Kodi pali aliyense m'banja mwanu amene wakhala ndi khansa ya m'matumbo? Tiyeni tikambirane za matenda a majini awa (Familial Adenomatous Polyposis - FAP)!

Kodi pali aliyense m'banja mwanu, mwina ali wamng'ono, amene wadwala khansa ya m'matumbo? Kapena dokotala wakuuzani kuti muli ndi zotupa zazing'ono (polyps) m'matumbo anu pamodzi ndi mavuto am'mimba? Kaya mwamvapo za chinthu choterocho kapena ayi, kungakhale kofunikira kwambiri kudziwa za vuto ili lotchedwa `FAP` lomwe tikambirane lero. Chifukwa ngakhale kuti ndi losowa pang'ono, ngati litazindikirika msanga, lingakupulumutseni ku mavuto akuluakulu ambiri.

Kodi FAP ndi chiyani mwachidule?

Mwachidule, Familyal Adenomatous Polyposis, kapena FAP mwachidule, ndi matenda a majini omwe amachititsa kuti tinthu tating'onoting'ono tomwe timatchedwa adenomas, tomwe timakhala khansa, tipangike m'matumbo anu ndipo nthawi zina mkati mwa rectum yanu.

Taganizirani izi, anthu ena amakhala ndi chotupa chimodzi kapena ziwiri m'matumbo awo akamakula. Zimenezo n'zachibadwa. Koma kwa munthu amene ali ndi `FAP`, nthawi zambiri amakhala ndi zotupa zoposa zana, nthawi zina zikwizikwi , koma amayamba kukula ali wamng'ono kwambiri, mwina ali ndi zaka khumi. Chifukwa chake, mwayi woti chimodzi mwa zotupazi chisanduke khansa (`khansa ya m'matumbo`), kutanthauza kuti, chiopsezo cha moyo wonse, ndi chachikulu kwambiri .

Pofuna kuwongolera chiopsezochi, madokotala nthawi zambiri amalimbikitsa opaleshoni yochotsa m'matumbo onse (kuchotsa kwathunthu kwa m'mimba) . Nthawi zina, rectum imachotsedwanso (kuchotsa kwa m'mimba). Izi zili choncho chifukwa mu FAP, zotupa izi zimakula mofulumira kwambiri moti sizingathe kuthetsedwa pozichotsa chimodzi ndi chimodzi. Ndipotu, ngati opaleshoniyi siichitika, anthu ambiri omwe ali ndi FAP adzadwala khansa akafika zaka zapakati .

Anthu omwe ali ndi FAP amatha kukhala ndi mitundu ina ya zotupa, osati m'matumbo okha, komanso m'malo ena, monga pakhungu, minofu yofewa, mano, ndi mafupa. Ngakhale m'matumbo mutachotsedwa, mungafunike kupitiliza kuyezetsa kuti muwone ngati muli ndi zotupa zina izi, ndipo mungafunikenso kuchitidwa opaleshoni.

Kodi chiopsezo chotenga khansa kwa anthu omwe ali ndi FAP ndi chotani?

Ngati munthu amene ali ndi FAP sanalandire chithandizo, ali ndi mwayi wokwana 100% wokhala ndi khansa ya m'matumbo. Komanso, khansayo imatha kuyamba msanga komanso ali wamng'ono kuposa anthu ena. Ngati wachibale wake akudziwika kuti ali ndi vutoli, ana m'mabanja amenewo ayenera kuchitidwa opaleshoni ya m'matumbo chaka chilichonse kuyambira ali ndi zaka 10 .

Kuwonjezera pa khansa ya m'matumbo, anthu omwe ali ndi FAP ali pachiwopsezo chotenga mitundu ina ya khansa:

• Khansa ya m'mimba mwaing'ono (khansa ya duodenal) - pafupifupi 8%
• Khansa ya chithokomiro cha papillary - pafupifupi 2%
• Khansa ya kapamba - pafupifupi 2%
• Khansa ya chiwindi yotchedwa ``Hepatoblastoma`` (makamaka mwa ana) - pafupifupi 1.5%
• Khansa ya m'mimba - pafupifupi 1%
• Zotupa za muubongo ndi msana - zosakwana 1%

Kodi pali mitundu yosiyanasiyana ya ma FAP?

Inde, pali mtundu waukulu wa `FAP` ndi mitundu ina ingapo yowonjezerapo.

• "Classic" FAP: Izi zimaphatikizapo ma adenoma opitilira 100 m'matumbo.
• "Attenuated" FAP (AFAP): Iyi ndi mtundu wochepa kwambiri. Pali ma cysts pakati pa 20 ndi 100 m'matumbo.
• Matenda a Gardner: Monga `FAP` yakale, pali zotupa zoposa 100 m'matumbo. Kuphatikiza apo, mitundu ina ya zotupa imapezeka kwina m'thupi.
• Matenda a Turcot: Chotupa cha khansa chimakula muubongo pamodzi ndi zotupa zingapo m'matumbo.

Kodi vutoli limatchedwa FAP ndi lofala bwanji?

FAP ndi matenda osowa kwambiri . Amakhudza pafupifupi munthu m'modzi mwa anthu 8,000. FAP imapanga pafupifupi 0.5% ya khansa zonse za m'matumbo. Mitundu ina monga AFAP, Gardner syndrome, ndi Turcotte syndrome ndi yosowa kwambiri.

Kodi kusiyana pakati pa FAP ndi Lynch syndrome ndi kotani?

Matenda a Lynch ndi matenda ena obadwa nawo omwe amawonjezera chiopsezo chotenga khansa ya m'matumbo ndi khansa zina. Matenda a Lynch amatchedwanso HNPCC (hereditary nonpolyposis colorectal cancer syndrome). Monga momwe dzinalo likusonyezera, izi sizikutanthauza kuti pamakhala zotupa zambiri za m'matumbo .

Anthu omwe ali ndi matenda a Lynch amatha kukhala ndi khansa ngakhale ali ndi chotupa chimodzi kapena zingapo m'matumbo. Komanso, zotupa ndi khansa zimakula mochedwa kuposa mu FAP, ndipo chiopsezo chake chimakhala chochepa pang'ono. Matenda awiriwa amayamba chifukwa cha kusintha kwa majini osiyanasiyana .

Kodi zizindikiro za FAP ndi ziti? Kodi timazizindikira bwanji?

Mu FAP, ma polyp a m'matumbo amayamba kupangika msanga kuposa anthu ambiri, nthawi zambiri ali achinyamata . Ma polyp amenewa sangayambitse zizindikiro zilizonse mpaka atakula kwambiri. Koma ngati mwachitidwa opaleshoni ya colonoscopy, dokotala wanu angawapeze.

Anthu omwe ali ndi `FAP` akhoza kukhala ndi ma polyp mazana kapena zikwi m'matumbo awo . Anthu omwe ali ndi `AFAP` ali ndi osachepera 20. Chifukwa ma polyp ndi ambiri ndipo amakula mwachangu mu `FAP`, amakhala ndi mwayi waukulu woyambitsa zizindikiro kuposa zomwe zimayambitsa ma polyp. Zizindikiro zake ndi izi:

• Kutuluka magazi m'thupi
• Kutsegula m'mimba
• Kupweteka kwa m'mimba kosatha

Anthu omwe ali ndi FAP nthawi zina amakhala ndi zizindikiro zomwe madokotala amatha kuzizindikira kunja:

• Dermatofibromas: Matumbo okhala ndi ulusi, ngati zipsera pansi pa khungu.
• Ma epidermal cysts: Matumbo ozungulira omwe ali pansi pa khungu, odzaza ndi keratin.
• Mafupa a Osteoma: Zotupa zomwe sizili khansa zomwe zimapangidwa m'mafupa. Zimapezeka kwambiri m'chibwano kapena m'chigaza.
• Mano owonjezera kapena mano okhudzidwa:Izi zitha kuwoneka pa X-ray ya mano.
• Congenital hypertrophy of the retinal pigment epithelium (CHRPE): Izi zitha kuoneka panthawi yoyezetsa maso. Komabe, nthawi zambiri sizimayambitsa mavuto a maso.

Zizindikiro za FAP nthawi zambiri zimayamba pa msinkhu wanji?

Mu FAP, kukula kwa m'matumbo nthawi zambiri kumayamba pafupifupi zaka 16. Mu AFAP, kungayambe pambuyo pake. Ngati makolo anu ndi madokotala akudziwa kuti muli pachiwopsezo cha vutoli, angayambe kukuyesani mutakwanitsa zaka 10. Ngati simutero, mungadziwike kuti muli ndi vutoli chifukwa cha zizindikiro zanu.

Kodi chifukwa chachikulu cha FAP ndi chiyani?

Chifukwa chachikulu cha FAP ndi kusintha kwa majini . Jini iyi imatchedwa jini ya adenomatous polyposis coli (APC) . Imatchedwanso jini yoletsa chotupa. Izi zikutanthauza kuti jini iyi imaletsa maselo kukula mosalamulirika ndikupanga zotupa. Kusintha kwa majini kukachitika, ntchito ya jini iyi imasokonekera.

Kusintha kwa majini komwe kumayambitsa FAP ndi kusintha kwa majini. Izi zikutanthauza kuti si chinthu chomwe chimachitika munthu akangobadwa, koma chinthu chomwe chimachitika munthu akatenga pakati . Ichi ndi chinthu chomwe chimapatsiridwa kuchokera ku mibadwomibadwo. Ngati m'modzi mwa makolo anu ali ndi kusintha kumeneku, muli ndi mwayi wa 50% wolandira . Komabe, pafupifupi 30% ya kusintha kumeneku ndi kwatsopano (kusintha koyambirira), ndiko kuti, sikutengera cholowa . Chifukwa chake, pafupifupi 30% ya odwala omwe adapezeka ndi FAP mwina alibe mbiri ya banja la matendawa.

Kodi mavuto omwe angakhalepo chifukwa cha FAP ndi ati?

Vuto lofunika kwambiri lomwe mungathane nalo mu FAP ndi khansa ya m'matumbo . Opaleshoni yochotsa m'matumbo anu (colectomy) ingachepetse kwambiri chiopsezochi. Komabe, kukhala opanda m'matumbo kungakhale ndi zotsatirapo zina pa moyo wanu chifukwa momwe mumachitira chopondapo chimasintha. Mungafunike kugwiritsa ntchito thumba la ileostomy kapena thumba la ileal m'malo mwa thumba la m'matumbo.

Popeza kusintha kwa majini komwe kumayambitsa FAP kumapezeka mu selo iliyonse m'thupi lanu, zotupa zimatha kufalikira kulikonse m'thupi lanu . Zotupa zina zimathanso kufalikira kunja kwa m'matumbo, ndipo nthawi zina zimatha kukhala khansa. Dokotala wanu angakulangizeni nthawi yoyezetsa kuti muwone mitundu iyi ya zotupa:

• Ma polyps a m'mimba mwa duodenal/periampullary:Izi zimakula kumtunda kwa matumbo anu ang'onoang'ono (duodenum), kapena komwe duct ya bile kapena pancreatic duct imalumikizana ndi matumbo ang'onoang'ono. Pafupifupi aliyense amene ali ndi FAP amakhala ndi izi. Anthu ochepa amatha kudwala khansa ya duodenal, khansa ya ampullary, khansa ya pancreatic mu duct, kapena khansa ya bile duct.
• Ma polyps a m'mimba: Pafupifupi 90% ya anthu omwe ali ndi FAP amakhala ndi ma polyps a m'mimba, koma 1% yokha mwa awa imasanduka khansa.
• Zotupa za Desmoid: Izi ndi zotupa zopanda khansa zomwe zimapangidwa m'minofu yolumikizana. Zimapezeka mwa anthu pafupifupi 15% omwe ali ndi FAP. Ngakhale kuti si khansa, nthawi zina (pafupifupi 5%) zimayambitsa mavuto aakulu azaumoyo komanso imfa. Zitha kukhala zaukali kwambiri, kufalikira ku ziwalo zapafupi, ndikukakamiza kapena kutseka mitsempha yamagazi, ziwalo, ndi mitsempha. N'zovuta kuzichotsa ndipo zimatha kubwereranso.
• Khansa ya chithokomiro cha papillary: Mtundu uwu wa khansa ya chithokomiro umapezeka mwa anthu pafupifupi 2% omwe ali ndi FAP. Ndi woopsa pang'ono ndipo nthawi zambiri umachiritsidwa.
• Khansa ya chiwindi: Ana omwe ali ndi FAP, makamaka, ali ndi chiopsezo chochepa chotenga khansa ya chiwindi yosowa kwambiri yotchedwa hepatoblastoma.
• Medulloblastoma: Iyi ndi khansa ya muubongo. Imatha kuchitika ndi Turcot syndrome, yomwe imagwirizana ndi FAP. Ngakhale ndi FAP, siichitika kawirikawiri.
• Ma polyps a m'matumbo: Ngati simuchotsa rectum yanu pamodzi ndi m'matumbo anu, muli pachiwopsezo chokhala ndi ma polyps a m'matumbo, kotero muyenera kuyesedwa proctoscopy moyo wanu wonse.

Kodi mungadziwe bwanji ngati muli ndi FAP?

Ngati mukufuna kudziwa ngati mwalandira APC gene mutation, mutha kupeza yankho mwa kuchita mayeso a majini . Kuyesa majini kumatenga chitsanzo cha DNA yanu (monga magazi kapena malovu) ndikuyang'ana kusintha kwa majini enaake. Ngati muli ndi kusinthaku, gawo lotsatira ndikuchitidwa colonoscopy kuti muwone ngati muli ndi adenomas.

FAP imapezeka ngati pali ma polyps osachepera 100 (20 ngati AFAP) komanso kupezeka kwa kusintha kwa majini a APC . FAP si vuto lokhalo lobadwa nalo lomwe limayambitsa ma polyps adenomatous. MUTYH-associated polyposis ndi vuto lofanana, koma limayambitsidwa ndi kusintha kwa majini ena otchedwa MUTYH.

Kodi ndondomeko ya chithandizo cha munthu amene ali ndi FAP ndi yotani?

Ndondomeko ya chithandizo cha FAP imaphatikizapo kuyang'aniridwa kwa moyo wonse ndi opaleshoni yokakamiza.Poyamba, ngati muli ndi ma polyps ochepa (kapena AFAP), dokotala wanu akhoza kuwachotsa payekhapayekha panthawi ya colonoscopy (polypectomy). Ma polyps akakula kwambiri kapena kukhala khansa, opaleshoni ingafunike.

Opaleshoni

Anthu ambiri omwe ali ndi FAP yachikhalidwe adzafunika kuchitidwa opaleshoni yonse ya colectomy ali kumapeto kwa zaka za m'ma 20 kapena kumayambiriro kwa zaka za m'ma 30. Dokotala wanu adzasankha nthawi yoti achite opaleshoni yanu kutengera zoopsa zanu. Adzakambirana nanunso za mitundu yosiyanasiyana ya opaleshoni ya colectomy yomwe mungakhale nayo.

M'mimba mwanu mukachotsedwa, pamakhala mpata pakati pa matumbo anu ang'onoang'ono ndi rectum (kapena anus). Nthawi zina, dokotala wa opaleshoni amatha kulumikizanso mbali ziwirizi. Kenako mutha kuchita chimbudzi kudzera mu rectum mwachizolowezi. Ngati sizingatheke, amapanga 'ostomy', yomwe ndi mpata watsopano m'mimba mwanu kuti choponda chanu chituluke.

Kuwunika

Gulu lanu lachipatala lidzakulangizani za kuchuluka kwa nthawi yomwe muyenera kuyezetsa mitundu yosiyanasiyana ya zotupa, kutengera zomwe zimayambitsa chiopsezo chanu. Pa FAP yakale, colonoscopy ya pachaka imalimbikitsidwa kuyambira ali ndi zaka 10 mpaka colectomy . Pa AFAP, kuyezetsa kuyenera kuyamba chaka chilichonse, kuyambira ali ndi zaka 20.

Mukamaliza opaleshoni ya colectomy, muyenera kupitiriza kuchitidwa mayeso a sigmoidoscopy, omwe amafufuza gawo la pansi la GI tract yanu. Ngati muli ndi gawo la rectum yanu yotsala, muyenera kuliyang'ana miyezi 6 mpaka 12 iliyonse. Ngati rectum yanu yachotsedwa ndikusinthidwa ndi thumba la ileal, muyenera kuliyang'ana chaka chimodzi mpaka zinayi chilichonse.

Mayeso ena omwe akukonzekera angaphatikizepo:

• Endoscopy Yapamwamba: Izi zikufanana ndi colonoscopy, koma zimachitika kumtunda kwa dongosolo lanu la m'mimba. Endoscope imaperekedwa pakhosi panu ndikulowa m'mimba mwanu ndi kumtunda kwa matumbo anu ang'onoang'ono (duodenum) kuti muwone ngati pali ma polyps. Ngati alipo, dokotala wanu akhoza kuwachotsa nthawi yomweyo ndi njira yochitira izi.
• Mayeso a Ultrasound a khansa ya chithokomiro kapena chiwindi.
• Kuyesa kwa CT (computed tomography scan) kapena MRI (magnetic resonance imaging) kuti mudziwe zotupa za desmoid.

Kodi FAP ingapewedwe?

Uphungu wa majini kuti mumvetse chiopsezo chopatsira ana anu matenda a FAPZingathandize. Kukambirana za zoopsazi kungakuthandizeni kukonzekera banja lanu. Nthawi zambiri, sizingatheke kuletsa kusintha kwa majini kuchitika panthawi yoyembekezera, koma pali njira zosiyanasiyana zolerera zomwe mungaganizire.

Kodi munthu amene ali ndi FAP amakhala ndi moyo wautali bwanji?

Ngati sanalandire chithandizo, anthu ambiri amakhala ndi moyo zaka pafupifupi 42. Komabe, ndi chithandizo choyenera chamankhwala komanso chithandizo, mutha kukhala ndi moyo wabwinobwino . Mukachotsa m'matumbo mwanu, chiopsezo chanu chachikulu chimachokera ku khansa zina zam'mimba komanso zotupa zomwe zimakhala zovuta kwambiri. Izi sizimafala kwambiri kuposa khansa ya m'matumbo.

Ngati ndili ndi vutoli, ndiyenera kuyembekezera chiyani?

Ngati mwapezeka ndi FAP, mutha kuyembekezera kuyesedwa kwa moyo wanu wonse komanso mwina opaleshoni zingapo kuti muchotse zotupazo . Zotupa zomwe zimakula kunja kwa m'matumbo mwanu sizingakhale khansa ngati ma polyps m'matumbo mwanu. Ngakhale kuti zotupa za desmoid si khansa, nthawi zina zimatha kukhala zosokoneza pang'ono kapena zosokoneza kwambiri.

Mungathe kuyembekezera kuchitidwa opaleshoni ya colectomy yonse musanayambe moyo wanu . Pambuyo pake, matumbo anu akhoza kulumikizidwanso, kapena mungakhale ndi thumba la ileal kapena ostomy. Njira iliyonseyi ili ndi chiopsezo cha zovuta zinazake. Komabe, mutha kukhalabe ndi moyo wautali komanso wathanzi mutatha opaleshoni ya colectomy.

Ndizachibadwa kumva chisoni komanso kuda nkhawa mukazindikira kuti muli ndi matenda a majini omwe amafunika chisamaliro chamankhwala kwa moyo wanu wonse. Komabe, mukadziwa, mutha kuchitapo kanthu kuti muchepetse chiopsezo chanu cha khansa . Ngakhale kuti opaleshoni ndi yamtsogolo, madokotala adzayesa kuchita bwino momwe angathere.

Anthu ambiri amatha kuchitidwa opaleshoni ya laparoscopic kuti achotse m'mimba, zomwe zimachitika kudzera m'mabala ang'onoang'ono ndikuchira mwachangu . Anthu omwe ali ndi thumba la ilial angafunike kuchitidwa opaleshoni zingapo, koma pamapeto pake adzatha kugwiritsa ntchito chimbudzi monga kale.

Zinthu zofunika kwambiri zomwe tiyenera kukumbukira kuchokera pa zomwe takambirana (Uthenga Wopita Nawo Kunyumba)

Chabwino, kuchokera pa zomwe takambirana za `FAP`, nazi zinthu zofunika kwambiri zomwe muyenera kukumbukira:

• FAP ndi matenda a majini omwe amatha kufalikira m'mibadwo yambiri.
• Izi zimapangitsa kuti ma polyps mazana kapena zikwizikwi apangidwe m'matumbo, omwe amatha kukhala khansa.
• Ngati sichinalandire chithandizo, chiopsezo chotenga khansa ya m'matumbo chimakhala chachikulu kwambiri .
• Ndikofunikira kwambiri kuyamba kuchita mayeso a colonoscopy kuyambira ali aang'ono (zaka 10-12) .
• Chithandizo chachikulu ndi opaleshoni yochotsa m'matumbo (colectomy) .
• Ngakhale m'matumbo atachotsedwa, kuyezetsa kwa moyo wonse kumafunika kuti muwone ngati pali zotupa zomwe zikukula kwina m'thupi.
• Ngakhale kuti vutoli ndi vuto la moyo wonse lomwe liyenera kuthandizidwa, ndi chithandizo chamankhwala choyenera komanso kuyezetsa, n'zotheka kukhala ndi moyo wabwinobwino komanso wathanzi .

Ngati inu kapena wina m'banja mwanu ali ndi zizindikiro izi, kapena ngati mukufuna kudziwa zambiri za izi, funsani upangiri wa dokotala . Kudziwa msanga ndi kuchitapo kanthu msanga ndiye zinthu zofunika kwambiri.

FAP , Familyal Adenomatous Polyposis, Matenda a Majini, Colps za m'matumbo, Khansa ya m'matumbo, APC Gene, Colonoscopy, Colectomy, Opaleshoni