Isifo se-Von Hippel-Lindau (i-VHL) yisimo sezakhi zofuzo esibangela ukukhuphuka kwegazi okungavamile ezindaweni zonke zomzimba wakho. Lezi zikhula ezingavamile zingathuthuka zibe izicubu nama-cysts. I-VHL ibangelwa ukuguqulwa kwesigenja esilawula ukukhula kwe-cell, okukhona ku-chromosome yakho yesithathu.
Bobabili abesilisa nabesifazane bazo zonke izizinda zithinteka yi-VHL, cishe cishe ku-1 ku-36,000 banesimo.
Abantu abaningi baqala ukuthola izimpawu ezineminyaka engama-23 ubudala, futhi, ngokwesilinganiso, bathola ukuxilongwa ngenkathi beneminyaka engu-32.
Izimpawu
Iningi lamathumba abangelwa i-VHL ayinalutho kodwa ingaba nomdlavuza. Izicubu zivame ukutholakala ku:
- Amehlo. Ebizwa ngokuthi i-retinal hemangioblastomas (izixuku zemithanjeni yegazi), lezi zicubu azikhambi komdlavuza kodwa zingabangela izinkinga emehlweni njengokulahlekelwa kombono nokunyuka kwesisindo (i- glaucoma ).
- Ubuchopho. Eyaziwa ngokuthi i-hemangioblastomas (izixuku zemithambo yegazi), lezi zixuku azikona umdlavuza kodwa zingabangela izimpawu ze-neurological (njengokunzima ukuhamba) ngenxa yengcindezi abafaka kuzo izingxenye zobuchopho.
- Izinso. Lezi zixuku yizo ezingase zibe nomdlavuza. Lolu hlobo lomdlavuza, olubizwa nge- renal cell carcinoma , luyisizathu esibangela ukufa kwabantu abane-VHL.
- Izigulane eziyingozi. Ebizwa nge-pheochromocytomas, ngokuvamile ayinomdlavuza kodwa ingabangela ukuba i-adrenaline eningi ikhiqizwe.
- I-Pancreas. Lezi zicubu ngokuvamile aziyona umdlavuza, kodwa, ngezikhathi ezithile, zingaba ngumdlavuza.
Imisipha ingakhula futhi emgodleni, indlebe yangaphakathi, i-genital tract, i-lung, nesibindi. Abanye abantu bangathola izicubu endaweni eyodwa kanti abanye bangathinteka ezindaweni eziningi. Amaphesenti ayishumi kuphela abantu abane-VHL bahlakulela izicubu zezindlebe.
Ama-tumors endlebe kufanele aphathwe ukuze avimbe ukuzithulu.
Ukuthola Ukuxilongwa
Ukuhlolwa kwegazi , ngokusebenzisa ukuhlolwa kwegazi , kuyindlela ephumelela kakhulu yokuthola i-VHL. Uma umzali wakho ene-VHL, khona-ke kunamathuba angu-50 wamaphesenti wokuthi uzuze lesi sifo. Nokho, akuzona zonke izimo ze-VHL ezizuzwa njengefa. Cishe, amaphesenti angu-20 e-VHL anezakhi zofuzo ezingazange zidluliselwe kubazali bazo. Uma une-VHL, kunethuba eliphakeme kakhulu ukuthi uzothuthukisa okungenani isisu esisodwa ngesikhathi sokuphila kwakho - amaphesenti angu-97 ezithombeni zesikhathi esakhula ngaphambi kokuba uneminyaka engama-60.
Ukwelapha
Izinketho zokwelapha zixhomeke lapho utholakala khona isisu sakho. Izicubu eziningi zingasuswa ngokuhlinzwa. Abanye akudingeki basuswe ngaphandle kokuthi babangele izimpawu (isibonelo, isisu sobuchopho sicindezela ebuchosheni bakho).
Uma une-VHL, kuzodingeka ube nokuhlolwa okwenziwe njalo, kanye nokucabangela kwamagnetic resonance ( MRI ) noma ukuhlolwa kwe-tomography ( CT ) yebuchopho, isisu, nezinso zokubuka izicubu ezintsha. Ukuhlolwa kwamehlo kufanele kwenziwe njalo.
I-watch watch kufanele igcinwe phezu kwanoma yiziphi izinsizwa zezinso. Lokhu kungasuswa ngokuhlinzwa ukuze kuncishiswe ingozi yokuthuthukisa umdlavuza wezinso. Cishe, amaphesenti angu-70 abantu abane-VHL bahlakulela umdlavuza wezinso uma uneminyaka engama-60.
Kodwa-ke, uma umdlavuza wezinso ungaqhubeki ngaleso sikhathi, kunethuba elihle lokuthi ngeke lenzeke.
Imithombo:
U-Evans, JP (2002). Isifo se-von Hippel-Lindau. iMedicine, ifinyelelwe ku-http: //www.emedicine.com/ped/topic2417.htm
I-VHL Alliance (2016). VHL Facts ,. http://vhl.org/about/resources/vhl-facts/