Matenda a Von Hippel-Lindau (VHL) ndi matenda omwe amachititsa kuti madzi asapitirire kukula m'madera osiyanasiyana a thupi lanu. Kukula kosazolowereka kungapangitse kukhala matumbo ndi makoswe. VHL imayambitsidwa ndi kusintha kwa jini komwe kumayendetsa kukula kwa maselo, komwe kuli kromosome yanu yachitatu.
Amuna ndi akazi a mitundu yonse amakhudzidwa ndi VHL, ndipo pafupifupi 1 mwa 36,000 ali ndi vutoli.
Ambiri amayamba kuwona zizindikiro zakubadwa ali ndi zaka 23, ndipo, kawirikawiri, amapezeka kuti ali ndi zaka 32.
Zizindikiro
Matenda ambiri omwe amapezeka ndi VHL alibe vuto koma akhoza kukhala khansa. Ziphuphu zimapezeka kwambiri mwa:
- Maso. Amatchedwa retinal hemangioblastomas (mitsempha ya mitsempha yamagazi), zotupazi sizimayambitsa khansa koma zingayambitse mavuto m'maso monga kuperewera kwa masomphenya komanso kuwonjezeka kwa diso ( glaucoma ).
- Ubongo. Amadziwika kuti hemangioblastomas (mitsempha ya mitsempha yamagazi), anthuwa sakhala ndi khansa koma amachititsa kuti zizindikiro zokhudzana ndi ubongo (monga kuvutika kuyenda) chifukwa cha kupsyinjika kumene amaika pambali pa ubongo.
- Impso. Misala imeneyi ndi omwe amatha kukhala ndi khansa. Mtundu umenewu wa khansa, wotchedwa renal cell carcinoma , ndi umene umayambitsa imfa kwa anthu omwe ali ndi VHL.
- Zozizira za adrenal. Amatchedwa pheochromocytomas, kawirikawiri si khansa koma amachititsa kuti adrenaline ikhale yopangidwa.
- Pancreas. Matendawa nthawi zambiri samakhala ndi khansa, koma nthawi zina amatha kukhala khansara.
Misa imatha kukhalanso m'kati mwa msana, khutu la mkati, chiwalo cha m'mimba, mapapo, ndi chiwindi. Anthu ena amatha kutenga zotupa m'madera amodzi pomwe ena akhoza kukhudzidwa m'madera ambiri. Ndi 10 peresenti ya anthu omwe ali ndi VHL amakhala ndi ziphuphu zamakutu.
Matenda a khutu ayenera kuthandizidwa kuti athetse kugontha.
Kupeza Kuzindikira
Kuyezetsa magazi, kudzera mu kuyesa magazi , ndiyo njira yabwino kwambiri yodziwira VHL. Ngati kholo lanu liri ndi VHL, ndiye kuti muli ndi mwayi wa 50 peresenti kuti mwatengera chikhalidwecho. Komabe, sizinthu zonse za VHL zomwe zalandidwa. Pafupifupi, 20 peresenti ya VHL imakhala ndi kusintha kwa majini komwe sikunaperekedwe kuchokera kwa makolo awo. Ngati muli ndi VHL, pali mwayi wapamwamba kwambiri kuti mudzakhazikitsa chotupa chimodzi m'moyo wanu - 97 peresenti ya zotupa za nthawi zimakula musanafike zaka 60.
Chithandizo
Njira zothandizira zimadalira kumene chifuwa chanu chiri. Matenda ambiri angathe kuchotsedwa ndi opaleshoni. Ena samayenera kuchotsedwa pokhapokha atayambitsa zizindikiro (mwachitsanzo, kupweteka kwa ubongo kumaganizo anu).
Ngati muli ndi VHL, muyenera kuyesedwa kawirikawiri, kuphatikizapo kujambula maginito ( MRI ) kapena computed tomography ( CT ) za ubongo, mimba, ndi impso kuti muyang'ane zotupa zatsopano. Mayeso a maso ayenera kuchitidwa nthawi zonse.
Mlonda ayenera kuyang'aniridwa ndi mphuno iliyonse ya impso. Izi zikhoza kuchotsedwa opaleshoni pofuna kuchepetsa chiopsezo cha khansa ya impso. Pafupifupi, anthu 70 pa 100 alionse a VHL amakhala ndi khansa ya impso ali ndi zaka 60.
Komabe, ngati khansara ya impso isakule panthawiyo, pali mwayi wabwino kuti sichidzachitika.
Zotsatira:
Evans, JP (2002). Matenda a Hippel-Lindau. eMedicine, imapezeka pa http://www.emedicine.com/ped/topic2417.htm
VHL Alliance (2016). Mfundo za VHL,. http://vhl.org/about/resources/vhl-facts/