Nevoid basal cell carcinoma syndrome

Nevoid basal cell carcinoma syndrome ndi gulu la zofooka zomwe zimadutsa m'mabanja. Vutoli limakhudza khungu, dongosolo lamanjenje, maso, mafupa a endocrine, kwamikodzo ndi ziwalo zoberekera, ndi mafupa.

Zimayambitsa mawonekedwe achilendo komanso chiopsezo chachikulu cha khansa yapakhungu ndi zotupa zopanda khansa.

Nevoid basal cell carcinoma nevus syndrome ndi matenda osowa kwambiri. Jini yayikulu yolumikizidwa ndi matendawa imadziwika kuti PTCH ("yamawangamawanga"). Jini yachiwiri, yotchedwa SUFU, yakhala ikugwirizananso ndi vutoli.

Zovuta zamtunduwu zimafalikira kudzera m'mabanja ngati chikhalidwe chodziyimira payokha. Izi zikutanthauza kuti mumakhala ndi matendawa ngati kholo lililonse likupatsirani jini. Ndikothekanso kukulitsa vuto la jini ili wopanda mbiri yabanja.

Zizindikiro zazikulu za matendawa ndi:

• Mtundu wa khansa yapakhungu yotchedwa basal cell carcinoma yomwe imayamba nthawi yakutha msinkhu
• Chotupa chosagwira khansa cha nsagwada, chotchedwa kerotocystic odontogenic chotupa chomwe chimayambanso munthu akamatha msinkhu

Zizindikiro zina ndizo:

• Mphuno yayikulu
• Chatsitsa m'kamwa
• Lododometsa, pankhope panja
• Nsagwada zomwe zimatuluka (nthawi zina)
• Maso otakata
• Kukhazikika pamikhatho ndi zidendene

Vutoli limatha kukhudza dongosolo lamanjenje ndikupangitsa kuti:

• Mavuto amaso
• Kugontha
• Kulemala kwamaluso
• Kugwidwa
• Zotupa zaubongo

Vutoli limayambitsanso kufooka kwa mafupa, kuphatikiza:

• Kupindika kumbuyo (scoliosis)
• Kupindika kwakukulu kumbuyo (kyphosis)
• Nthiti zachilendo

Pakhoza kukhala mbiri yabanja yamatendawa komanso mbiri yakale ya khansa yapakhungu yapakhungu.

Mayeso atha kuwulula:

• Zotupa zamaubongo
• Mphutsi nsagwada, zomwe zingayambitse kukula kwa mano kapena nsagwada
• Zofooka za mtundu wachikuda (iris) kapena mandala a diso
• Kutupa kwa mutu chifukwa chamadzi muubongo (hydrocephalus)
• Zovuta za nthiti

Mayeso omwe angachitike ndi awa:

• Echocardiogram yamtima
• Kuyezetsa magazi (mwa odwala ena)
• MRI yaubongo
• Khungu limatulutsa zotupa
• X-ray ya mafupa, mano, ndi chigaza
• Ultrasound kuti muwone zotupa zamchiberekero

Ndikofunika kukayezetsa ndi dokotala wa khungu (dermatologist) pafupipafupi, kuti khansa yapakhungu ichiritsidwe ikadali yaying'ono.

Anthu omwe ali ndi vutoli amathanso kuwonedwa ndikuchiritsidwa ndi akatswiri ena, kutengera gawo lakuthupi lomwe lakhudzidwa. Mwachitsanzo, katswiri wa khansa (oncologist) amatha kuchiza zotupa m'thupi, ndipo sing'anga wa mafupa angathandize kuthana ndi mavuto a mafupa.

Kutsata pafupipafupi ndi madotolo osiyanasiyana ndikofunikira kuti pakhale zotsatira zabwino.

Anthu omwe ali ndi vutoli atha kukhala:

• Khungu
• Chotupa chaubongo
• Kugontha
• Mipata
• Zotupa zamchiberekero
• Matenda a mtima
• Kuwonongeka kwa khungu komanso mabala chifukwa cha khansa yapakhungu

Lumikizanani ndi omwe amakuthandizani kuti mupite kukakumana ndi:

• Inu kapena abale anu muli ndi matenda a basal cell carcinoma, makamaka ngati mukufuna kukhala ndi mwana.
• Muli ndi mwana yemwe ali ndi zizindikilo za matendawa.

Mabanja omwe ali ndi mbiri yakubanja ya matendawa amatha kulangiza za majini asanatenge mimba.

Kukhala kunja kwa dzuwa ndikugwiritsa ntchito zoteteza ku dzuwa kungathandize kupewa khansa yatsopano ya khungu.

Pewani ma radiation monga x-ray. Anthu omwe ali ndi vutoli amakhudzidwa kwambiri ndi ma radiation. Kuwonetsedwa ndi radiation kumatha kubweretsa khansa yapakhungu.

Matenda a NBCC; Matenda a Gorlin; Matenda a Gorlin-Goltz; Matenda a basal cell nevus (BCNS); Khansara ya basal cell - nevoid basal cell carcinoma syndrome

• Matenda a basal cell nevus - kutseka kwa kanjedza
• Basal cell nevus syndrome - maenje obzala
• Matenda a basal cell nevus - nkhope ndi dzanja
• Matenda a basal cell nevus
• Matenda a basal cell nevus - nkhope

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Skelsey MK, Peck GL. Nevoid basal cell carcinoma syndrome. Mu: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson IH, olemba. Kuchiza kwa Matenda a Khungu: Njira Zambiri Zakuchiritsira. 5th ed. Philadelphia, PA: Elsevier; 2018: mutu 170.

Walsh MF, Cadoo K, Salo-Mullen EE, Dubard-Gault M, Stadler ZK, Offit K. Zomwe zimayambitsa matendawa: matenda obadwa nawo a khansa. Mu: Niederhuber JE, Armitage JO, Kastan MB, Doroshow JH, Tepper JE, olemba. Chipatala cha Abeloff's Oncology. Lachisanu ndi chimodzi. Philadelphia, PA: Elsevier; 2020: mutu 13.