Isifo seVon Hippel-Lindau (i-VHL) sisimo semfuza esenza ukuba ukukhula kweempahla zegazi kwiindawo ezahlukeneyo zomzimba wakho. Ezi zikhula ezingavamile zingakhula phambili zibe ziimvumi kunye nama-cysts. I-VHL ibangelwa ukuguqulwa kwimizimba elawula ukukhula kweeseli, kwi-chromosome yakho yesithathu.
Bobabini abesilisa kunye nabesifazane bazo zonke iintlanga zichaphazeleka yi-VHL, malunga ne-1 kuma-36,000 abanemeko.
Uninzi lwabantu luqala ukufumana iimpawu ezineminyaka engama-23 ubudala, kwaye, ngokuqhelekileyo, zifumana ukuxilongwa ngexesha eli-32.
Iimpawu
Uninzi lweemathumba ezibangelwa yi-VHL alinakuzilimaza kodwa zingabangela umdlavuza. Izidumbu ziqhelekileyo zifumaneka kwi:
- Amehlo. Ebizwa ngokuba yi-retinal hemangioblastomas (imibala yemithambo yegazi), ezi zicubu hayikrakra kodwa zingabangela iingxaki kwiso, njengokulahlekelwa kombono kunye nokunyuswa kwamehlo ( glaucoma ).
- Ubunjani. Eyaziwa ngokuba yi-hemangioblastomas (imibala yemigodi yegazi), aba bantu abanomdlavuza kodwa bangabangela iimpawu ze-neurological (ezifana nobunzima bokuhamba) ngenxa yoxinzelelo ababeka kuzo kwiingqondo.
- Zezintso. Ezi zihlwele ziyakwazi ukuba nomhlaza. Olu hlobo lomhlaza, olubizwa ngokuba yi- renal cell carcinoma , luyimbangela ebangela ukufa kwabantu abane-VHL.
- Izilonda ze-Adrenal. Ebizwa ngokuthi i-pheochromocytomas, ayidla ngokukrakra kodwa ingabangela ukuba i-adrenaline iveliswe.
- Pancreas. Ezi zicubu zivame ukuba zingasomdlavuza, kodwa, ngamanye amaxesha, zinokuba ngumhlaza.
Imizi ingahlakulela emlanjeni, indlebe yangaphakathi, umzila wesisu, umphunga kunye nesibindi. Abanye abantu banokufumana iimvumba endaweni enye xa abanye bechaphazeleka kwiindawo ezininzi. Iipesenti ezili-10 kuphela zabantu abane-VHL bahlakulela indlebe.
Iimvumi zendlebe kufuneka ziphathwe ukunqanda ukungeva.
Ukufumana iSifo
Ukuhlolwa kwe-Genetic, ngokusebenzisa uvavanyo lwegazi , yindlela efanelekileyo kakhulu yokufumanisa i-VHL. Ukuba ngaba umzali wakho u-VHL, ngoko kukho ikhesenti elingama-50 ukuba uzuze ilifa. Nangona kunjalo, akuzona zonke iimeko ze-VHL ezizuze ilifa. Phantse, iipesenti ezingama-20 ze-VHL zinokuguqulwa kofuzo olungapheliyo kubazali babo. Ukuba unayo i-VHL, kukho ithuba eliphezulu kakhulu lokuba uza kuphuhlisa ubuncinane i-tumor enye ngexesha lobomi bakho-i-97 ekhulwini yexesha le-tumors likhula ngaphambi kokuba uneminyaka engama-60.
Unyango
Izinyango zokwelapha zixhomekeke apho i-tumor yakho ikhona khona. Iimvumi ezininzi zingasuswa kunye nokuhlinzwa. Abanye akudingeki ukuba basuswe ngaphandle kokuba babangele iimpawu (umzekelo, isifo seengqondo zixinzelele ingqondo yakho).
Ukuba unayo i-VHL, kuya kufuneka uhlolwe rhoqo, kunye ne-imagination magnontic resonance ( MRI ) okanye i-computed tomography ( CT ) ezibonwe ngengqondo, isisu, kunye neentso zokubukela izicubu ezintsha. Iimviwo zamehlo kufuneka zenziwe rhoqo.
Umlindi osondeleyo kufuneka agcinwe phezu kwazo zonke iinjongo zesifo. Ezi zinokuthi zisuswe ngokugqithiseleyo ukwenzela ukunciphisa umngcipheko wokuba nomdlavuza wezintso. Phantse, iipesenti ezingama-70 zabantu abane-VHL bavelisa umdlavuza weengtso xa beneminyaka engama-60.
Nangona kunjalo, ukuba umhlaza wengqondo awuyi kukhula ngelo xesha, kukho ithuba elihle lokuba lungeke lenzeke.
Imithombo:
Evans, JP (2002). isifo sikaVippel-Lindau. eMedicine, kufumaneka kwi http://www.emedicine.com/ped/topic2417.htm
VHL Alliance (2016). VHL Facts,. http://vhl.org/about/resources/vhl-facts/