ye30) uye vanowana kenza gare gare muhupenyu.
Zviratidzo
Chiratidzo chikuru cheFAP ndeche mazana kana kunyange zviuru zve polyps zvinokura mumatumbo makuru ne rectum, kazhinji zvichiita kutanga pakati pemakore ekuyaruka. Polyps dzacho dzine chokwadi chekuita 100% kuti dzive cancer yematumbo makuru kana cancer ye rectum kana wasvika kumakore makumi mana.
Zvinokonzera
Familial adenomatous polyposis inokonzerwa nekusakwana kwegene rinowanzo gadzirwa kubva kuna mubereki. Asi vamwe vanhu vanowana gene isina kunaka inokonzera chirwere ichi.
Zvinhu zvinokonzera njodzi
Ngozi yako yekuwana familial adenomatous polyposis yakakwira kana uine mudzimai, mwana, munun'una, kana hanzvadzi ane chirwere ichi.
Matambudziko
Kuwedzera kukanza yemakoroni, familial adenomatous polyposis inogona kukonzera zvimwe zvinetso: Duodenal polyps. Aya mapolyps anokura muchikamu chepamusoro cheura hwako duku uye anogona kuva kenza. Asi nekuongororwa kwakanyatsojeka, duodenal polyps kazhinji inogona kuwanikwa uye kubviswa usati wasvika kenza. Periampullary polyps. Aya mapolyps anoitika uko bile nepancreas ducts inopinda duodenum (ampulla). Periampullary polyps inogona kuva kenza asi kazhinji inogona kuwanikwa uye kubviswa usati wasvika kenza. Gastric fundic polyps. Aya mapolyps anokura mukati mechidya chako. Desmoids. Aya maseru asina kenza anogona kuoneka chero kupi muviri asi kazhinji anokura munzvimbo yemudumbu (abdomen). Desmoids inogona kukonzera matambudziko akakomba kana ikakura mune nerves kana ropa vessels kana kuisa pressure kune dzimwe nhengo mumuviri mako. Dzinoverengeka kenza. Zvisingawanzoitiki, FAP inogona kukonzera kenza kukura mu thyroid gland yako, central nervous system, adrenal glands, chiropa kana dzimwe nhengo. Zvisina kenza (benign) ganda tumors. Benign bone growths (osteomas). Congenital hypertrophy ye retinal pigment epithelium (CHRPE). Aya ndiwo pigment shanduko isina kenza mu retina yeziso rako. Dental abnormalities. Izvi zvinosanganisira mazino akawedzerwa kana mazino asingauyi. Nhamba shoma ye red blood cells (anemia).
Kudzivirira
Kudzivirira FAP hakuitike, sezvo iri chirwere chinogara chiri mumagene. Zvisinei, kana iwe kana mwana wako vari pangozi yeFAP nekuda kwehama ine chirwere ichi, uchazoda bvunzo yemajini nekurairwa. Kana uine FAP, uchazoda kuongororwa nguva dzose, uchiteverwa nekuvhiyiwa kana zvichidiwa. Kuvhiyiwa kunogona kubatsira kudzivirira kukura kenza yemakoroni kana zvimwe zvinetso.
Kuongorora
Uri panjodzi yekuva nefamilial adenomatous polyposis kana uine mubereki, mwana, munun'una kana hanzvadzi ine chirwere ichi. Kana uri panjodzi, zvakakosha kuongororwa kazhinji, kutanga muhudiki. Kuongororwa pagore kunogona kuona kukura kwemapolyps usati asvika pakenza.
- Sigmoidoscopy. Chubhu inotsvedza inoiswa mumatumbu ako kuti uongorore rectum ne sigmoid — mamita maviri ekupedzisira ecolon. Kune vanhu vane chirwere cheFAP kana nhengo dzemhuri dziri panjodzi dzisina kuitwa bvunzo yemajini, American College of Gastroenterology inokurudzira sigmoidoscopy pagore, kutanga pamakore gumi kusvika gumi nemaviri.
- Colonoscopy. Chubhu inotsvedza inoiswa mumatumbu ako kuti uongorore colon yese. Kana mapolyps akawanikwa mucolon yako, unofanirwa kuita colonoscopy pagore kusvika waopareta kubvisa colon yako.
- Esophagogastroduodenoscopy (EGD) uye side-viewing duodenoscopy. Marudzi maviri emakamera anoshandiswa kuongorora esophagus, dumbu nechikamu chekumusoro cheura duku (duodenum ne ampulla). Chiremba anogona kubvisa sampuli diki yetsinga (biopsy) kuti aongorore zvakare.
- CT kana MRI. Kuongororwa kwemudumbu ne pelvis kunogona kushandiswa, kunyanya kuongorora matumour e desmoid.
Bvunzo iri nyore yeropa inogona kuona kana uine gene isina kunaka inokonzera FAP. Kuongororwa kwemajini kunogona zvakare kuona kana uri panjodzi yematambudziko eFAP. Chiremba wako anogona kukurudzira bvunzo yemajini kana:
- Uine nhengo dzemhuri dzine FAP
- Une zvimwe, asi kwete zvese, zviratidzo zveFAP
Kubvisa FAP kunoponesa vana vari panjodzi makore ekutsvaga uye kushushikana kwepfungwa. Kune vana vane gene, kuongororwa kwakakodzera nekurapa kunoderedza njodzi yekenza zvikuru.
Chiremba wako anogona kukurudzira bvunzo dze thyroid nebvunzo dzakasiyana kuti aone mamwe matambudziko emuviri anogona kuitika kana uine FAP.
Kurapa
Pakutanga, chiremba wenyu achabvisa chero polyps diki dzakawanikwa pakuongororwa kwako kwecolonoscopy. Zvisinei, pakupedzisira, polyps idzodzo dzichava dzakawanda zvekuti hadzikwanisi kubviswa imwe neimwe, kazhinji kazhinji panguva yauchangopinda mumakore ekuma20. Ipapo uchazoda kuvhiyiwa kudzivirira kenza yecolon. Uchazodawo kuvhiyiwa kana polyp iri kenza. Ungasazoda kuvhiyiwa kweAFAP.
Nyanzvi yako yekuvhiya inogona kusarudza kuita kuvhiya kwako laparoscopically, kuburikidza nekucheka duku kwakawanda kunoda chete kushona rimwe kana maviri kuti kuvharwa. Iyi kuvhiya shoma inonyanya kukanganisa kazhinji inopfupisa nguva yako yekurwara muchipatara uye inokubvumira kupora nekukurumidza.
Zvichienderana nemamiriro ako ezvinhu, ungave une rumwe rudzi rwekubvhiyiwa kuti ubvise chikamu kana zvese zvecolon:
- Subtotal colectomy ne ileorectal anastomosis, umo rectum inosiiwa munzvimbo
- Total proctocolectomy ne continent ileostomy, umo colon ne rectum zvinobviswa uye kuvhurwa (ileostomy) kunogadzirwa, kazhinji kurutivi rwerudyi rwechidumbu chako
- Total proctocolectomy ne ileoanal anastomosis (inodaidzwawo kuti kuvhiya kweJ-pouch), umo colon ne rectum zvinobviswa uye chikamu cheura duku chinobatanidzwa ne rectum
Kuvhiya hakurapi FAP. Polyps inogona kuramba ichigadzira muzvikamu zvakasara kana zvakavakwa patsva zvecolon yako, dumbu neura duku. Zvichienderana nehuwandu nehukuru hwe polyps, kuzvibvisa endoscopically kungasakwana kuderedza njodzi yako yekenza. Ungada kuvhiyiwa kuwedzerwa.
Uchazoda kuongororwa nguva dzose — uye kurapwa kana zvichidiwa — kune matambudziko e familial adenomatous polyposis anogona kukura mushure mekuvhiya kwecolorectal. Zvichienderana ne nhoroondo yako uye rudzi rwekubvhiyiwa rwauwana, kuongororwa kunogona kusanganisira:
- Sigmoidoscopy kana colonoscopy
- Upper endoscopy
- Thyroid ultrasound
- CT kana MRI kuongorora matumors e desmoid
Zvichienderana nemhedzisiro yekuongororwa kwako, chiremba wako anogona kuwedzera kurapwa kwezvinetso zvinotevera:
- Duodenal polyps ne periampullary polyps. Chiremba wako anogona kukurudzira kuvhiyiwa kubvisa chikamu chepamusoro cheura duku (duodenum ne ampulla) nekuti marudzi aya e polyps anogona kuenderera mberi kuita kenza.
- Desmoid tumors. Ungapihwa musanganiswa wemishonga, kusanganisira mishonga isina steroid anti-inflammatory, anti-estrogen ne chemotherapy. Muzvimwe zviitiko, ungada kuvhiyiwa.
- Osteomas. Vanachiremba vanogona kubvisa aya matumors asina kenza emapfupa kuti vadzikamise marwadzo kana zvikonzero zvekunaka.
Vatsvakurudzi vanoramba vachiongorora kurapwa kuwedzerwa kweFAP. Zvikuru, kushandiswa kwemishonga inorwisa marwadzo senge aspirin ne mishonga isina steroid anti-inflammatory (NSAIDs), pamwe ne mushonga we chemotherapy, iri kuongororwa.
Vamwe vanhu vanoona zvinobatsira kutaura nevamwe vanhu vane zvakaenzana. Funga kubatana nechikwata chekutsigira pamhepo, kana kubvunza chiremba wako nezvemapoka ekutsigira munzvimbo yako.
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