Kodi mwana wanu ali ndi madontho ang'onoang'ono akuda ozungulira milomo yake, mkamwa mwake, kapena m'manja mwake? Mungaganize kuti ndi madontho chabe. Koma mungadabwe kudziwa kuti madonthowa akhoza kukhala okhudzana ndi mtundu wa chotupa chomwe chimayamba m'thupi, makamaka m'matumbo. Lero tikambirana za vuto losowa koma lofunika kudziwa. Izi zimatchedwa Peutz-Jeghers Syndrome, kapena mwachidule PJS.
Mwachidule, kodi Peutz-Jeghers Syndrome (PJS) ndi chiyani?
Matenda a Peutz-Jeghers (PJS) ndi matenda a majini omwe amachititsa kuti tinthu tating'onoting'ono (polyps) tipangidwe m'thupi mwathu ndipo mawanga akuda amaonekera pakhungu lathu komanso m'kamwa mwathu. Chabwino kwambiri n'chakuti mawanga ndi mawanga amenewa si khansa (osaopsa) . Koma vuto ndilakuti anthu omwe ali ndi matenda a PJS ali pachiwopsezo chachikulu chotenga khansa mtsogolo kuposa ena.
Madontho akuda awa (omwe amatchedwa `mucocutaneous hyperpigmentation`) nthawi zambiri amawoneka ali ana. Koma pang'onopang'ono amachepa tikamakula. Mtundu wa chotupa chomwe ndatchulachi (`hamartomatous polyps`) nthawi zambiri umayamba m'mimba mwathu (`gastrointestinal tract`). Izi zikutanthauza kuti, m'malo monga matumbo ang'onoang'ono, m'mimba, ndi m'matumbo akulu . Koma nthawi zina, amathanso kukula m'malo monga impso, chikhodzodzo, mapapo, ndi mphuno. Zotupa izi zimatha kuyambitsa mavuto osiyanasiyana, ndipo nthawi zina zimatha kusanduka khansa.
Ngati muli ndi matenda a PJS, dokotala wanu nthawi zonse amafufuza khansa ndi zotupa zomwe zili pachiwopsezo chachikulu.
Kodi matendawa ndi ofala bwanji?
Imeneyi ndi vuto losowa kwambiri. Ngakhale kuti sizikudziwika bwino kuti ndi anthu angati omwe ali ndi vutoli, ofufuza akuti limakhudza munthu mmodzi pa anthu 300,000 ndi mmodzi pa anthu 25,000 .
Kodi zizindikiro za PJS ndi ziti?
PJS imayambitsa makamaka mawanga akuda (paubwana) ndi ma polyps (mwa ana ndi akulu omwe). Tiyeni tiwone zizindikiro izi padera.
| Mtundu wa chizindikiro | Zinthu zoti muwone |
|---|---|
| Madontho Amdima | Ana omwe ali ndi PJS akhoza kukhala ndi madontho abuluu-imvi kapena abulauni. Anthu ena amaganiza kuti awa ndi madontho ang'onoang'ono chabe. Nthawi zambiri amawoneka ali ndi zaka 1-2 ndipo amatha kutha akafika zaka 18-19. Ndi ang'onoang'ono, kukula kwake ndi pafupifupi milimita 1 mpaka 5. Madontho amenewa amapezeka kwambiri mu:
|
| Zizindikiro za polyps | Zizindikiro za zotupa za m'mimba nthawi zambiri zimawonekera pakati pa zaka 10 ndi 30. Zikuphatikizapo:
|
N’chifukwa chiyani PJS imeneyi imayamba? Kodi chifukwa chake n’chiyani?
Anthu ambiri omwe ali ndi PJS ali ndi kusintha kwa majini otchedwa STK11. STK11 ndi jini yoletsa khansa. Mwachidule, ntchito ya jini iyi ndikuwongolera kukula kosalamulirika kwa maselo m'thupi lathu.
Ganizirani za jini iyi ngati chosinthira magetsi. Imazimitsa "kuwala" komwe kumalamulira kukula kwa maselo. Pamene jini iyi sikugwira ntchito bwino, zimakhala ngati chosinthiracho chasweka ndipo kuwala kumakhala koyatsidwa nthawi zonse. Popeza palibe woletsa maselo kukula, amapitiriza kugawikana ndikukula pamodzi, ndikupanga zotupa.
Pafupifupi 80% ya anthu omwe ali ndi PJS amalandira kusintha kwa majini kuchokera kwa amayi awo kapena abambo awo. 20% yotsalayo mwina alibe mbiri ya banja la matendawa, koma akhoza kukhala ndi kusintha kwa majini okha. Anthu ena amadwala PJS popanda kusintha kulikonse mu jini ya STK11. Asayansi sakudziwabe chifukwa chake.
Kodi matendawa amabadwa bwanji?
Kawirikawiri, mwana amalandira jini yosinthidwa iyi kuchokera kwa kholo limodzi. Imalandira mu "autosomal dominant" pattern. Izi zikutanthauza kuti kaya mayi kapena bambo alandira jini yosinthidwa iyi ya ``STK11``, mwanayo ali pachiwopsezo chachikulu chokhala ndi zizindikiro ndi zovuta za PJS. Ngati muli ndi kusintha kwa jini kumeneku, mwana wanu ali ndi mwayi wa 50% wolandira.
Kodi mavuto omwe angakhalepo chifukwa cha PJS ndi ati?
Vuto lalikulu kwambiri ndi khansa .Ofufuza amati munthu amene ali ndi PJS ali pachiwopsezo cha khansa mpaka 93% . Ichi ndichifukwa chake madokotala nthawi zonse amafufuza khansa ndikuyesera kuizindikira msanga.
Mavuto ena ndi awa:
- Kulowa m'mimba: Izi zimachitika pamene chipolopolo chachikulu m'matumbo ang'onoang'ono chikudutsa ndikupangitsa kuti gawo lina la m'matumbo lipinde mkati. Matendawa amatha kukhudza anthu 69% omwe ali ndi PJS.
- Kutsekeka kwa matumbo ang'onoang'ono: Zotupa zimatha kutsekeka kwa matumbo ang'onoang'ono. Pafupifupi 50% ya anthu omwe ali ndi PJS amakhala ndi kutsekeka kwakukulu kwa matumbo komwe kumafunika opaleshoni asanakwanitse zaka 20.
- Kutuluka magazi m'mimba: Zotupa zimatha kukakamiza minofu ya m'mimba ndikuyambitsa kutuluka magazi.
- Kuchepa kwa magazi m'thupi chifukwa cha kusowa kwa ayoni: Kutuluka magazi nthawi zonse kumayambitsa kuchepa kwa ayoni m'thupi, zomwe zimapangitsa kuti pakhale kuchepa kwa magazi m'thupi.
Mavuto enieni kwa akazi ndi amuna
- Akazi: Zotupa za m'chiberekero (zotupa za m'chiberekero) ndi khansa yowopsa komanso yosowa yotchedwa adenoma malignanum, yomwe imayamba m'chiberekero. Izi zingayambitse kusakhazikika kwa msambo kapena kutha msinkhu msanga.
- Amuna: Akhoza kukhala ndi zotupa m'machende (zotupa za maselo a Sertoli). Izi zingayambitse kukula kwa mawere (gynecomastia), kutha msinkhu msanga, ndipo mafupa awo amatha kukula mofulumira kuposa masiku onse, zomwe pamapeto pake zimapangitsa kuti akhale ndi msinkhu waufupi.
PJS ndi chiopsezo cha khansa
PJS imawonjezera kwambiri chiopsezo chotenga khansa ya m'mimba ndi ziwalo zina m'thupi. Avereji ya zaka zomwe wodwala wa PJS amapezeka ndi khansa ndi pafupifupi zaka 42.
| Mtundu wa khansa | Chiŵerengero cha odwala a PJS |
|---|---|
| Khansa ya m'matumbo | Kufikira 40% |
| Khansa ya m'mawere | 30% - 50% |
| Khansa ya kapamba | 11% - 36% |
| Khansa ya m'mimba | Kufikira 30% |
| Khansa ya m'chiberekero | 20% |
| Khansa ya m'mapapo | 15% |
| Khansa ya m'matumbo ang'onoang'ono | Kufikira 13% |
Kodi matenda a PJS amachiritsidwa bwanji?
Anthu ambiri amapezeka ndi matenda a PJS akapita kwa dokotala chifukwa cha zizindikiro monga kutsekeka kwa matumbo kapena kutsekeka kwa m'mimba. Avereji ya zaka zomwe munthu amadwala matendawa ndi pafupifupi zaka 23. Kuti adziwe matendawa, madokotala amafufuza zotsatirazi:
- Kodi pali aliyense m'banjamo amene ali ndi matenda a PJS?
- Madontho akuda pakhungu.
- Kaya pali ma polyps m'mimba.
Kuphatikiza apo, mayeso a majini angachitike kuti muwone ngati muli ndi kusintha kwa majini kwa `STK11`.
Mayeso odziwira matendawa
Dokotala wanu angakulangizeni mayeso osiyanasiyana kuti muwone ngati muli ndi zotupa m'matumbo anu. Ena mwa iwo ndi awa:
- Colonoscopy: Kufufuza matumbo akuluakulu pogwiritsa ntchito chubu chokhala ndi kamera.
- Endoscopy yapamwamba: Kufufuza m'mero, m'mimba, ndi m'mimba mwa matumbo ang'onoang'ono.
- Kapisozi endoscopy: Kumeza kapisozi yokhala ndi kamera kakang'ono mkati. Kamera iyi imajambula zithunzi pamene ikuyenda m'mimba.
Mukhozanso kutenga magazi kuti muwone ngati muli ndi vuto la kuchepa kwa magazi chifukwa cha kusowa kwa ayoni.
Kodi mankhwala a PJS ndi ati?
PJS singathe kuchiritsidwa kwathunthu, kotero cholinga chachikulu cha chithandizo ndikuwunika chiopsezo cha khansa ndikupewa mavuto omwe amabwera chifukwa cha chotupacho.
Madokotala amatha kuchotsa zotupa m'matumbo akulu panthawi ya colonoscopy. Amathanso kuchotsa zotupa m'mimba ndi kumtunda kwa matumbo ang'onoang'ono ngati pakufunika kutero. Nthawi zina, njira zapadera monga balloon-assisted enteroscopy zimagwiritsidwa ntchito kuchotsa zotupa zomwe zili m'matumbo ang'onoang'ono.
Nthawi zina, opaleshoni ingafunike kuchotsa chotupacho.
Kodi ndi mayeso otani omwe ndiyenera kuchita pafupipafupi?
Ngati muli ndi matenda a PJS, muyenera kuyesedwa nthawi zonse m'moyo wanu wonse kuti mupeze khansa ndi mavuto ena msanga. Izi zingawoneke ngati zokhumudwitsa pang'ono, koma ndikofunikira kuti muteteze moyo wanu.
| Mtundu wa mayeso | Nthawi yoti tichite zimenezo |
|---|---|
| Mayeso ofala kwa onse | |
| Kufufuza matumbo ang'onoang'ono (CT/MRI enterography kapena video capsule endoscopy) | Kuyambira ali ndi zaka 8-10, ndipo zaka 2-3 zilizonse ngati pali zotupa. |
| Endoscopy yapamwamba | Kuyambira ali ndi zaka 12, pachaka ngati pali zotupa, kapena zaka 2-3 zilizonse. |
| Kufufuza kwa Pancreatic (MRCP kapena Endoscopic ultrasound) | Kuyambira ali ndi zaka 25-30, kamodzi pa zaka 1-2 zilizonse. |
| Mayeso apadera a akazi | |
| Kuyezetsa bere | Kuyambira ali ndi zaka 25, pitani kwa dokotala kawiri pachaka ndipo chaka chilichonse muzichita mammogram ndi MRI ya m'mawere. |
| Kuyezetsa matenda a akazi | Kuyezetsa m'chiuno ndi kupaka Pap smear chaka chilichonse kuyambira azaka zapakati pa 18-20. |
| Mayeso enieni a amuna | |
| Kuyezetsa ma testicular | Kuyezetsa kwa dokotala pachaka kuyambira ali ndi zaka 10. |
Kodi matendawa angapewedwe?
Popeza PJS nthawi zambiri imakhala yobadwa nayo, palibe chomwe tingachite kuti isakule.
Koma ngati muli ndi matenda a PJS, chinthu chofunika kwambiri chomwe mungachite ndi kudziwitsa banja lanu za matendawa ndikuwatumiza kuti akalandire uphungu wa majini . Kenako akhoza kuyesedwa ngati ali ndi kusintha kwa majini ndikupeza upangiri wofunikira kuti apewe khansa.
Ngati muli ndi PJS, mwana wanu ali ndi mwayi wa 50% woti adwale matendawa. Koma tsopano pali njira zochepetsera chiopsezo chimenecho. Mwachitsanzo, ngati mukubereka mwana kudzera mu IVF, njira yotchedwa preimplantation genetic diagnosis (PGD) ingagwiritsidwe ntchito pofufuza kusintha kwa majini mu mluza. Izi ndi njira zovuta komanso zodula, choncho ndikofunikira kulankhula ndi dokotala wanu za izi.
Uthenga Wopita Nawo Kunyumba
- Matenda a Peutz-Jeghers (PJS) ndi matenda obadwa nawo makamaka m'majini.
- Izi zimapangitsa kuti mawanga akuda apangidwe pakhungu ali aang'ono, makamaka kuzungulira milomo ndi mkati mwa pakamwa.
- Ma polyps (zomera zopanda khansa) zimapangika m'mimba, zomwe zingayambitse mavuto monga kupweteka m'mimba, kutuluka magazi, komanso kutsekeka kwa matumbo.
- Munthu amene ali ndi matenda a PJS amakhala pachiwopsezo chachikulu chotenga khansa pa moyo wake wonse.
- Ngakhale kuti matendawa sangachiritsidwe, poyesedwa nthawi zonse, mavuto ndi khansa zimatha kuzindikirika msanga ndipo moyo ungapulumutsidwe. Ndikofunikira kwambiri kulankhulana ndi dokotala wanu.
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