Iimpawu, ukuxilongwa, unyango, kunye nokuhlaziya
I-Retinoblastoma ngumhlaza welihlo kwaye yenza malunga ne-3 ukuya kwi-4 ekhulwini le-cancer yobudala. Itholakala ngokuqhelekileyo kubantwana abancinci kunye neentsana, kwaye nangona i-prognosis yayingamahlwempu kwixesha elidlulileyo, uninzi lwabantwana abasinda esi sifo.
I-Retinoblastoma Overview
I-Retinoblastoma ngumhlaza ongabonakali wobuntwaba owenzeka kwi-20,000 yabantwana.
Iqala ekukhanyeni kweethambo ezinomso emva kweso (i-retina) kunye nama-80 ekhulwini amatyala avela kubantwana abangaphantsi kweminyaka engama-3. Umhlaza awufumaneki kangangoko kubantwana abangaphezu kweminyaka emihlanu.
Ngelixa i-retinoblastoma yayisisifo esihle, i-9 kwabantwana abayi-9 abasaphila ngoku, kunye nabaninzi abanokubona. Oko kwathiwa, unyango lunokuba nzima kubazali abajongene nale meko kubantwana babo, kwaye inkxaso iyadingeka kakhulu.
Masiqwalasele iimpawu eziqhelekileyo kunye nendlela i-retinoblastoma ephathwa ngayo. Ekubeni le meko ngezinye iindawo ifa, siya kuthetha kwakhona nge-genetics ye-retinoblastoma kunye noko kufuneka ukuba wazi ukuba umntwana usetshenziswe.
I-Anatomy ye-Eye kunye ne-Retina
Ukuqonda iimpawu ze-retinoblastoma kulula ukuyiqonda ukuba ucinga nge-anatomy yelihlo.
I- retina iquka uhla lwangaphakathi lweliso kwaye lenziwe ngamaseli e-nerve (ukubona i-photoreceptors) ebizwa ngokuba yi-rod and cones.
I-retina inqabile kakhulu, malunga ne-1/5 ye-millimeter ubukhulu, kunye nobukhulu bekota.
Xa ugcina umfanekiso, umfanekiso uqondiswa ngumfundi wakho kwaye ugxile kwi-retina. Ezi iisensi ze-nererve zithumela umqondiso wombane waloo mfanekiso kwinxalenye yengqondo ebonisa imbono (i-occipital lobes).
Iimpawu zeRetoblastoma
I-retinoblastoma idla ngokuxilongwa ngexesha lokutyelela umntwana ngokusemntwaneni wakho, nokuba emva kweminye impawu yokuxilonga ibonwa kwiifoto. Iimpawu zingabandakanya:
- I-white pupillary reflex (leukocoria): Xa udokotela wezingane ekhanyisa ukukhanya kwaye ekhangele emehlweni omntwana wakho ubona into ebizwa ngokuthi "i-reflex ebomvu." I-retina ilayishwe imithana yegazi eyenza indawo ibone ngaphaya komfundi ebonakala ebomvu. Xa i-retinoblastoma ikhona, lo mmandla ungabonakala mhlophe kunoko. Ngamanye amaxesha imeko ikhunjulwa ukuba ifoto yamehlo yomntwana ibonisa i-reflex emhlophe.
- Iliso elinesilumko (i-strabismus): Amehlo omntwana angabonakala ekhangeleka kwiindlela ezahlukeneyo okanye abe nombono ongeniso. Gcina ukhumbule ukuba ukubukeka kwamehlo ezinsana kuzalwa kuya kuba yinto eqhelekileyo kude kube neenyanga ezi-3 okanye ezine. Abanye abantwana abane-retinoblastoma nabo babonakala bexhala kakhulu.
- I-reflex yokukhanya engabikhoyo: Ootitshala bezonyango bakho baya kubakhanyisa abafundi bakho abantwana ukuba babone ukuba ngaba bayavuma. Ukuba i-retinoblastoma ikhoyo, le ngcamango ingabikho kwiso linye.
- Ukukhupha kweso, ubomvu, intlungu, okanye ukuvuvukala
- I-Glaucoma (uxinzelelo olwandisiweyo kwiso): I- Glaucoma iyakwenzeka xa iisilini zee-tumor zivimba iindlela eziqhelekileyo zokuhambisa amanzi.
Ukuxilongwa kweRetoblastoma
Ukuxilongwa kwe-retinoblastoma ngokuqhelekileyo kukhankanywa ngokusekelwe kumgca we-pupillary reflex ngexesha lovavanyo lomntwana. (Kwiintsana ezithatha utshintsho, jonga ngezantsi). Njengoko kuphawuliwe ngasentla, ngabazali maxa wambi bacinga ukuba into ephosakeleyo xa bebona umfundi omhlophe u-reflex kwisithombe somntwana. Kukho ne-app ye-smartphone ekhoyo eyenzelwe ukukhenkca kule ngxubusho.
Uphando lwezifundo zidlalwa ngokuzayo. I-ultrasound okanye i-OCT (i-optical coherence tomography) ngokuqhelekileyo ibonisa uvavanyo lokuqala kwaye inokunika ulwazi olubalulekileyo ngobukhulu besisu. Olu pho nonongo lubeka umntwana umngcipheko we-radiation.
I-MRI idla ngokucetyiswa ngokunjalo, ukuba ihlolisise loo ndawo, kwaye luvavanyo olunzulu lokujonga izinto ezingafaniyo ezifana ne-retinoblastoma. Ngamanye amaxesha i-CT iyadingeka njengoko kubalwa kulula ukufumana kwi-CT, kodwa ibonisa umntwana kumngcipheko we-radiation, ngoko ngokuqhelekileyo ayiyilo vavanyo lokuqala.
Ngokungafani namaninzi omdlavuza, i-biopsy ye-tumor ayidingekanga njengoko ukufunyaniswa kweemviwo kuphela kwisoloko kubangela ukuxilongwa (kukho imimiselo embalwa kakhulu ebonakalayo). Umngcipheko we-biopsy unokuquka umonakalo kwiso kunye ne-optic nerve kunye nethuba lokuba amangqamuzana omhlaza angahlwayelwa, okubangele ukusasazeka komhlaza.
Ukuba kukho inkxalabo yokuba umhlaza usasazeke ngaphaya kweso, izilingo ezenzelwe ukujonga izifo zesimetast zingenziwa. Ezi zingabandakanya ukugqitywa kwe-lumbar (ukukhangela amangqamuzana omhlaza emlanjeni), ukufundwa komongo we-bone (ukukhangela ubungqina bentsholongwane yesifo somnxeba), okanye ukukhangela amathambo (ukukhangela amathambo e-boneastases).
Abanye abantwana banama-tumor gland (i-retinoblastoma ye-trilateral), ngoko ukuphonononga ukuhlola le ngxenye yengqondo (njenge-MRI) ibalulekile.
Ukuxilongwa Ngokwahlukileyo-Yintoni Enokuthi Yayibe Ngayo?
Kukho ngokwenene imimiselo embalwa efana ne-retinoblastoma, okwenza kube lula ukwenza ukuxilongwa ngokusekelwe ekuhloleni nasekufundiseni iziqu kuphela. Ezinye iimeko ezinokubonakala zifana ziquka:
- Isifo se-Coates: I-Coates isifo esingabonakaliyo sokuzalwa (kodwa esingenalo ifa) esichazwe yimilambo yegazi engavamile emva kwe-retina. Iinqanawa ezingaqhelekanga zibangela ukuba igazi lingene emlenzeni. Ukuvuza kwegazi kunokubangela izicubu ezinamafutha ekwakheni (okubonakalayo okwesibhakabhaka esimhlophe) okungenzeka iphosakele nge-retinoblastoma.
- I-Toxocara Canis: I-Toxocara Canis injengeenkumbi eziyinkuku ezithe zithunyelwa kubantu kwizinja ezikhulelwe. I-parasite ikhula ikhula kwisehlo (kunye nezinye iindawo) kwaye inokukhokelela kwintsimbi yokubuyisela .
- I-Retinopathy yokuqala kwangaphambili: I- Retinopathy ye-prematurity yimeko ehambelana nokugqithiswa kwemithambo yegazi kwiso kwaye ifunyanwa rhoqo kubantwana abazalwa ngaphambi kweeveki ezingama-31.
- Ukuqhubeka kwe-vitraous (PHPV) eyona nto ihamba phambili: I-PHPV ingxaki engabonakaliyo yintsholongwane ebangelwa yimithambo yegazi engabonakaliyo kwiso.
- Intraocular epithelioma: Intraocular medulloepithelioma yintlungu engavumelekanga yokuzalwa yintsholongwane eqala kwi-body ciliary kunokuba i-retina.
Izizathu kunye neengozi
Akuqinisekanga oko kubangela ukuba utshintsho olujongene nophuhliso lwe-retinoblastoma. Uninzi lweengqungquthela, ukutya, ukuzivocavoca, kunye nokukhuselwa kwendalo kukudlala indima, kodwa ekubeni i-retinoblastoma idla ngokukhawuleza emva koko, okanye nangaphambi kokuzalwa, ezi zinto zinokudlala indima encinci. Siyazi ukuba i-genetics idlala indima kwipesenti enkulu yalezi zicubu.
Genetics of Retinoblastoma
I-retinoblastoma ingenzeka ngenxa yokuguqulwa kwemfuza kwi-gene eyaziwa njenge-RB1 efunyenwe kwi-chromosome yesi-13. Lo mfuzo uyisifo soxinzelelo lwe - tumor , esineenkcukacha zeeprotheni ezinciphisa ukukhula kweeseli. Leli lifa kwi-autosomal fowuni kunye nomgubo ongaqhelekanga unokufumana ilifa kumama okanye utata. Kukho nezinye iinguqu, ezifana nezo ze-MYCN ezidibene nesi sifo.
Ngenguqu ye-RB1 kucatshulwa ukuba malunga neepesenti ezingama-25 zinguqu zintsholongwane (ezivela kumzali omnye) kunye nezinye iipesenti ezingama-75 ezifumanekayo (utshintsho olwenzeka ngexesha lokukhula komntwana). Kubantwana abatshintshileyo kwi-RB1 (okanye ifa elivela kumzali okanye xa ukuguqulwa kwentshukumo kwenzeka ekuqaleni kwentsholongwane) ithuba lokuphuhlisa i-retinoblastoma (ukungena kwi-90%).
I-retinoblastoma ye-Hereditary ihlala iphakathi kunye kwaye ivela kwincinci kune-retinoblastoma ye-sporadic. I-retinoblastoma ye-Hereditary inokuthi ibe ne-multitifocal, kunye neengxaki ezininzi eziphuhlisayo ngexesha elifanayo. Abantwana abanokufumana i-retinoblastoma yelifa nabo basengozini yokuphuhlisa ezinye i-Cancer esikhathini esizayo.
I-retinoblastoma yesistim
Izigaba ze-retinoblastoma zingabelana ngezigaba I ukuya ku-IV njengamanye amagciwane, kodwa iinkqubo ezahlukileyo zikwasetyenziswa rhoqo. (I-Stage I ibhekisela kumagciwane apho iliso linokugcinwa khona, kwaye isigaba II ukuya kwe-IV yimigulane apho iliso lisuswa khona). Ekubeni isifo sihlala sibanjwe ekuqaleni kumazwe athuthukileyo, ezinye iisistim zokuhlala zihlala zisetyenziswa.
Kukho izigaba ezibini zesifo:
- I-Intraocular-Tumors ephakathi kweso
- I-Extraocular-Izibilini ezigqitha ngaphaya kweso. Ezi zigqityiwe kwakhona zibe ziimvumba zangasese zomzimba apho zisasazeka kulo lonke ummandla welihlo (i-orbit) kunye nezidumbu ze-extraocular metastatic ezazisasazeka kwingqondo, emathambo, emathambo, nakweminye imimandla.
EUnited States nakwamanye amazwe athuthukileyo, i-retinoblastoma idla ngokufumaneka kwi-intraocular stage.
Izicubu ze-intraocular zikwahlulwa kwakhona kwizigaba ezilandelayo:
- A-Kuquka izicubu ezingaphantsi kwama-3 mm (millimeters) ububanzi kwaye azibandakanyi i-disc optic (phakathi kwembono)
- B-Mkhulu kunama-3 mm ububanzi
- C-naluphi na ucuba oluncinane oluchazwe phantsi kwe-retina okanye kwi-vitreous humor
- D-Nayiphi na isisu esininzi okanye esichazekileyo esasasazeka phantsi kwe-retina okanye kwi-vitreous humor
- Amathumbu amakhulu amakhulu asasaze phambili kwiso, anzima ngama-glaucoma, okanye i-retinal detachment. Ngala ma-tumors kunokuba nzima ukugcina iso.
Xa i-retinoblastoma isasazeka, inokungena kwiindawo ezisele zaso (ihlabathi). Iindawo eziqhelekileyo zeemastastases ezikude ziquka i-lymph nodes, ingqondo kunye nomgudu womgudu, isibindi, umongo wenthambo kunye namathambo.
Izinketho zokwelapha iRetoblastoma
Iipilisi ezifanelekileyo ze-retinoblastoma zixhomekeke kwisigaba somdlavuza, indawo yesikhumba, nokuba i-tumor ayihambelani kunye okanye iyenye, kunye nezinye izinto. Kuba iimvumba ezincinci, i-chemotherapy kunye nezonyango zijoliswe rhoqo.
Iinjongo zonyango ziquka:
- Ukulondoloza ubomi bomntwana
- Ukugcina iso (okanye amehlo) ukuba kunokwenzeka
- Ukugcina umboniso omkhulu ngangokunokwenzeka
- Ukunciphisa igalelo elide elide elichaphazelekayo kunyango
Izinyango zokwelapha ziquka:
- I-Chemotherapy: I-Chemotherapy ingasetyenziselwa yedwa okanye iyancipha ubukhulu be-tumor phambi kokuhlinzwa. Iindidi ezahlukeneyo ze-chemotherapy zingasetyenziswa nge-intra-arteriolar chemotherapy esetyenziswa rhoqo. Kule ndlela, isichemotherapy isicrotherapy isithunyelwa ngqo kwi-artery eyenzela iso, ukunciphisa umphumo weziyobisi kwezinye iifom. I-chemotherapy engathabathiyo ingasetyenziswa kwakhona. Kuba iimvumi ezinkulu okanye eziye zaphindaphindiweyo okanye eziphindaphindiweyo, i-chemotherapy ephezulu ye-dose ne- stem cell cell ingasetyenziswa.
- Ionyango zengingqi: Kukho iintlobo ezahlukeneyo zonyango lwengingqi ezingasetyenziswa ekuphatheni i-retinoblastoma. Oku kubangele ukususwa kweeseli zomhlaza kodwa kungenasiphelo esincinci ngaphezu kokuhlinzwa ukuze kususwe iliso. Ezi ziquka i-cryotherapy (ipholisa i-tumor) i-thermography (ukutshisa i-tumor), ukuhlinzwa kwelaser (photocoagulation), nabanye.
- Uphulo lwe-radiation: Unyango lwamayeza lungasetyenziselwa ngaphandle (unyango lwe-radiation lwangaphandle), okanye ngaphakathi (ibrachytherapy) apho i-radiation ifakwa ngaphakathi emzimbeni eduze kwe-tumor. Ulwaphulo lwe-proton lwentambo luvandlakanywa njengenye indlela yokwelashwa kwemithi yendabuko njengoko kungenokuba neyona nto inokubangela ukuba i-cancer yesibini ibone.
- Ukuhlinzwa (inucleation): Kuba iimvumba ezincinci, i-chemotherapy kunye neenkqubo zendawo zenziwa ngokuthemba ukugcina iso. Kwiimvumi ezinkulu, okanye ezo ziqhubela phambili okanye zibuyele, ukususwa kweso kunye nenye ye-nertic optic (inucleation) ingadingeka. Ukufakelwa kwamehlo kudlalwa kufakwe ngexesha lokuhlinzwa, kwesojoni elenziwe phezulu phezu kokufakela emva komhla.
- Uvavanyo lwezonyango: Kukho iindlela ezahlukeneyo zonyango eziphandwa kwizilingo zonyango, ukusuka kwi-nanoparticulate chemotherapy ukuya kwiindlela ezintsha zonyango lwendawo.
Ukulandelwa Emva kokunyanga
Uninzi lwe-retinoblastomas lunokunyangwa ngonyango, kodwa ukulandeleka kubaluleke kakhulu. Ezi zihlamba ngamanye amaxesha zibuye zibuye, kwaye i-MRIs yokulandelelana isetyenziswa rhoqo. Ukuvavanywa nokulandelelana kombono, ukuba kugcinwe, kuyafuna ingqalelo ekhethekileyo. Ukongezelela, ukulibaziseka kokuphuhlisa akuqhelekanga. Ukulandeleka kubaluleke kakhulu kunye nokuqwalaselwa okhethekileyo kwimiphumo emiselweyo yonyango. Iqela le-Oncology Group (i-COG) I-Survivor Guidelines ichaza ezininzi zezi nkxalabo.
Iimiphumo eziPhezulu kunye neeKhansela zesiBini
Imiphumo emfutshane yonyango lomhlaza ibhekisela kwiimeko ezibangelwa unyango lwezonyango ezinokukhula iminyaka kwiminyaka emva kokunyanga. Sifunda ukuba abasengozini bentsholongwane yengane banomngcipheko ophezulu wezonyango ezivela kwisifo senhliziyo (ngokuqhelekileyo zihambelana ne-chemotherapy), ukungabikho komntwana, ukuya kwintsholongwane yesibini.
Kwisifundo esibhekisele kwiziphumo zesikhathi eside zabantwana abane-retinoblastoma, kwafunyaniswa ukuba emva kweminyaka eminyaka (iminyaka yobudala yayingama-42 kwisifundo), ama-87 ekhulwini kwabantwana abaye basinda kwi-retinoblastoma babenomlinganiselo omnye wonyango ochaphazelekayo unyango. Nangona oku kunokubandezela ukuxoxa, le ngcaciso ibonisa ukuba kubaluleke kangakanani ukususelwa kwintsholongwane yengane ukuba kube nokulandela ixesha elide kunye nodokotela onolwazi ekufumaneni nasekupheni iziphumo zonyango zexesha elide.
Abantwana abaye basinda kwi-retinoblastoma banomngcipheko okhulayo wokuba neekliniki zesekondari (ezitsha zonyango). Nge-retinoblastoma enelifa, inxalenye yale nyukeko ibangelwa ukungasebenzi kwe-genetic suppressor gene kwizinye iifom. Nge-retinoblastoma engenalo ilifa, ezi zintsholongwane zesibili zinokuthi zenzeke njengempembelelo yecandelo le-chemotherapy okanye unyango lwe-radiation. Iingqungquthela eziqhelekileyo zesibini ziquka i- osteosarcoma (umhlaza wesifuba), i-sarcomas, i- melanoma , umdlavuza wamaphaphu kunye ne-lymphoma.
Ukongeza kwimiba yesibini yomhlaza, iimiphumo zengcinezelo yexesha elide ze- chemotherapy kunye neempembelelo zangexesha elide zonyango lwe-radiation zibaluleke kakhulu kuba abaninzi ba bantwana baya kuphila nale miphumo emisiweyo okanye besengozini yemiphumo engapheliyo yonke ixesha elide.
Prognosis
Uninzi lwabantwana olufunyaniswa ukuba ne-retinoblastoma ngoku luya kunyangwa. Umlinganiselo weminyaka emihlanu yokusindisa i-retinoblastoma e-United States yayingama-97.3 ekhulwini ekhangele ixesha eliphakathi kuka-2000 no-2012, kwaye unyango luqhubeka luphucula. Kwaye nabantwana abaneemastastases kwiindawo ezingaphandle kwengqondo (njengomnatha wethambo) bavame ukuphiliswa. Oko kwathiwa, isasazeka ngaphakathi kwengqondo (isifo se-intracranial) sinxulumene nesiphumo esibi.
Ezinye izinto ezihlambulukileyo ezichazayo zibandakanya ukuphuhlisa umhlaza kwilizwe eliphuhlisayo (apho kukho i-cancer ehlobene ne-HPV). I-retinoblastoma yesithathu ixanduva lokufa kwabantu abaninzi ukusuka kwi-retinoblastoma kumazwe athuthukileyo.
Ukuthotyelwa kwangaphambili kwaBantwana abanokuguqulwa kweMigodi
Okwangoku asikho naliphi na indlela yokuthintela ukuphuhliswa kwe-retinoblastoma, kodwa abantwana abasengozini eyongeziweyo ngenxa yokuguquka kwentsholongwane yentsholongwane okanye imbali yentsapho yesi sifo sinokuhlolwa ngethemba lokubamba i-tumor ekuqaleni kwenzeka. Uvavanyo lweFunduscopic (ukuhlolwa kwamehlo ekhangela i-retina) phantsi kwe-anesthesia jikelele ngokukhawuleza emva kokuzalwa kunye nenyanga nganye kunyaka wokuqala wobomi ikhuthazwa rhoqo. Imfuza ingafumaneka kwi-amniotic fluid, kwaye abantwana abachaphazelekayo banokuhlolwa kwaye bahanjiswe kwangaphambili ukuba iimpawu zomhlaza ziphawulwe.
Ukuba umntwana uhlaziya i-retinoblastoma, abanye abantwana kunye namalungu entsapho banokuhlolwa ukuba babone ukuba bane-gene gene.
Ukunyamezela kunye nenkxaso
Njengomzali, ulwazi lunamandla, kwaye luncedo ukufunda konke onako.
Inkxaso nayo iphakamileyo. Ngenxa yobunzima be-tumor, ininzi yoluntu ayinayo amaqela enkxaso ye-retinoblastoma, kodwa kukho uluntu oluxhasayo kwi-intanethi kunye ne-Facebook. Ukubandakanya kule mimandla akunikezeli ngenkxaso njengoko ujamelana nokungena kunye nokuphuma komzali womntwana onomdlavuza, kodwa unokuba yindawo efanelekileyo yokufunda malunga nophando olutsha malunga ne-retinoblastoma. Akukho mntu ukhuthazwayo ukufunda kunye nokwabelana ngezinto ezitsha zokufumana ngaphezu kwabazali abajongene nesi sifo kumntwana wabo.
Njengoko abantwana bekhula, banamathuba amaninzi okuxhasa abantwana. Kukho amaqela enkxaso yomdlavuza owenzelwe ngokukodwa abantu abaselula abanomdlavuza, kunye neenkampu kunye nokuhlaselwa kwakhona.
ILizwi
I-Retinoblastoma iyisisu esiqhekezayo esichaphazelekayo ebandakanya i-nerve tissue kwi-retina. Kubonakala kuninzi kakhulu kubantwana abaselula kakhulu. Ekubeni kunqabile, kubalulekile ukuba abantwana babonakale kwiklinikhi enkulu yomhlaza okanye esibhedlele sezingane, ezineengcali eziqhelanise nezona zonyango ezintle zesifo. Isigulana esiphezulu sisisigxina, kodwa ukufika apho kunokunyanzelisa abazali nabantwana ngemvakalelo, ngokwenyama, nangokwezimali. Ukuba umntwana wakho ufumene unyango, fumana ufumane inkxaso ekhoyo.
> Imithombo:
> Fernandes, A., Pollock, B., no F. Rabito. I-Retinoblastoma eUnited States: Ukunyuka kweMinyaka eyi-40 kunye noHlolo lokuSinda. I-Journal ye-Pediatric Opthalmology kunye neStrabismus . 2017. (Epub ngaphambi kokuprinta).
> Friedman, D., Chou, J., Oeffinger, K. et al. Izimo zonyango ezingapheliyo kumaxhoba angama-Retinoblastoma: Iziphumo ze-Retinoblastoma Survivor Study. Cancer . 2016. 122 (5): 773-81.
> INational Cancer Institute. I-Retinoblastoma Ukwelashwa (PDQ) -Ingxelo yeNgcali yezobuNtu. Ukuhlaziywa 09/19/17. https://www.cancer.gov/types/retinoblastoma/hp/retinoblastoma-treatment-pdq
> Parma, D., Ferrer, M., Luce, L., Giliberto, F., no-I. Szijan. RB1 Utshintsho lweMfuyo kwi-Argentina Retinoblastoma Izigulane. Impembelelo yokuHeliswa kweGenesis. PLoS One . 2017. 12 (12): e0189736.
> Ilayibrari yeSizwe ka-US yamayeza. I-Reference Reference Home. Retinoblastoma. 12/17/17. https://ghr.nlm.nih.gov/condition/retinoblastoma#sourcesforpage