Von Hippel-Lindau chirwere (VHL) chibereko chinokonzera kuti ropa risina kuchena rikure munzvimbo dzakasiyana dzomuviri wako. Izvi kukura zvisingawanzo zvinogona kuwedzera kukura kuva matumire uye ma cysts. VHL inokonzerwa nekuchinja kwegene rinodzora kushamba kwekamuri, iri pane chechitatu chromosome.
Vose varume nemadzimai vemarudzi ose akanganisirwa neVHL, vane inenge 1 muna 36,000 vane mamiriro acho ezvinhu.
Vanhu vazhinji vanotanga kuona zviratidzo zvinenge makore makumi maviri nemaviri, uye, paavhareji, vanogamuchira kuongororwa nenguva iyo vari 32.
Zviratidzo
Mazhinji emakumbo anokonzerwa neVHL haana njodzi asi anogona kuva kenza. Tumha dzakawanda dzinowanika mu:
- Eyes. Inonzi retinal hemangioblastomas (masasa emidziyo yeropa inoputika), izvi zvipembenene hazvisi chekenza asi zvinogona kukonzera matambudziko muziso sezvakaita kurasikirwa nekuona uye kuwedzerwa kweziso ( glaucoma ).
- Ubongo. Anozivikanwa sehemangioblastomas (masasa emidziyo yeropa yakavhiringidzika), vanhu vazhinji havatengesi asi vanogona kuita zviratidzo zvehutano (zvakadai sekuoma kufamba) nemhaka yezvinetso zvavanoisa pane zvikamu zveuropi.
- Kidyo. Idzi vanhu vazhinji ndivo vanowanzova nekenza. Rudzi urwu rwekenza, inonzi renal cell carcinoma , ndiyo inokonzera rufu rwevanhu vane VHL.
- Adrenal glands. Zvinonzi pheochromocytomas, hazviwanzo kenza asi zvinogona kuita kuti adrenaline yakawanda ibudiswe.
- Pancreas. Izvi zvipembenene hazviwanzo kenza, asi zvinogona, pane dzimwe nguva, kuva chirwere.
Misa inogonawo kukura mumutsipa, mukati mezeve, chikamu chepabonde, mapapu, uye chiropa. Vamwe vanhu vanogona kungotora tumarara mune imwe nzvimbo asi vamwe vanogona kuchinjwa munzvimbo dzakawanda. Vapfumbamwe chete muzana yevanhu vane VHL vanoita matumire emakumbo.
Mhete dzekunzwa dzinofanira kutarisirwa kudzivirira matsi.
Kuwana Kuongororwa
Kuongorora zvirwere, kuburikidza neropa rekuongorora ropa , ndiyo nzira yakanakisisa yekuongorora VHL. Kana mubereki wako ane VHL, ipapo pane 50 muzana yeruzivo yekuti wakagara nhaka mamiriro acho. Zvisinei, haasi matambudziko ose eVHL akagara nhaka. Inenge, 20 muzana yeVHL vane genetic mutation iyo haina kudarika kubva kuvabereki vavo. Kana iwe uine VHL, pane chiitiko chakakwirira kwazvo kuti iwe uchakurira imwe tumarara mukati mehupenyu hwako - 97 muzana yenguva dzomumakumbo dzinokura usati wava nemakore makumi matanhatu.
Kurapa
Mishonga inoshandiswa inobva pane iyo gumbo rako riripo. Matumbu akawanda anogona kubviswa neokuvhiyiwa. Vamwe havadi kubviswa kunze kwekunge vari kukonzera zviratidzo (somuenzaniso, chirwere chepfungwa chinowedzera kuuropi yako).
Kana iwe uine VHL, iwe uchada kuongororwa nguva dzose, uyewo magnetic resonance imaging ( MRI ) kana kuti computed tomography ( CT ) kuongorora kweuropi, mimba, uye itsvo kuti uone mishonga mitsva. Mavhenekeri eziso anofanira kuitwawo nguva dzose.
Iro rinotarisa rinofanira kuchengetedzwa pamusoro pemakumbo epipi. Izvi zvinogona kubviswa chirwere kuti zvideredze njodzi yekuve nekenza yepenyo. Anenge makumi manomwe kubva muzana yevanhu vane VHL vanoita kuti kenza yempfungwa ive nemakore makumi matanhatu.
Zvisinei, kana chirwere chetsvo hachikudziri panguva iyoyo, pane mukanano wakanaka hazvizoitiki.
Sources:
Evans, JP (2002). von Hippel-Lindau chirwere. eMedicine, yakawanikwa pa http://www.emedicine.com/ped/topic2417.htm
VHL Alliance (2016). VHL Facts ,. http://vhl.org/about/resources/vhl-facts/