Une mapundu aya asinganzwisisike pamuviri wako here? Ngatitaure nezve chirwere chevon Hippel-Lindau Disease (VHL).

Ungave uchishamisika kuti sei ndichiramba ndiine mapundu asinganzwisisike mumuviri wangu wese, kana kuti nei zvimwe zvirwere zvisingamboperi. Dzimwe nguva chikonzero cheizvi chinogona kunge chakaoma kupfuura zvatinofunga. Nhasi tichataura nezvechirwere chisingawanzoitiki asi chakakosha chekuziva. Icho chirwere chevon Hippel-Lindau, kana kuti tinochidana muchidimbu kuti `(VHL)`.

Chii chinonzi von Hippel-Lindau (VHL)? Ngatizvinzwisise zviri nyore.

Zvakanaka, ikozvino ungangove uchishamisika kuti iyi `(VHL)` chii. Zvichitaurwa zviri nyore, von Hippel-Lindau chirwere chisingawanzoitiki chemajini. Chinoitika kana paine `(genetic mutation)` kana shanduko mune mamwe majini mumuviri wako. Nekuda kweizvi, une mukana wekuva nemabundu `(malignant)`, pamwe nemabundu `(benign)` nemacysts, muzvikamu zvakasiyana zvemuviri wako. Vanachiremba dzimwe nguva vanodana chirwere ichi kuti `(von Hippel-Lindau syndrome`.

Ongororo yakawana kuti 97% yevanhu vane chirwere ichi chekuchinja kwemajini vachawana magomarara aya nemamwe ane chekuita neVHL pavanosvika makore 65. Kazhinji, mushonga mukuru wemagomarara ane chekuita neVHL kuvhiyiwa kubvisa magomarara.

Ndezvipi zvirwere zvegomarara zvinogona kusanganiswa nechirwere cheVHL?

Kana uine chirwere ichi (VHL), uri panjodzi huru yekuwana imwe kana kupfuura yemhando dzekenza dzinotevera:

• Kansa yetsvo yakasviba (ccRCC): Iyi ndiyo mhando yekenza yetsvo yakajairika. Sekureva kwenyanzvi, pakati pe25% ne60% yevanhu vane VHL vanogona kuwana kenza iyi. Yeukai, itsvo ndeimwe yenhengo dzakakosha mumuviri wedu, saka kenza iri mavari chinhu chekunyanya kunetseka nezvacho.
• Matumbu epancreatic neuroendocrine (pancreatic NETs): Aya ndiwo mhando yebundu risingawanzoitiki rinotanga mumasero eendocrine ari mupancreas yako. Anogona kukura pakati pe9% ne17% yevanhu vane VHL.
• Pheochromocytoma: Iri ibundu risingawanzoitiki asi rinorapika rinokura muadrenal gland. Mabundu aya anogona kutanga semabundu asiri ekenza, asi anogona kuzopedzisira ava kenza. Anowanikwa mu10% kusvika 20% yevanhu vane VHL.
• Broad ligament cystadenomas: Chirwere ichi chinokanganisa vakadzi. Chinoitika muvanhu vanosvika 10% vane VHL. Ibundu rinoita sebundu riri pedyo nemachubhu efallopian.

Ndeapi mabundu asiri ekenza anouya neVHL?

Haisi kenza chete, asiwo bundu risiri rekenza, kureva kuti, bundu risingapararire kune dzimwe nhengo dzemuviri (metastasize), rinogona kukura kana VHL iripo. Chinonyanya kukosha pazvose izvi ihemangioblastoma.Aya mabundu asiri ekenza anoumbwa mutsinga dzeropa dzeuropi, musana, kana retina. Kunyangwe asingapararire, anogona kukura uye kukanganisa nhengo dzakapoteredza, zvichikonzera matambudziko makuru ehutano.

Mhando dzehemangioblastomas dzinogona kuonekwa dzine chekuita ne `(VHL)` ndedzi:

• Retinal hemangioblastoma: Iri ibundu rinokura muziso. Rinogona kutokonzera kurasikirwa nekuona. Tsvagiridzo inoratidza kuti rinokura muvanhu 60% vane (VHL).
• Matumbu europi neCerebellar hemangioblastomas: Aya mabundu ari muuropi. Anogona kukanganisa kuenzana kwako uye kukonzera mamwe matambudziko. Anokanganisa pakati pe13% ne72% yevanhu vane VHL.
• Spinal cord hemangioblastoma: Rudzi urwu rwehemangioblastoma runokura pakati pe13% ne50% yevanhu vane (VHL).

Pamusoro pezvo, kana uine VHL, uri panjodzi yekuve nemabundu asina kenza uye ma cysts akadai se:

• Epididymal cystadenomas: Aya mabundu anokanganisa varume. Anokura muepididymis, chimiro chidiki chakaita sechubhu chinochengeta urume, pedyo nemachende. Anowanikwa pakati pe25% ne60% yevarume vane VHL.
• Matumbu emudumbu eEndolymphatic (ELST): Aya matumbu asingawanzoitiki. Kana uine VHL, anogona kukura munzeve yemukati. Dambudziko iri rinokanganisa vanhu vari pakati pe10% ne25% vane VHL.
• MaCysts: Aya maCysts ane mvura. Vanhu vane VHL vanogona kuwana maCysts aya muitsvo nemupancreas.

Chirwere cheVHL chakajairika zvakadii?

Ichi chirwere chisingawanzoitiki. Chinobata munhu mumwe chete pavanhu 36,000. Vanhu vazhinji vane VHL vanotanga kuratidza zviratidzo zvechirwere ichi vari pakati pemakore makumi maviri. Izvi zvinoreva kuti unofanira kungwarira izvi uchiri mudiki.

Ndezvipi zviratidzo zvechirwere chevon Hippel-Lindau?

Nekuti chirwere ichi chinogona kukonzera bundu mumuviri wako wese, zviratidzo zvinogona kusiyana. Zvinoenderana nekuti bundu riri kupi uye saizi yemabundu acho. Unogona kuona zviratidzo zvakaita seizvi:

• Kurwadziwa nemusoro nguva nenguva.
• Kurasikirwa nekunzwa kana kurira nguva dzose munzeve (tinnitus).
• Kukwira kweropa.
• Kurasikirwa nekuenzana panguva yekufamba.
• Kuderera kwesimba remhasuru kana kuoma kuronga mafambiro.
• Matambudziko ekuona.
• Kurutsa.

Fungidzirai kuti taizonetseka zvakadini dai taiva nechimwe kana zviviri zvezviratidzo izvi? Saka kana uine zvimwe zvezviratidzo izvi panguva imwe chete, zvakakosha kutsvaga rubatsiro rwechiremba.

Chii chinokonzera chirwere cheVHL?

Von Hippel-Lindau syndrome chirwere chinoberekwa nhaka . Chinoitika kana ukagara nhaka kopi isina kujairika yejini rinodzvinyirira bundu rinonzi VHL kubva kune mumwe wevabereki vako.

Zvichitaurwa zviri nyore, aya "majini anodzvinyirira bundu" anomisa masero kuti asapatsanuke nekukurumidza, izvo zvinogona kukonzera kenza. Zvakafanana nemabhureki emotokari. Asi kana majini aya akachinja-chinja, zvakafanana nekubvisa mabhureki woisa accelerator. Kukura kwemasero kunokurumidza zvisingadzoreki.

Chirwere ichi chinotapuriranwa nenzira ye autosomal dominant inheritance pattern. Izvi zvinoreva kuti kana mumwe wevabereki vako aine gene reVHL risingawanzoitiki, une mukana we50% wekurigara nhaka uye kutanga chirwere che von Hippel-Lindau. Zvisinei, tsvakurudzo yakaratidza kuti vanhu vanosvika 10% vane VHL havana nhoroondo yemhuri yechirwere ichi. Izvi zvinoreva kuti kuchinja kwemajini matsva kunogona kuitikawo.

Chirwere cheVHL chinoonekwa sei?

Vanachiremba vanogona kufungidzira kuti une VHL kana uine zviratidzo zvechirwere chinokonzerwa neVHL, zvakaita sehemangioblastoma kana clear cell renal carcinoma. Asi nzira chete yekusimbisa izvi ndeyekuongorora majini. Kana mumwe munhu mumhuri yako aine chirwere cheVon Hippel-Lindau, zvakakosha kubvunza chiremba wako nezvekuongororwa majini kwako.

Ndeipi mishonga yekurapa chirwere cheVHL?

Nzira dzekurapa dzinosiyana zvichienderana nerudzi rwebundu reVHL kana cyst. Chiremba wako achatsanangura nzira dzekurapa dzakakunakira. Nzira dzinonyanya kushandiswa dzekurapa ndeidzi:

• Kuvhiyiwa kubvisa bundu.
• Kurapa nemakemikari.
• Kurapa nemwaranzi.
• Kurapa kwakarongwa kunosanganisira zvinhu zvakaita se peptide receptor radionuclide therapy (PPRT) uye tyrosine kinase inhibitors (TKI).
• Kurapa nekudzivirira muviri.
• Kurapa nemahormone.

Chinhu chakakosha ndechekuongorora chirwere ichi nekukurumidza uye kutanga kurapwa kwakakodzera. Ndipo chete paunowana mhedzisiro yakanaka.

Chirwere cheVHL chinogona kurapwa zvachose here?

Zvinosuwisa kuti pari zvino hapana mushonga wechirwere chevon Hippel-Lindau. Chinangwa chikuru chekurapa ndechekuwana nekubvisa bundu nekukurumidza. Chiremba wako anogona kukurudzira kuvhiyiwa kubvisa bundu pamwe chete nedzimwe nzira dzekurapa.

Kana ndine VHL, ndinofanira kutarisira chii?

Kana uine chirwere ichi, uchafanirwa kuongororwa nguva nenguva kuti uone kana paine zviratidzo zvezvirwere zvakasiyana. Kuonekwa nekurapwa kwebundu nekukurumidza kunogona kubatsira kuderedza kukanganisa kunoita chirwere chevon Hippel-Lindau muhupenyu hwako. Ndosaka zvichikosha kuti uwane bvunzo dzinokurudzirwa nachiremba wako.

Chirwere cheVHL chinogona kudzivirirwa here?

Kwete, hazvigone kudzivirirwa. Izvi zvinokonzerwa nekuti chirwere chevon Hippel-Lindau chinokonzerwa nekuchinja kwemajini kunopfuudzwa kubva kumubereki mumwe kuenda kune mumwe. Kana mumwe munhu mumhuri yako aine VHL, kuongororwa kwemajini kunogona kukubatsira kuona kana iwewo uine kuchinja uku.

Kunyangwe usingakwanise kudzivirira chirwere chevon Hippel-Lindau, zvinobatsira kuziva njodzi yako uye kunzwisisa kuti `(VHL)` ingakanganisa sei iwe. Kana uchiziva kuti uri panjodzi, vanachiremba vanogona kutarisa zviratidzo zvemabundu ane chekuita ne`(VHL)` uye kutora matanho ekuzvibvisa nekukurumidza.

Kana uine jini re (VHL) uye uchironga kuva nemwana, zvakanaka kusangana nenyanzvi yezvemajini kuti mukurukure nezvengozi yekuti mwana wako agare nhaka jini iri.

Pane here bvunzo dzekuongorora zvirwere zvine chekuita neVHL?

Hapana gwara rekuongorora izvi, sezvariripo kune mamwe marudzi ekenza (senge kuongororwa kwekenza kunokurudzirwa neUS Preventive Services Task Force). Zvisinei, kana kuongororwa kwemajini kukaratidza kuti une gene reVHL risingawanzoitiki, timu yako yehutano inogona kukurudzira mimwe miedzo kuti ikubatsire kuona matambudziko nekukurumidza. Semuenzaniso, vanogona kukurudzira kuti uite abdominal magnetic resonance imaging scan (MRI) makore maviri ega ega kuti utarise kenza ine chekuita neVHL, senge clear cell renal carcinoma.

Ndingazvitarisira sei? Ndingaitei?

Kurarama neVHL zvinoreva kurarama usina chokwadi. Kana ukagara nhaka gene mutation inokonzera VHL, une mukana we50% wekuva nechirwere ichi. Kana ukadaro, une mukana we97% wekuva nemamwe marudzi ekenza nezvimwe zvirwere zvakakomba.

Zvisinei, hapana anogona kufanotaura chaizvo kuti `(VHL)` ichakanganisa sei hupenyu hwako. Heano mamwe maitiro anogona kukubatsira kugadzirisa matambudziko aya:

• Wana rubatsiro rwehutano hwepfungwa: Imwe ongororo yakaratidza kuti kuongororwa kweVHL kunogona kukanganisa pfungwa, zvakaita sekuzvidya mwoyo uye kutya. Saka, zvakakosha kutaura nachiremba wepfungwa kana mupi wezano.
• Dzivirira hutano hwako hwese: Idya chikafu chakakwana - nyama dzakawanda dzisina mafuta akawanda, zviyo zvakakwana, miriwo yemashizha, nemichero. Edza muviri (izvo zvinogonawo kuderedza kushushikana). Bvunza timu yako yezvechiremba nezvemajekiseni akakunakira.

Ndinofanira kutsvaga mazano ekurapa rini?

Kana ukaona zviratidzo zvakaita seizvi, unofanira kufonera chiremba wako nekukasika:

• Kuchinja kwekuona kana kunzwa kwako.
• Kurwadziwa nemusoro nguva nenguva.
• Kusvotwa kana kurutsa kusingafungidzirwe kuti kunokonzerwa nechimwe chirwere.
• Kukwira kweropa kamwe kamwe.
• Kana ukangoerekana wava nedambudziko rekufamba, kuchengetedza kuenzana, kana kufambisa zvinhu pamwe chete.

Mibvunzo ipi yandinofanira kubvunza chiremba wangu?

Fungidzira kuti wakaudzwa kuti mumwe munhu mumhuri mako ane chirwere chevon Hippel-Lindau. Kana zvakadaro, heano mimwe mibvunzo yaunogona kubvunza chiremba wako:

• Zvakabuda muongororo yangu yemajini zvinoti chii?
• Vamwe vese vemhuri yangu vanofanira kuongororwa majini here?
• Ndinofanira kuongororwa kangani kuti ndione zviratidzo zvekutanga zvezvirwere zvinogona kunge zvakabatana ne `(VHL)`?

Pakupedzisira, zvinhu zvekurangarira (Mharidzo Yekuenda Kumba)

Chirwere cheVon Hippel-Lindau (VHL) chirwere chisingawanzoitiki chemajini. Chinobata munhu mumwe chete pavanhu 36,000. Saka, kuziva kuti uri mumwe wevanhu 36,000 ivavo, kunogona kushamisa zvikuru, kunyanya kana pasina munhu mumhuri yako akamborwara nechirwere ichi.

Kana kuongororwa kwemajini kukaratidza kuti une chirwere (VHL), zvipe nguva yekuongorora nhau. Wobva watora nguva yekunzwisisa zvinorehwa nechirwere chako. Semuenzaniso, unogona kunge uine mibvunzo nezvenjodzi yako yekubatwa nekenza yemhando dzakasiyana uye bundu, kana kuti chirwere chako chichakanganisa sei vamwe mumhuri yako. Usambozeza kubvunza timu yako yezvekurapa nezvezvaunotarisira. Uye usakanganwa kukumbira rubatsiro rwekugadzirisa kushushikana kunouya nekugara nechirwere (VHL). Hausi wega, uye kune vanhu vazhinji vanogona kukubatsira parwendo urwu.

Von Hippel-Lindau, VHL, chirwere chemajini, kenza, bundu, zviratidzo, kurapwa