Kodi mumapeza ziphuphu zachilendo izi pathupi lanu? Tiyeni tikambirane za matenda a von Hippel-Lindau (VHL).

Mwina mukudabwa chifukwa chake ndimapeza ziphuphu zachilendo thupi langa lonse, kapena chifukwa chake matenda ena samatha. Nthawi zina chifukwa cha izi chingakhale chovuta kwambiri kuposa momwe timaganizira. Lero tikambirana za vuto losowa koma lofunika kwambiri loti tidziwe. Imeneyo ndi matenda a von Hippel-Lindau, kapena mwachidule timatcha `(VHL)`.

Kodi von Hippel-Lindau (VHL) ndi chiyani? Tiyeni timvetse mosavuta.

Chabwino, tsopano mwina mukudabwa kuti '(VHL)' iyi ndi chiyani. Mwachidule, von Hippel-Lindau ndi matenda osowa kwambiri a majini. Amachitika pakakhala '(masinthidwe a majini)' kapena kusintha kwa majini ena m'thupi lanu. Chifukwa cha izi, mumakhala ndi mwayi waukulu wokhala ndi zotupa '(zoyipa)', komanso zotupa '(zoyipa)' ndi ma cysts, m'mbali zosiyanasiyana za thupi lanu. Madokotala nthawi zina amatcha vutoli '(von Hippel-Lindau syndrome'.

Kafukufuku wapeza kuti 97% ya anthu omwe ali ndi kusintha kwa majini kumeneku adzakhala ndi zotupa izi ndi zina zokhudzana ndi VHL akafika zaka 65. Nthawi zambiri, chithandizo chachikulu cha zotupa zokhudzana ndi VHL ndi opaleshoni yochotsa zotupazo.

Ndi khansa ziti zomwe zingagwirizane ndi matenda a VHL?

Ngati muli ndi vuto la `(VHL)` ili, muli pachiwopsezo chachikulu chotenga mtundu umodzi kapena ingapo wa khansa iyi:

• Khansa ya impso yoyera (ccRCC): Iyi ndi khansa ya impso yofala kwambiri. Malinga ndi akatswiri, pakati pa 25% ndi 60% ya anthu omwe ali ndi VHL amatha kudwala khansa imeneyi. Kumbukirani kuti impso ndi chimodzi mwa ziwalo zofunika kwambiri m'thupi lathu, kotero khansa yomwe ili m'thupi mwawo ndi chinthu chofunikira kuganizira kwambiri.
• Matenda a Pancreatic neuroendocrine (pancreatic NETs): Izi ndi mtundu wosowa wa chotupa womwe umayamba m'maselo a endocrine mu kapamba wanu. Amatha kufalikira pakati pa 9% ndi 17% ya anthu omwe ali ndi VHL.
• Pheochromocytoma: Ichi ndi chotupa chosowa koma chochiritsika chomwe chimayamba mu adrenal gland. Chotupachi chingayambe ngati chotupa chosakhala khansa, koma pambuyo pake chikhoza kukhala khansa. Chimapezeka mwa 10% mpaka 20% ya anthu omwe ali ndi VHL.
• Matenda a Broad ligament cystadenomas: Matendawa amakhudza akazi. Amachitikira pafupifupi 10% ya akazi omwe ali ndi VHL. Ndi chotupa chomwe chimapangika pafupi ndi machubu a fallopian.

Kodi ndi zotupa ziti zomwe sizili khansa zomwe zimabwera ndi VHL?

Sikuti khansa yokha, komanso zotupa zomwe si khansa, zomwe ndi zotupa zomwe sizifalikira ku ziwalo zina za thupi (metastasize), zimatha kuchitika VHL ikakhalapo. Chofunika kwambiri mwa izi ndi hemangioblastoma.Izi ndi zotupa zomwe sizili khansa zomwe zimapangika m'mitsempha yamagazi ya ubongo, msana, kapena retina. Ngakhale sizifalikira, zimatha kukula ndikukhudza minofu yozungulira, zomwe zimayambitsa mavuto akulu azaumoyo.

Mitundu ya hemangioblastomas yomwe ingawoneke yogwirizana ndi `(VHL)` ndi iyi:

• Retinal hemangioblastoma: Iyi ndi mtundu wa chotupa chomwe chimayamba m'diso. Chingayambitsenso kulephera kuona bwino. Kafukufuku akusonyeza kuti chimayamba mwa anthu 60% omwe ali ndi (VHL).
• Ma hemangioblastoma a tsinde la ubongo ndi cerebellar: Awa ndi zotupa mu ubongo. Zingakhudze momwe mumakhalira bwino komanso zimayambitsa mavuto ena. Zimakhudza pakati pa 13% ndi 72% ya anthu omwe ali ndi VHL.
• Hemangioblastoma ya msana: Mtundu uwu wa hemangioblastoma umapezeka pakati pa 13% ndi 50% ya anthu omwe ali ndi (VHL).

Kuphatikiza apo, ngati muli ndi VHL, muli pachiwopsezo chokhala ndi zotupa ndi ma cysts omwe si a khansa monga:

• Epididymal cystadenomas: Izi ndi zotupa zomwe zimakhudza amuna. Zimakula mu epididymis, kapangidwe kakang'ono konga chubu komwe kamasunga umuna, pafupi ndi machende. Zimapezeka pakati pa 25% ndi 60% ya amuna omwe ali ndi VHL.
• Matenda a m'thumba la m'mimba otchedwa Endolymphatic sac tumors (ELST): Izi ndi zotupa zomwe sizipezeka kawirikawiri. Ngati muli ndi VHL, zimatha kufalikira mkati mwa khutu. Matendawa amakhudza anthu pakati pa 10% ndi 25% omwe ali ndi VHL.
• Ma cysts: Awa ndi ma cysts odzaza ndi madzi. Anthu omwe ali ndi VHL amatha kukhala ndi ma cysts awa mu impso ndi kapamba.

Kodi matenda a VHL ndi ofala bwanji?

Vutoli ndi losowa kwambiri. Limakhudza munthu m'modzi pa anthu 36,000 aliwonse. Anthu ambiri omwe ali ndi VHL amayamba kusonyeza zizindikiro ali ndi zaka zapakati pa 20. Izi zikutanthauza kuti muyenera kusamala ndi vutoli ali aang'ono.

Kodi zizindikiro za matenda a von Hippel-Lindau ndi ziti?

Popeza vutoli lingayambitse zotupa m'thupi lonse, zizindikiro zimatha kusiyana. Zimatengera komwe zotupazo zili komanso kukula kwa zotupazo. Mutha kukumana ndi zizindikiro monga izi:

• Kupweteka mutu pafupipafupi.
• Kutaya kumva kapena kulira kosalekeza m'makutu (tinnitus).
• Kuthamanga kwa magazi.
• Kutaya mphamvu mukuyenda.
• Kuchepa kwa mphamvu ya minofu kapena kuvutika kugwirizanitsa mayendedwe.
• Mavuto a masomphenya.
• Kusanza.

Tangoganizirani, kodi tikadakhala ndi nkhawa bwanji titakhala ndi chimodzi kapena ziwiri mwa zizindikirozi? Choncho ngati muli ndi zizindikiro zingapo nthawi imodzi, ndikofunikira kwambiri kufunafuna upangiri wa dokotala.

Kodi n’chiyani chimayambitsa matenda a VHL?

Von Hippel-Lindau syndrome ndi matenda obadwa nawo . Amayamba mukalandira kopi yachilendo ya jini yoletsa chotupa yotchedwa VHL kuchokera kwa m'modzi mwa makolo anu.

Mwachidule, "majini oletsa chotupa" awa amaletsa maselo kugawikana mwachangu kwambiri, zomwe zingayambitse khansa. Zili ngati mabuleki a galimoto. Koma ngati majini awa asinthidwa, zili ngati kuchotsa mabuleki ndikuyika accelerator. Kukula kwa maselo kumafulumira kwambiri.

Matendawa amafalikira mu njira yopezera cholowa cha autosomal dominant. Izi zikutanthauza kuti ngati m'modzi mwa makolo anu ali ndi jini ya VHL yosazolowereka, muli ndi mwayi wa 50% wolandira ndikupeza matenda a von Hippel-Lindau. Komabe, kafukufuku wasonyeza kuti pafupifupi 10% ya anthu omwe ali ndi VHL alibe mbiri ya m'banja lawo ya matendawa. Izi zikutanthauza kuti kusintha kwa majini atsopano kungachitikenso.

Kodi matenda a VHL amapezedwa bwanji?

Madokotala angaganize kuti muli ndi VHL ngati muli ndi zizindikiro za vuto lomwe limayambitsidwa ndi VHL, monga hemangioblastoma kapena clear cell renal carcinoma. Koma njira yokhayo yotsimikizira izi ndi kudzera mu mayeso a majini. Ngati wina m'banja mwanu ali ndi matenda a Von Hippel-Lindau, ndikofunikira kufunsa dokotala wanu za mayeso a majini anu.

Kodi mankhwala a matenda a VHL ndi otani?

Njira zochiritsira zimasiyana malinga ndi mtundu wa chotupa cha VHL kapena chotupa. Dokotala wanu adzakufotokozerani njira zochiritsira zomwe zili zoyenera kwa inu. Mankhwala odziwika kwambiri ndi awa:

• Opaleshoni yochotsa chotupacho.
• Mankhwala a chemotherapy.
• Chithandizo cha radiation.
• Chithandizo cholunjika chimaphatikizapo zinthu monga peptide receptor radionuclide therapy (PPRT) ndi tyrosine kinase inhibitors (TKI).
• Chithandizo cha chitetezo chamthupi.
• Chithandizo cha mahomoni.

Chofunika kwambiri ndikupeza matendawa msanga ndikuyamba kulandira chithandizo choyenera. Mukatero ndiye kuti mutha kupeza zotsatira zabwino kwambiri.

Kodi matenda a VHL angachiritsidwe kwathunthu?

Tsoka ilo, pakadali pano palibe mankhwala a matenda a von Hippel-Lindau. Cholinga chachikulu cha chithandizo ndikupeza ndikuchotsa zotupa mwachangu momwe zingathere. Dokotala wanu angakulangizeni opaleshoni yochotsa zotupa pamodzi ndi mankhwala ena.

Ngati ndili ndi VHL, ndiyenera kuyembekezera chiyani?

Ngati muli ndi vutoli, muyenera kuyesedwa pafupipafupi kuti muwone ngati muli ndi zizindikiro za matenda enaake. Kuzindikira msanga ndi kuchiza zotupa kungathandize kuchepetsa zotsatira zomwe matenda a von Hippel-Lindau amakhudza pa moyo wanu. Ndicho chifukwa chake ndikofunikira kupeza mayeso omwe dokotala wanu akukulangizani.

Kodi matenda a VHL angapewedwe?

Ayi, sizingatheke kupewedwa. Izi zili choncho chifukwa matenda a von Hippel-Lindau amayamba chifukwa cha kusintha kwa majini komwe kumapatsirana kuchokera kwa kholo limodzi kupita kwa lina. Ngati wina m'banja mwanu ali ndi VHL, kuyezetsa majini kungakuthandizeni kudziwa ngati inunso muli ndi kusintha kwa majini.

Ngakhale simungathe kupewa matenda a von Hippel-Lindau, ndikofunikira kudziwa chiopsezo chanu ndikumvetsetsa momwe `(VHL)` ingakukhudzireni. Ngati mukudziwa kuti muli pachiwopsezo, madokotala amatha kuyang'anira zizindikiro za zotupa zokhudzana ndi `(VHL)` ndikuchitapo kanthu kuti azichotse msanga.

Ngati muli ndi jini (VHL) ndipo mukukonzekera kukhala ndi mwana, ndi bwino kukumana ndi mlangizi wa majini kuti mukambirane za chiopsezo cha mwana wanu cholowa jini.

Kodi pali mayeso apadera owunikira matenda okhudzana ndi VHL?

Palibe malangizo enieni oyezera matendawa, monga momwe zilili ndi mitundu ina ya khansa (monga kuyeza khansa komwe kumalimbikitsidwa ndi US Preventive Services Task Force). Komabe, ngati kuyeza majini kukutsimikizirani kuti muli ndi jini ya VHL yosazolowereka, gulu lanu lazaumoyo lingakulangizeni mayeso ena kuti akuthandizeni kuzindikira mavuto msanga. Mwachitsanzo, angakulangizeni kuti muyesedwe ndi m'mimba ndi magnetic resonance imaging scan (MRI) zaka ziwiri zilizonse kuti muwone ngati muli ndi khansa yokhudzana ndi VHL, monga clear cell renal carcinoma.

Kodi ndingathe bwanji kudzisamalira ndekha? Kodi ndingachite chiyani?

Kukhala ndi VHL kumatanthauza kukhala ndi moyo wosatsimikizika. Ngati mwalandira kusintha kwa majini komwe kumayambitsa VHL, muli ndi mwayi wa 50% wopeza vutoli. Ngati mutatero, muli ndi mwayi wa 97% wopeza mitundu ina ya khansa ndi matenda ena akuluakulu.

Komabe, palibe amene anganeneretu momwe `(VHL)` idzakhudzira moyo wanu. Nazi zinthu zina zomwe zingakuthandizeni kuthana ndi mavuto awa:

• Pezani chithandizo cha thanzi la maganizo: Kafukufuku wina wasonyeza kuti kupezeka ndi VHL kumatha kukhudza maganizo, monga nkhawa komanso mantha. Chifukwa chake, ndikofunikira kwambiri kulankhula ndi dokotala wa matenda amisala kapena mlangizi.
• Tetezani thanzi lanu lonse: Idyani zakudya zopatsa thanzi - nyama zambiri zopanda mafuta ambiri, tirigu wonse, masamba obiriwira, ndi zipatso. Kuchita masewera olimbitsa thupi (zomwe zingachepetsenso nkhawa). Funsani gulu lanu lachipatala za katemera woyenera kwa inu.

Kodi ndiyenera kupeza upangiri wa dokotala liti?

Ngati mukukumana ndi zizindikiro ngati izi, muyenera kuyimbira dokotala nthawi yomweyo:

• Kusintha kwa masomphenya kapena kumva kwanu.
• Kupweteka mutu pafupipafupi.
• Kusanza kapena kusanza komwe sikunachitike chifukwa cha matenda ena.
• Kuwonjezeka kwadzidzidzi kwa kuthamanga kwa magazi.
• Ngati mwadzidzidzi mukuvutika kuyenda, kukhalabe ndi thanzi labwino, kapena kugwirizanitsa mayendedwe anu.

Ndi mafunso ati omwe ndiyenera kufunsa dokotala wanga?

Tangoganizani kuti mwauzidwa kuti winawake m'banja mwanu ali ndi matenda a von Hippel-Lindau. Ngati ndi choncho, nayi mafunso ena omwe mungafunse dokotala wanu:

• Kodi zotsatira za mayeso anga a majini zikuti chiyani?
• Kodi banja langa lonse liyenera kukayezetsa majini?
• Kodi ndiyenera kuyezetsa kangati kuti ndizindikire zizindikiro zoyambirira za matenda omwe angagwirizane ndi `(VHL)`?

Pomaliza, zinthu zoti muzikumbukira (Uthenga Wopita Nawo Kunyumba)

Matenda a Von Hippel-Lindau (VHL) ndi matenda osowa kwambiri omwe amapezeka m'majini. Amakhudza munthu m'modzi mwa anthu 36,000. Choncho, kudziwa kuti ndinu m'modzi mwa anthu 36,000 amenewo, kungakhale kodabwitsa kwambiri, makamaka ngati palibe aliyense m'banja mwanu amene adadwalapo matendawa.

Ngati kuyezetsa majini kukutsimikizirani kuti muli ndi (VHL), dzipatseni nthawi yoti muganizire nkhaniyo. Kenako, tengani nthawi kuti mumvetse tanthauzo la matenda anu. Mwachitsanzo, mungakhale ndi mafunso okhudza chiopsezo chanu chotenga mitundu yosiyanasiyana ya khansa ndi zotupa, kapena momwe matendawa angakhudzire ena m'banja mwanu. Musazengereze kufunsa gulu lanu lachipatala za zomwe mungayembekezere. Ndipo musaiwale kupempha thandizo kuti muthane ndi nkhawa zomwe zimabwera chifukwa chokhala ndi (VHL). Simuli nokha, ndipo pali anthu ambiri omwe angakuthandizeni paulendowu.

Von Hippel-Lindau, VHL, matenda a majini, khansa, zotupa, zizindikiro, chithandizo