Kumvetsa Retinoblastoma

Zizindikiro, Kuzindikira, Kuchiza, ndi Kugonjetsa

Retinoblastoma ndi khansa ya diso ndipo imapanga pafupifupi 3 mpaka 4 peresenti ya khansa yaunyamata. Amapezeka kawirikawiri m'mabwana ndi makanda, ndipo ngakhale zizindikirozo zinali zoperewera m'mbuyomu, ana ambiri tsopano amadwala matendawa.

Retinoblastoma Mwachidule

Retinoblastoma ndi khansa yosawerengeka ya ana yomwe imapezeka pafupifupi mmodzi mwa ana 20,000.

Amayamba kuyang'anitsitsa mitsempha kumbuyo kwa diso (retina) ndipo 80 peresenti ya matenda amapezeka kwa ana osakwana zaka zitatu. Khansa imapezeka kawirikawiri kwa ana osapitirira zaka zisanu.

Ngakhale kuti retinoblastoma imakhala yosauka kwambiri, ana 9 pa 10 alionse tsopano amakhala ndi moyo, ndipo ambiri omwe akuwonawo amasungiranso. Izi zikuti, chithandizo chikhoza kukhala cholimba kwa makolo omwe akukumana ndi vutoli mwa ana awo, ndipo thandizo likufunika kwambiri.

Tiyeni tione zizindikiro zomwe zimapezeka komanso momwe retinoblastoma imathandizira. Popeza kuti nthawi zina zimakhala zobadwa, tidzanenanso za chibadwa cha retinoblastoma ndi zomwe muyenera kudziwa ngati mwana wanu m'banja adapezeka.

Jekeseni la Diso ndi Retina

Kumvetsetsa zizindikiro za retinoblastoma n'kosavuta kumvetsa ngati mukuganiza kuti maso amatha bwanji.

Retina imakhala ndi mkati mwa diso ndipo imapangidwa ndi maselo a mitsempha (photoreceptors) omwe amatchedwa ndodo ndi cones.

Retina ndi yopapuka kwambiri, pafupifupi 1 / 5m millimeter wandiweyani, ndi kuzungulira kukula kwa kotala.

Mukamawona chithunzi, fanolo limayendetsedwa kudzera mwa wophunzira wanu ndipo limayang'ana pa retina. Maselo a mitsemphawa amatumiza chizindikiro cha magetsi cha fanolo kumbali ya ubongo yomwe imachita masomphenya (occipital lobes).

Zizindikiro za Retinoblastoma

Retinoblastoma kawirikawiri imapezeka pamene mwana akuyendera mwana wanu, kapena ngakhale chimodzi cha zizindikiro zowunikira chikuwonetsedwa pa chithunzi. Zizindikiro zingakhalepo:

• White pupillary reflex (leukocoria): Pamene dokotala wa ana amawala ndi kuyang'anitsitsa m'maso mwa mwanayo amawona chinachake chotchedwa "red reflex." Retina imanyamula mitsempha ya magazi yomwe imapangitsa kuti dera liwonedwe mopitirira wopenya akuwoneka wofiira. Pamene retinoblastoma ilipo, dera ili likhoza kuwoneka loyera m'malo mwake. Nthaŵi zina vutoli limakayikira ngati chithunzi cha maso a mwana chikuwonekera bwino.
• Diso laulesi (maso): Maso a mwana angawoneke akuwoneka mosiyana kapena amaoneka ndi maso. Kumbukirani kuti kuyang'ana maso kwa ana akhanda kungakhale koyenera kufikira zaka zitatu kapena 4. Ana ena omwe ali ndi retinoblastoma amawoneka ngati akuwombera kwambiri.
• Kuwala kosavuta: Katswiri wanu wa ana adzawunika kuwala kwa ophunzira a mwana wanu kuti awone ngati akugwira ntchito. Ngati retinoblastoma ilipo, kuganizira uku sikungakhale kopanda diso limodzi.
• Kuwopsya kwa diso, kufiira, kupweteka, kapena kutupa
• Glaucoma (kuwonjezeka kwa diso): Glaucoma ikhoza kuchitika pamene maselo otupa amaletsa njira zowonongeka za diso.

Kuzindikira kwa retinoblastoma

Kafukufuku wa retinoblastoma kawirikawiri amayamba kukayikira pogwiritsa ntchito white pupillary reflex panthawi yoyezetsa mwana. (Kwa ana amene amanyamula kusintha, onani pansipa). Monga tanenera kale, nthawi zina makolo amakayikira kuti pali chinachake cholakwika akawona woyera mwana wa reflex pa chithunzi cha mwanayo. Pali ngakhale pulogalamu yamapulogalamu yamakono yomwe ilipangidwe kuti iwonetsere izi.

Kafukufuku woganizira amachitika nthawi yotsatira. An ultrasound kapena OCT (optical coherence tomography) kawirikawiri ndi mayeso oyambirira ndipo angapereke chidziwitso chofunikira pa chifuwa chachikulu. Phunziroli limathandizanso mwanayo kuti asamawonongeke.

Kawirikawiri, MRI imalimbikitsidwanso, kuti ipitirize kufufuza derali, ndipo ndiyeso yabwino kwambiri poyang'ana zovuta zosaoneka ngati retinoblastoma. Nthaŵi zina CT imakhala yofunika ngati mawerengedwe amawoneka mosavuta pa CT, koma imapereka mwana pachiswe chowopsa, motero nthawi zambiri sizomwe zimayesedwa.

Mosiyana ndi khansa zambiri, chidziwitso cha chotupacho sichinthu chofunika kwambiri pamene zofufuza zokha zimapangitsa kuti munthu azindikire (pali zochepa zofanana zomwe zimawonekera). Kuopsa kwa chiwopsezo kungaphatikizepo kuwonongeka kwa diso ndi mitsempha ya optic komanso mwayi woti maselo a khansa akhoza kufalikira, zomwe zimachititsa kufalikira kwa khansa.

Ngati pali vuto kuti kansa yafalikira kupyola diso, mayesero omwe amayenera kuyang'ana matenda a metastatic akhoza kuchitidwa. Izi zingaphatikizepo kutayika kwa khungu (kuyang'ana maselo a khansa pamtsempha), fupa la fupa (kufufuza umboni wa maselo a khansa mu fupa la mafupa), kapena fupa la fupa (kuyang'ana mitsempha ya fupa).

Ana ena amakhalanso ndi zilonda zapineal (retinablastoma yamtunduwu), kotero kuti kufufuza kuti azindikire mbali iyi ya ubongo (monga MRI) ikhoza kukhala yofunikira.

Kusiyanasiyana Kusiyana-Kodi N'kotheka Bwanji?

Pali zinthu zochepa kwambiri zomwe zimafanana ndi retinoblastoma, zomwe zimapangitsa kuti zikhale zovuta kupanga matendawa pogwiritsa ntchito kafukufuku ndi zojambula zokha. Zochitika zina zomwe zingawonekere zofanana ndi izi:

• Matenda a Coates: Matenda a Coates ndi matenda ochepa omwe amapezeka chifukwa cha mitsempha yambiri yomwe imapezeka m'mbuyo mwa retina. Zida zosadziwika zimayambitsa magazi kumbuyo kwa diso. Kuphulika kwa magazi kungayambitse minofu yambiri kuti imangire (yomwe ili ndi maonekedwe achikasu oyera) omwe angawonongeke ndi retinoblastoma.
• Toxocara Canis: Toxocara Canis ndi njoka zam'mimba zomwe nthawi zina zimafalitsidwa kwa agalu odwala. Tizilombo toyambitsa matenda timakula kumbuyo kwa diso (komanso malo ena) ndipo zingabweretse kuchitetezo cha retina .
• Kuchokera m'mimba mwa chizoloŵezi choyambitsa matenda: Kutaya kachilombo koyambitsa matendawa kumayenderana ndi kuwonjezeka kwa mitsempha ya magazi m'maso ndipo nthawi zambiri imapezeka ana omwe amatha masabata 31 asanakwane.
• Kupitirizabe kusakaniza mavitreous (PHPV): PHPV ndi vuto losabereka kwa ana omwe amayamba chifukwa chokhala ndi mitsempha yopanda magazi m'maso.
• Intraocular epithelioma: Intraocular medulloepithelioma ndi chotupa chochepa chimene chimayambira mu thupi la thupi m'malo mwa retina.

Zifukwa ndi Zoopsa

Sitikudziwa chomwe chimayambitsa kusintha kwazomwe zimayambitsa chitukuko cha retinoblastoma. Ndi khansa zambiri, zakudya, masewera olimbitsa thupi, komanso malo owonetsera zachilengedwe amathandiza, koma popeza retinoblastoma imayamba nthawi yayitali, kapena ngakhale asanabadwe, izi zimakhala zochepa kwambiri. Tikudziwa kuti majini amathandiza kwambiri m'mimbayi.

Genetics ya Retinoblastoma

Retinoblastoma ikhoza kuchitika chifukwa cha jini mutation mu jini yotchedwa RB1 yomwe imapezeka pa chromosome ya 13. Jini imeneyi ndi chotupa choyambitsa matenda , chomwe chimayambitsa mapuloteni omwe amachepetsa kukula kwa maselo. Izi zimapangidwa mwachikhalidwe chodziwika kwambiri ndipo jini losadziwika likhoza kulandira kuchokera kwa amayi kapena abambo. Palinso kusintha kwina, monga ku MYCN komwe kumayenderana ndi matendawa.

Ndi kusintha kwa RB1 kuganiza kuti pafupifupi 25 peresenti ndi kusintha kwa majeremusi omwe amachokera kwa kholo limodzi ndipo 75% amapeza (kusintha komwe kumachitika panthawi ya kukula kwa mwana). Kwa ana omwe ali ndi kusintha kwa RB1 (mwina anabadwa kuchokera kwa kholo kapena pamene kusinthika kumachitika msinkhu msinkhu) mwayi wa kukhala ndi retinoblastoma (penetrance) ndi 90 peresenti.

Matenda a retinablastoma amadziwika bwino nthawi zambiri ndipo amapezeka msinkhu kusiyana ndi retinoblastoma ya sporadic. Retinablastoma yowonjezera ikhoza kukhala multifocal, ndi zotupa zingapo zikukula panthawi yomweyo. Ana omwe ali ndi retinoblastoma omwe ali ndi cholowa chawo ali pangozi yowonjezera khansa zina m'tsogolomu.

Kukhazikitsa Retinoblastoma

Zigawo za retinoblastoma zikhoza kugawidwa mu magawo 1 mpaka 4 monga khansa zina, koma machitidwe osiyana amagwiritsidwa ntchito nthawi zambiri. (Gawo I limatchula khansa imene diso lingasungidwe, ndipo gawo 2 mpaka IV ndi khansa imene diso limachotsedwa). Popeza nthendayi imagwidwa kale m'mayiko otukuka, machitidwe ena a ma stage amagwiritsidwa ntchito nthawi zambiri.

Pali magawo akulu awiri a matendawa:

• Matenda a m'matumbo omwe ali mkati mwa diso
• Zozizwitsa zazing'onoting'ono zomwe zimapitirira kupitirira diso. Izi zimaphatikizidwanso kuti zikhale zowonongeka zapadera zomwe zimafalikira kudera lonse la diso (mphambano) ndi ziphuphu zowonjezera m'mimba zomwe zafalikira ku ubongo, mafupa, mafupa, kapena madera ena.

Ku United States ndi m'mayiko ena otukuka, retinoblastoma kawirikawiri imapezeka mu intraocular stage.

Ziphuphu za m'mimba zimapangidwanso m'magulu otsatirawa:

• A-Zimaphatikizapo zotupa zomwe ziri zosakwana 3 mm (millimeters) m'mimba mwake ndipo siziphatikizapo optic disc (pakati pa masomphenya)
• B-Zoposa 3 mm m'mimba mwake
• C-Vuto laling'ono komanso lodziwika bwino lomwe lafala pansi pa retina kapena mu vitreous humor
• D-Chotupa chachikulu kapena chosadziwika chomwe chafalikira pansi pa retina kapena mu vitreous humor
• Ziphuphu zazikuluzikulu zomwe zafalikira kutsogolo kwa diso, zimakhala zovuta ndi glaucoma, kapena maselo a retina. Ndi zotupa izi zingakhale zovuta kupulumutsa diso.

Pamene retinoblastoma imafalikira, ikhoza kuwononga malo otsala a diso (globe). Malo otchuka kwambiri a metastase akutali ndi amphithi, ubongo ndi msana, chiwindi, fupa la mafupa, ndi mafupa.

Retinoblastoma Chithandizo Chasankho

Mankhwala abwino a retinoblastoma amadalira pa siteji ya khansa, malo a chotupacho, kaya chotupacho sichingagwirizanenso kapena padera, ndi zina. Kwa zotupa zazing'ono, mankhwala a mankhwala ndi mankhwala omwe amagwiritsidwa ntchito nthawi zambiri amagwiritsidwa ntchito.

Zolinga za mankhwala ndi izi:

• Kusunga moyo wa mwanayo
• Kusunga diso (kapena maso) ngati n'kotheka
• Kusunga masomphenya ochuluka momwe zingathere
• Kuchepetsa zotsatira za nthawi yaitali zokhudzana ndi chithandizo

Njira zothandizira ndizo:

• Chemotherapy: Chemotherapy ingagwiritsidwe ntchito yokha kapena kuchepetsa kukula kwa chotupacho asanayambe opaleshoni. Mitundu yosiyanasiyana ya mankhwala otchedwa chemotherapy ingagwiritsidwe ntchito ndi mankhwala otchedwa intra-arteriolar chemotherapy omwe amagwiritsidwa ntchito kwambiri. Mwa njirayi, mankhwala amadzimadzi amatumizidwa mwachindunji ndi mitsempha yomwe imapereka maso, kuchepetsa zotsatira za mankhwala pa zida zina. Intravenous chemotherapy ingagwiritsidwe ntchito. Kwa zotupa zazikulu kapena zowonongeka kapena zowonongeka, mankhwala apamwamba a mankhwala akuluakulu ndi kupulumutsidwa kwa maselo osungunuka angagwiritsidwe ntchito.
• Zochitika zapakhomo: Pali mitundu yambiri ya mankhwala omwe angagwiritsidwe ntchito pochizira retinoblastoma. Izi zimabweretsa kuchotsedwa kwa maselo a khansa koma ndi zochepa kwambiri kuposa opaleshoni kuchotsa diso. Izi zimaphatikizapo cryotherapy (kutentha kwa mpweya) kutentha kwapadera (kutenthetsa chotupa), opaleshoni ya laser (photocoagulation), ndi ena.
• Mankhwala othandizira ma ARV: Mankhwalawa akhoza kugwiritsidwa ntchito kunja (kunja kwa mankhwalawa), kapena mkati (brachytherapy) imene imayikidwa mkati mwa thupi pafupi ndi chotupacho. Thandizo la pulotoni likuyendetsedwa ngati njira ina yothandizira mazira omwe amatha kupangitsa khansa yachiwiri.
• Kuchita opaleshoni (maukhutu): Pakuti zimbudzi zochepa, chemotherapy ndi njira zam'deralo zimagwiritsidwa ntchito poyembekeza kusunga diso. Kwa zotupa zazikulu, kapena zomwe zikupita kapena kubwerera, kuchotsedwa kwa diso ndi gawo la mitsempha ya optic (enucleation) ingafunike. Kuika maso nthawi zambiri kumaikidwa panthaŵi ya opaleshoni, ndi diso lopangidwira loikidwa pamwamba pa kuikidwa pamapeto pake.
• Zochitika zachipatala: Pali njira zambiri zochiritsira zomwe zimafufuzidwa m'mayesero a kachipatala, kuyambira nanoparticulate chemotherapy kupita ku mitundu yatsopano ya mankhwala.

Tsatirani Patatha Chithandizo

Matenda ambiri a retinoblastomas amachiritsidwa ndi chithandizo, koma kutsata ndikofunika kwambiri. Ziphuphuzi nthawi zina zimatha kubwereranso, ndipo mapulogalamu a MRIs amatha kugwiritsidwa ntchito nthawi zambiri. Kupenda ndikutsata masomphenya, ngati kusungidwa, kumafuna chidwi chenicheni. Kuonjezera apo, kuchedwa kwachitukuko sizodziwika. Kuwongolera n'kofunikanso kwambiri ndi chidwi chapadera chomwe chimaperekedwa kwa zotsatira za mankhwala. Gulu la Ana la Oncology (COG) la Survivor Guidelines limafotokoza zambiri mwazinthu izi.

Zotsatira Zotsalira ndi Khansa Yachiwiri

Zotsatira za kansalu ya khansa zimatchula zinthu zomwe zimayambitsidwa ndi khansa zomwe zingapangitse zaka makumi angapo chitatha chithandizo. Tikuphunzira kuti opulumuka khansara a ana ali ndi chiopsezo chachikulu cha matenda omwe amachokera ku matenda a mtima (nthawi zambiri okhudzana ndi mankhwala a chemotherapy), operewera, kwa khansa yachiwiri.

Pofufuza zotsatira za zotsatira za nthawi yaitali za ana omwe ali ndi retinoblastoma, zinapezeka kuti zaka makumi angapo pambuyo pake (zaka zapakati pazaka makumi awiri ndi ziwiri), ana 87 peresenti ya ana amene anapulumuka retinoblastoma anali ndi matenda amodzi okhudzana ndi chithandizo. Ngakhale izi zingakhale zokhumudwitsa kuti zikambirane, mfundoyi ikuwunikira kufunika kwa opulumuka a khansa ya ana kuti akhale ndi nthawi yayitali ndi dokotala wodziwa bwino kupeza ndi kulandira zotsatira za mankhwala a nthawi yaitali.

Ana amene apulumuka retinoblastoma ali ndi chiopsezo chowonjezereka chokhala ndi khansa yachiwiri ( khansa zatsopano) pamsewu. Chifukwa cha retinoblastoma yachilendo, mbali imodzi ya kuwonjezeka kumeneku ndi chifukwa cha kusokonekera kwa chotupa choyambitsa matenda m'zinthu zina. Ndi retinoblastoma yomwe siidalandira, khansa yachiwiri iyi ikhoza kuchitika monga mankhwala a chemotherapy kapena mankhwala a radiation. Ma khansa achiwiri amodzi ndi osteosarcoma (khansara ya fupa), minofu yofewa, khansa ya khansa , khansa ya m'mapapo , ndi lymphoma.

Kuwonjezera pa khansa yachiŵiri, zotsatira za nthawi yaitali za chemotherapy ndi zotsatira za nthawi yaitali za mankhwalawa zimakhala zofunika kwambiri chifukwa ana ambiri amakhala ndi zotsatirazi kapena amapezeka pangozi kwa nthawi yaitali.

Kuthamangitsani

Ana ambiri omwe amapezeka kuti ali ndi retinoblastoma tsopano adzachiritsidwa. Kuchuluka kwa zaka 5 zomwe zimachitika ku retinoblastoma ku United States kunali 97.3 peresenti poyang'ana nyengo ya pakati pa 2000 ndi 2012, ndipo mankhwala akupitirirabe. Ngakhale ana omwe ali ndi metastase kupita kumadera kunja kwa ubongo (monga mafupa) amachiritsidwa nthawi zambiri. Zomwe zinati, kufalikira mkati mwa ubongo (intracranial matenda) zimakhudzidwa ndi zotsatira zopanda pake.

Zina zowonongeka zazomwe zikuphatikizapo kupanga khansara m'dziko lotukuka (komwe kuli khansa yokhudzana ndi HPV). Katatu retinoblastoma amachititsa anthu ambiri kufa kuchokera ku retinoblastoma m'mayiko otukuka.

Kuzindikira Kwambiri kwa Ana Amene Ali ndi Kusintha kwa Mzere Wambiri

Pakalipano tilibe njira iliyonse yothetsera chitukuko cha retinoblastoma, koma ana amene ali pangozi yaikulu chifukwa cha kusintha kwa majeremusi kapena mbiri ya banja ya matendawa angathe kufufuzidwa ndi kuyembekezera kugwidwa ndi vutoli. zotheka. Sewero la Funduscopic (kuyang'anitsitsa maso pa retina) pansi pa anesthesia mwamsanga pakatha kubereka ndi mwezi uliwonse kwa chaka choyamba cha moyo nthawi zambiri imalimbikitsidwa. Geni amatha kupezeka mu amniotic fluid, ndipo ana okhudzidwa akhoza kuyang'anitsanso ndikuperekedwa mofulumira ngati zizindikiro za khansara zatchulidwa.

Ngati mwana akukula retinoblastoma, ana ena ndi mamembala angayang'anenso kuti aone ngati ali ndi jini lokayikira.

Kulimbana ndi Kuthandizidwa

Monga kholo, chidziwitso ndi mphamvu, ndipo ndizothandiza kuphunzira zonse zomwe mungathe.

Thandizo ndilofunika kwambiri. Chifukwa cha vutoli, anthu ambiri alibe magulu othandizira a retinoblastoma, koma pali anthu ambiri othandizira pa intaneti komanso kudzera pa Facebook. Kuphatikizidwa m'maderawa sikungopereka chithandizo pamene mukulimbana ndi kukhala ndi kholo la mwana yemwe ali ndi khansara, koma akhoza kukhala malo abwino kwambiri kuti aphunzire za kafukufuku wam'tsogolo wokhudza retinoblastoma. Palibe amene alimbikitsidwa kwambiri kuphunzira ndi kufotokozera zatsopano zomwe akupeza kuposa makolo omwe akulimbana ndi matendawa kwa mwana wawo.

Pamene ana akukula tsopano ali ndi mwayi wothandizira ana. Pali magulu othandizira odwala matenda a khansa omwe amapangidwa makamaka kwa achinyamata omwe ali ndi khansa, ndipo makampu ndi kubwezeretsa amapezeka.

Mawu Ochokera

Retinoblastoma ndi chotupa chosowa chophatikizapo mitsempha ya mitsempha mu retina. Zimapezeka kawirikawiri m'mabuku aang'ono kwambiri. Popeza ndizosowa, ndizofunika kuti ana awonekere kuchipatala chachikulu cha khansa kapena chipatala cha ana, omwe ali ndi akatswiri omwe amadziŵa bwino chithandizo chabwino cha matendawa. Zonsezi zimakhala zabwino kwambiri, koma kupita kumeneko kungakhale kovuta kwa amayi, ana, komanso ndalama. Ngati mwana wanu watengedwa, funsani thandizo limene likupezeka.

> Zotsatira:

> Fernandes, A., Pollock, B., ndi F. Rabito. Retinoblastoma ku United States: Kuwonjezeka Kwazaka 40 ndi Kukhazikika kwa Moyo. Journal of Opthalmology ndi Strabismus . 2017. (Epub patsogolo pa kusindikiza).

> Friedman, D., Chou, J., Oeffinger, K. et al. Zinthu Zochiritsira Zosalekeza Kwa Anthu Okalamba Achikulire a Retinoblastoma: Zotsatira za Retinoblastoma Survivor Study. Khansa . 2016. 122 (5): 773-81.

> National Cancer Institute. Matenda a Retinoblastoma Treatment (PDQ) -Achilengedwe Professional Version. Kusinthidwa 09/19/17. https://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq

> Parma, D., Ferrer, M., Luce, L., Giliberto, F., ndi I. Szijan. RB1 Kusinthika kwa Chibadwa M'thupi la Retinoblastoma ya Argentina. Zotsatira za uphungu wa Genetic. PLoS One . 2017. 12 (12): e0189736.

> National Library of Medicine. Zolemba za Kunyumba za Genetics. Retinoblastoma. 12/17/17. https://ghr.nlm.nih.gov/condition/retinoblastoma#sourcesforpage