Pane mumwe munhu mumhuri yako, kunyanya achiri mudiki, akabatwa nekenza yemudumbu here? Kana kuti chiremba akakuudza here kuti une ma polyps akawanda mudumbu rako? Pamwe izvi zvinokonzerwa nechirwere chemajini chinowanikwa mumhuri. Nhasi tichataura nezvechimwe chirwere chisingawanzoitiki asi chakakosha zvikuru. IFAP, kana kuti Familyal Adenomatous Polyposis. Kunyangwe zita racho rakaoma zvishoma, ngatirinzwisisei zviri nyore.
Zvichitaurwa zviri nyore, chii chinonzi FAP?
FAP chirwere chemajini chinogona kupfuudzwa kubva kuchizvarwa kuenda kune chimwe. Vanhu vane chirwere ichi vanokura huwandu hwakawanda hwemabundu anonzi adenomas , ayo anogona kukura kuita kenza, muura hwavo nemurectum.
Iye zvino ungave uchifunga kuti, "Zvakajairika here kuva necyst muura hwako?" Ehe, zvakajairika kuti vamwe vanhu vagadzire cyst imwe kana mbiri idzi pavanenge vachikura. Asi munhu ane FAP anogona kukura mazana, pamwe kunyange zviuru, zvecysts idzi. Uye zvinotanga pazera diki kwazvo, pamwe pazera remakore gumi nemashanu kana gumi nematanhatu.
Mapolyps aya haasi ekenza pakutanga. Asi anonzi 'precancerous'. Izvi zvinoreva kuti kana akasarapwa, pane njodzi huru yekuti rimwe kana akawanda emapolyps aya anozopedzisira ava kenza yemudumbu .
Fungidzira kuti zvaizova zvakaoma sei kubvisa mazana emabundu aya rimwe nerimwe. Hazvigoneki. Ndosaka, kuti vagadzirise njodzi iyi yakakura, vanachiremba vanokurudzira kubviswa ura hwese neoparesheni, kubviswa kwecolectomy yese . Dzimwe nguva, rectum inobviswawo (proctocolectomy).
Pasina kuvhiyiwa uku, vanhu vazhinji vane FAP vangangobatwa nekenza pazera repakati nepakati. Uyewo, vanhu vane FAP vari panjodzi yekuva nemamota kwete muura chete asiwo mune dzimwe nhengo dzemuviri (semuenzaniso, mudumbu, muura hudiki, ganda, nemapfupa). Saka, kunyangwe mushure mekunge ura hwabviswa, vachada kuongororwa nachiremba nguva nenguva muhupenyu hwavo hwese.
Ndeipi njodzi yekubatwa nekenza neFAP?
Kana ikasarapwa, FAP ine mukana wekuti iite kenza yemuura mukuru ne100% . Ichi chirwere chakakomba zvikuru. Vanhu vane FAP vanobata kenza vachiri vadiki uye nekukurumidza kupfuura munhuwo zvake. Saka, kana uchiziva kuti kune nhoroondo yemhuri yeFAP, mwana wako anofanira kuongororwa colonoscopy gore rega rega kutanga pazera remakore gumi.
Pamusoro pekenza yemuura, vanhu vane FAP vari panjodzi yekubatwa nemamwe marudzi ekenza.
| Rudzi rwegomarara | Njodzi yekuitika ( netsaona ) |
|---|---|
| Kenza yeDuodenal | ~8% |
| Kenza yethyroid yePapillary | ~2% |
| Kenza yepancreatic | ~2% |
| Kenza yechiropa (Hepatoblastoma) - kunyanya muvana | ~1.5% |
| Kenza yemudumbu | ~1% |
| Matumbu europi nemusana | Zvisingasviki 1% |
Kune mhando dzakasiyana dzeFAP here?
Ehe, kune mhando huru yeFAP nedzimwe mhando dzinoverengeka dzakasiyana zvishoma uye dzisina kuoma zvakanyanya. Ngationei kuti ndedzipi.
| Rudzi rweFAP | Tsananguro |
|---|---|
| Classic FAP (Classic FAP) | Rudzi urwu ndirwo rwakajairika. Mapolyps anopfuura zana, pamwe zviuru, anokura muura. |
| Kuderedza FAP (AFAP) | Izvi hazvina kunyanya kuipa. Huwandu hwema cysts anokura muura huri pakati pe20 ne100. |
| Gardner syndrome | Izvi zvakafanana neClassic FAP, zvichireva kuti bundu rinokura rinodarika 100. Pamusoro pezvo, mamwe marudzi emabundu anokurawo mune dzimwe nhengo dzemuviri, semuenzaniso, muganda, munyama dzakapfava, uye mumapfupa. |
| Turcot syndrome | Izvi zvinokonzera kuti bundu rakawanda riumbwe muura, pamwe nebundu rekenza riumbwe muuropi. |
Chii chakasiyana pakati peFAP neLynch Syndrome?
Lynch syndrome chimwe chirwere chemajini chinogona kupfuudzwa nezvizvarwa uye chinowedzera njodzi yekubatwa nekenza yemuura. Asi pane musiyano mukuru pakati pezviviri izvi. Vanhu vane Lynch syndrome havabereki mazana emapolyps, sezvavanoita muFAP. Dzimwe nguva, polyps imwe kana mbiri dzinokura, uye dzinogona kukurumidza kuva kenza. Ndosaka Lynch syndrome dzimwe nguva ichinzi "nonpolyposis". Mamiriro ese ari maviri anokonzerwa nekukanganisa kwemajini akasiyana.
Ndezvipi zviratidzo zveFAP?
MuFAP, bundu remuura rinotanga kukura nekukurumidza kupfuura muvanhu vose, kazhinji vachiri vadiki. Bundu iri hariwanzo kukonzera zviratidzo kusvika rakura zvine njodzi. Ndosaka kuongororwa kwakakosha.
Zvisinei, nekuda kwekuti huwandu hwema cysts hwakakura uye hunokura nekukurumidza, dzimwe nguva zviratidzo zvinogona kuoneka. Zviratidzo zvakadaro zvinosanganisira:
- Kubuda ropa mutsvina
- Manyoka anogara achiitika
- Marwadzo emudumbu asingaperi
Pamusoro peizvi, vanhu vane FAP vanogonawo kuva nezviratidzo zvekunze izvo chiremba anogona kuona zviri nyore.
- Dermatofibromas: Mapundu akaomarara, akaita sevanga anoumbwa pasi peganda.
- Mapundu eganda: Mapundu akatenderera azere nekeratin anoumbwa pasi peganda.
- MaOsteomas:Mabundu asiri ekenza anoumba mumapfupa. Anowanzoonekwa mudehenya kana muchiuno.
- Mazino ekuwedzera kana mazino akakuvara: Izvi zvinogona kuonekwa neX-ray yemazino.
- Mavara eretinal ane pigmented (CHRPE): Aya anogona kuonekwa panguva yekuongororwa kwemaziso. Zvisinei, kazhinji haakanganisi kuona.
Chii chinonyanya kukonzera FAP?
Sezvatakambotaura, FAP inokonzerwa nekuchinja kwemajini. Jini rinokanganisa izvi rinonzi APC (Adenomatous Polyposis Coli) .
Zvese zviri mumuviri wedu zvinodzorwa nemajini, sepurogiramu yekombuta. Jini iri reAPC ijini rinodzvinyirira bundu. Kureva kuti, basa guru rejini iri nderekumisa masero kuti asaparadzane zvisingadzoreki ogadzira bundu. Kungofanana nemabhureki emotokari.
Munhu ane FAP ane jini reAPC rakachinja, zvichireva kuti harina kunaka. Zvadaro, semota ine mabhureki akatyoka, masero anopatsanuka zvisingadzoreki oumba mazana emabundu.
Kukanganisika kwemajini uku kunogarwa nhaka. Kana mumwe wevabereki aine APC gene mutation iyi, mwana ane mukana we50% wekuigarwa nhaka . Izvi zvinoreva kuti mwana wega wega anogona kuikura kana kusaikura. Zvisinei, vanhu vanosvika 30% vanoonekwa vaine FAP havana nhoroondo yemhuri. Izvi zvinoreva kuti kukanganisika kwemajini uku kwakaitika mumuviri wavo munguva pfupi yapfuura (original mutation).
Ndezvipi zvinetso zvinogona kuitika nekuda kweFAP?
Dambudziko guru uye rine njodzi rekugadzirisa neFAP igomarara remuura . Njodzi iyi inodzikira zvikuru kana ura hukabviswa. Zvisinei, pane mamwe matambudziko ekurarama usina ura. Maitiro ekufamba kwemuura anochinjwa. Pakuita izvi, chikwama che ileostomy kana chikwama che ileal chinogona kushandiswa.
Nekuti dambudziko remajini eAPC riripo musero rega rega mumuviri, bundu rinogonawo kuumbwa kunze kweura. Mamwe acho ane njodzi yekuva kenza.
- Duodenal polyps: Idzi dzinokura muvanhu vese vane FAP. Nhamba shoma yeidzi dzinogona kukura kuita kenza ye duodenum, bile duct, kana pancreatic duct.
- Mapundu emudumbu: Kunyangwe achiitika muvanhu vanosvika 90%, chikamu chidiki chete, chinosvika 1%, chinokura kuita kenza.
- Matumbu eDesmoid: Aya haasi kenza. Asi anogona kuva nehasha uye anogona kupararira kunhengo dzemuviri dziri pedyo netsinga dzeropa, zvichikuvadza. Anonetsa kubvisa uye anogona kudzoka.
- Gomarara rethyroid: Izvi zvinogona kuitika muvanhu vanosvika 2% vane FAP. Izvi zvinowanzo rapa zvachose.
- Kenza yechiropa: hepatoblastoma, kunyanya muvana vane FAPPane mukana mudiki wekuva nekenza yechiropa isingawanzoitiki inonzi.
Inorapwa uye inodzorwa sei?
Chirongwa chekurapa FAP chinogona kukamurwa kuita zvikamu zviviri: kuvhiyiwa uye kuongororwa kwehupenyu hwese.
1. Kuvhiyiwa
Vanhu vazhinji vane FAP yekare vachafanirwa kuvhiyiwa colectomy yakazara, iyo inosanganisira kubviswa kweura hwese, pavanenge vava nemakore ekuma20 kana kutanga kwe30. Chiremba wako ndiye achasarudza kuti ovhiyiwa iyi inoitwa riini uye sei, zvichienderana nemamiriro ako ezvinhu.
2. Kuongororwa nguva dzose
Uchafanirwa kuongororwa zvakasiyana-siyana usati wavhiyiwa uye mushure mekuvhiyiwa, uye muhupenyu hwako hwese. Iyi ndiyo nzira yakanakisisa yekuchengetedza hupenyu hwako.
| Rudzi rwekuyedza | Chinangwa | Nguva inowanzo kurudzirwa |
|---|---|---|
| Colonoscopy | Kutarisa bundu riri muura nemurectum. | Kune avo vari panjodzi, gore rega rega kubva pazera remakore gumi kusvika pakuvhiyiwa. |
| Sigmoidoscopy | Kuongorora bhegi rasara remudumbu kana remudumbu mushure mekuvhiyiwa. | Mushure mekuvhiyiwa, mwedzi 6-12 yega yega kana gore 1-4 rega rega. |
| Endoscopy yepamusoro | Kutarisa bundu riri mudumbu nemuura hudiki (mudumbu). | Panguva dzakatarwa nachiremba. |
| Kuskena kweUltrasound | Kune kenza yethyroid kana chiropa. | Pamusoro pezano rechiremba. |
| CT kana MRI scan | Kutarisa bundu re desmoid. | Pamusoro pezano rechiremba. |
Hupenyu huri sei neFAP?
Zvakajairika kunzwa kusuruvara uye kushamisika paunoziva kuti une chirwere chemajini chakadai. Zvisinei, kuziva nezvechirwere ichi nekukurumidza ndiyo nzira yakanakisisa yekudzivirira njodzi yako yekenza.
Kana munhu ane FAP akaongororwa zvakanaka uye akaongororwa nguva nenguva, anogona kurarama hupenyu hwakanaka, hwakareba, uye hune hutano . Mushure mekubviswa kweura, njodzi huru inobva kune dzimwe kenza dzemudumbu kana bundu re desmoid. Asi idzi hadziwanzowaniki kupfuura kenza yemudumbu.
Kunyangwe kuvhiyiwa kuri shanduko huru muhupenyu, nehunyanzvi hwemazuva ano, kuvhiyiwa kwe laparoscopic kunogona kukubatsira kuti upore nekukurumidza.
Chinhu chinonyanya kukosha kuziva kuti hausi wega. Kune vanachiremba, mhuri, neshamwari vanogona kukutungamira nekukutsigira parwendo urwu. Saka, kana uine mibvunzo kana zvinokunetsa, taura nachiremba wako nezvazvo pachena.
Mharidzo Yekuenda Nayo Kumba
- FAP chirwere chakakomba chemajini chinopfuudzwa kubva kuzvizvarwa kusvika kuzvizvarwa uye chinokonzera mazana emamota muura.
- Kana ikasarapwa, njodzi yekubatwa nekenza yemuura inenge iri pedyo ne100%.
- Kana mumwe munhu mumhuri yako akambova nekenza yemuura kana mapundu akawanda achiri mudiki, taura nachiremba wako nezvekuongororwa kwemajini.
- Kuonekwa nekukurumidza uye kuongororwa nguva nenguva kwakakosha kuponesa hupenyu. Vana vari panjodzi vanofanira kutanga kuongororwa pazera remakore gumi.
- Kurapa kukuru kwekudzivirira njodzi yekenza kubviswa kweura hukuru (colectomy).
- Kana ukarapwa zvakanaka uye uchitarisirwa, unogona kurarama hupenyu hwakazara uye hune hutano kunyangwe uine FAP.
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