Kodi matenda Von Hippel-Lindau?

matenda, tikambirana kenako m'nkhani, zambiri zimachitika gulu la matenda chibadwa chathu. Mbali yake yaikulu ili mapangidwe zotupa angapo mu thupi lachokera ku zimakhala ziwalo zosiyanasiyana. Thupi lotani adzakhala opweteka kwambiri, zimatengera, zinadziwika syndrome Von Hippel-Lindau.

Komabe, nkhani kudziwika yokhudza odwala eyiti, wochokera ku banja mofanana chizindikiro chinali matenda polycystic impso. Matenda Pankhaniyi lakhazikitsidwa ntchito chizindikiritso cha chilema majini (masinthidwe).

matenda Von Hippel-Lindau chokhudzana ndi matenda osowa, ndipo pafupipafupi pakuwonekera kwake - chitsanzo chimodzi pa kubadwa 36,000.

Limagwirira chitukuko matenda

Maziko Von Hippel Lindau matenda ndi cholowa chikhalidwe zowombetsa mkota matenda yodziwika ndi kuchuluka kwambiri nsalu capillary ndi maonekedwe chifukwa ambiri zotupa neoplastic.

matenda imafalikira ku m'badwo wotsatira mu autosomal unsembe lalikulu, mwachitsanzo, mu kukhalapo kwa losokonekera kabisidwe ndi kudwala, kholo limodzi, Mwina odwala mbewu ndi 50%.

Chilema (masinthidwe) zotsatira yimitsira la jini, amene ndi suppressor kukula chotupa.

Mawonetseredwe - njira khansa

Mawonetseredwe waukulu Von Hippel Lindau syndrome - pituitary zotupa, cerebellum, msana, diso, ziwalo. List of neoplasms zotheka:

• Hemangioblastoma - ndi kutanthauzira mu cerebellum ndi diso; zichokera kwa minofu mantha. CNS neoplasms ndi pafupipafupi yatsoka matenda a odwala ndi Von Hippel-Lindau osachepera 60%. Avereji odwala amene apeza mtundu uwu chapakati dongosolo zotupa mantha, zaka 42.
• Angioma - ndi neoplasm chiyambi mtima, ndi kutanthauzira mu chingwe msana.
• Cysts. neoplasm izi ndi mwa M'mimbamo wodzazidwa ndi madzi. zinthu zoterozo zikhoza kupangidwa mu chiwindi, impso, testis ndi kapamba. Mwachindunji, impso ndi pancreatic cysts zimapezeka 33-54% ya milandu.
• Pheochromocytoma - chotupa anachokera ku maselo adrenal medulla. Zochitika - za 7% ya anthu odwala. Avereji odwala ndi pheochromocytoma - zaka 25.

• Cell carcinoma. Makamaka, zaka pafupifupi milandu ndi chotupa ichi - zaka 43.
• Mu amuna ndi matenda Von Hippel-Lindau angathe neoplasms chosaopsa chilengedwe machende (mwachitsanzo, papillary cystadenoma).
• Akazi aona chotupa chotupa cha Mitsemphayi uterine.

I imene matenda ndi

The matenda a "matenda Von Hippel-Lindau" akhoza aziwapatsa vuto la kudziwika mmodzi wa anapereka Mu mndandanda uli mmunsiwu zizindikiro. Koma n'zotheka ngati muli ndi mbiri ya mmodzi wa anthu a m'banja chapakati mantha dongosolo hemangioblastoma wodwalayo, retinal hemangioblastoma, aimpso enaake formations kapena adenocarcinoma:

• hemangioblastoma ndi kutanthauzira mu minofu cerebellar komanso mbali zina za chapakati mantha dongosolo;
• hemangioblastoma wa chingwe msana;
• hemangioblastoma ndi kutanthauzira mu m'mafupa;
• hemangioblastoma pa diso;
• enaake aimpso minofu kuwonongeka;
• aimpso adenocarcinoma;
• pancreatic cysts;
• tsistadenokartsinoma;
• pheochromocytoma;
• epididymal adenoma.

Tikumbukenso kuti zachipatala amene mamembala zikugwira vuto la matenda Von Hippel-Lindau - ubongo, pochiza matenda a maso urology, mankhwala mkati.

CNS hemangioblastoma

Zizindikiro kaŵirikaŵiri lipoti syndrome Von Hippel-Lindau hemangioblastoma ndi chitukuko cha cerebellum ndi, msana, kapena diso. Kodi pa cerebellum chotupa ali ndi zizindikiro zotsatirazi:

• Mutu Expander chikhalidwe.
• Nseru, kusanza.
• Kukayikakayika kuyenda, chifukwa kugwirizana chakhungu.
• Chizungulire.
• Kuwonongeka kwa chikumbumtima (khalidwe la magawo ina ya matenda).

Pamene hemangioblastoma kutanthauzira mu chingwe msana, zizindikiro zazikulu matenda ndi kuchepa tilinazo, paresis ndi ziwalo, matenda micturition ndi ndondomeko defecation. Ululu syndrome amatchuka ndi apo.

Mtundu uwu wa chotupa ali wa pang'onopang'ono patsogolo. A nkhani kwambiri kwa matenda ndi ulamuliro njira loyang'anira anazindikira, Maginito kumveka kulingalira (Kujambula) Tayerekezani chitsanzo gawo umalimba.

Njira womuzunza hemangioblastoma cerebellum lero opaleshoni kuchotsa. The ntchito cheza ndi njira zachipatala zotsatira wosatsutsika zabwino simungathe kuziona.

Pambuyo microsurgical kuchotsa hemangioblastoma, monga ulamuliro, zilibe anawathetsa akuyambiranso, koma angaoneke mu zotupa zina. Mankhwalawa opaleshoni ya chotupa n'kofunika kuganizira kuchuluka kwake, lomwe ndi khalidwe la matenda Von Hippel-Lindau.

retinal angiomatosis

Pakuti ophthalmic kutanthauzira kwa pathological ndondomeko khalidwe ndi maonekedwe wa ku:

• kupezeka kwa angioma;
• vasodilation mu fundus ya;
• pamaso pa subretinal exudation (kudzikundikira madzimadzi - kutukusira kwa mankhwala - pansi diso la), yomwe INGATHE kuyambitsa chitukuko cha exudative retinal detachment mu gawo zapamwamba za matendawa.

Pakuti magawo koyamba matenda ambiri ndichepe mtundu mu fundus ndi kuchuluka tortuosity Mitsempha. Pamene mbamuikha pa mwana wa diso ndi ikusonyezedwa pulsation ya mitsempha ndi mitsempha.

Mu masiteji ina ya vasodilatation ndi mtima tortuosity patsogolo, mapangidwe aneurysms ndi angiomas, ndi chosiyana tione wozungulira glomeruli - ichi ndi chizindikiro yeniyeni matenda Von Hippel-Lindau.

pheochromocytoma

Ngati amakhudza zopangitsa adrenal, matenda Von Hippel-Lindau ndi owonetseredwa mawonekedwe pheochromocytoma. neoplasm Izi zikuchitika zinthu za medulla adrenal zambiri chosaopsa m'chilengedwe. Nthawi zambiri anapezeka pa zaka pafupifupi zaka 30 ndipo amakhala ndi ziwiri amaganiza, mayunitsi angapo ndi luso amasamukira kuti zimakhala moyandikana.

Pheochromocytoma anawonetsera zinthu zotsatirazi:

• Kugonjetsedwa ndi mankhwala antihypertensive, wolumikizira oopsa.
• Kugwirizana ndi magazi cranialgia.
• khungu wotumbululuka.
• Chizolowezi hyperhidrosis.
• Tachycardia.

Khansa ya kapamba kugonjetsedwa

Pancreatic khansa kudwala variable: zotupa akhoza kukhala mwina chosaopsa kapena zilonda chikhalidwe. A dongosolo malinga kapena cysts kapena neuroendocrine chotupa.

njira zosiyanasiyana khansa zokhudza kapamba zimapezeka theka la odwala matenda Von Hippel-Lindau. Zizindikiro kugwirizana ndi, zambiri ndi ntchito mkhutu pancreatic.

Avereji odwala pa nthawi ya kudziwika zotupa wa zaka 33-35. Pankhani ya zilonda ndondomeko ndondomeko metastatic imatsogoleredwa chiwindi.

Endolymphatic sac chotupa

Ikusintha ndi matenda dzina lake, ndondomeko oncologic nthawi zonse ali ndi mtima wabwino Inde ndi akuonetsedwa ngati motere:

• Kumva matenda, mpaka imfa okwana kumva.
• Chizungulire.
• Diskoordinatornye kuyan'anila.
• Tinnitus.
• Paresis minofu nkhope.

diagnostics

Matenda kuyambirira ikuchitika pamaziko a Kupenda ambiri ndi ophthalmologic wodwalayo, anamnesis, kuphatikizapo banja cholowa.

nkhani kwambiri ndi kuphunzira magazi, kudziwa wokhutira a shuga, ndi makatekolamaini.

Kuona a zotupa ayenera kuchitidwa ntchito chapamwamba kwambiri (Kujambula).

Amawatenga lofunika kuzindikira mwamsanga matenda diso ndi fulorosenti angiography. Njira imeneyi matenda facilitates chizindikiritso cha mawonetseredwe koyamba matenda mu fundus m'magazi amene sanalembedwe pa ophthalmoscopy (monga telangiectasia, utangoyamba ziwiya). Pankhani ya kupitirira za ndondomeko pathological, njira imeneyi imasonyeza chotengera kudyetsa chotupa kale kwambiri kuposa ophthalmoscopy.

Malizitsani tinafotokoza mwatsatanetsatane tso zotsatirazi njira yodziwira:

1. Kompyuta ndi kumveka maginito tomography. Pogwiritsa ntchito maluso awa ali kwambiri akuchulukirachulukira anapezeka mu magawo oyambirira a matenda. Ndipo mu nkhani iyi, iwo zimakhala zovuta kuchiza.
2. Ultrasound kulingalira.
3. Angiography ndi nkhani zofotokoza cholinga zimene zikuchitika zimene limba chotupa.
4. Pneumoencephalography.

Nkofunika kudziwa kuti vuto la kudziwika chotupa limodzi la matupi, m'pofunika kuti atsogolere kufufuza zonse kuti achotse zina kudwala.

chithandizo

likuyandikira Common mankhwalawa wa syndrome Von Hippel-Lindau ndi mfundo za mankhwala khansa. Therapy ayenera Integrated ndi ophatikizana opaleshoni ndi cheza njira. Aliyense wa iwo ntchito bukuli motsata zikuonetsa kuti, contraindications, ndi mbali yapadera ya chotupa mtima.

Mankhwala ntchito makamaka ngati mankhwala symptomatic kuti awongolera ntchito zina za matupi munthu ndi chikhalidwe ambiri wodwala.

kaonedwe

Ndi matenda yake ndi molondola anasankha chithandizo, Kukula kwa matendawa, komanso zotsatira zake zoipa pa thupi amalephera kuzilamulira. Kuvomerezedwa chofunika - matenda ndi mwambo wa mankhwala mu magawo oyambirira.

Komabe, nthawi zina, ngakhale achitapo, kuti madokotala ananena zawo amakhalabe osauka wodwalayo chifukwa cha chiopsezo zosokonezeka.