Sidee cudurka Von Hippel-Lindau?

Cudurku wuxuu, kaas oo looga hadli doonaa goor dambe oo maqaalka, sida caadiga ah loo aaneeyey inay koox ka mid ah oo hiddo adag. Its feature ugu muhiimsan waa formation of burooyinka badan ee jirka ku jira oo ka soo jeeda ee unugyada xubnaha kala duwan. Oo jidh caynkee ah lagu dhufan doono adag, waxay ku xiran tahay, sida muujiyey von Hippel-Lindau syndrome.

Si kastaba ha ahaatee, kiiska loo yaqaan ku lug leh siddeed bukaan ah, oo laga keenay isku qoys ah sida calaamad oo kaliya ahaa cudur kalyaha polycystic. Waxaa cudurka kiiskan ayaa la sameeyey iyadoo la isticmaalayo aqoonsiga of iin hidaha (isbedel).

cudurka Von Hippel-Lindau la xiriira cudurka dhif ah, iyo inta jeer ee ay dhacdo - hal kiis halkii 36,000 dhalmada.

farsamo ee horumarka cudurka

Cudurku wuxuu ku salaysan von Hippel Lindau waa nooca cudurka guud dhaxlo lagu gartaa kororka xad-dhaafka ah maro gaddoonka iyo muuqaalka ay sabab u tahay tiro badan oo ah burooyinka neoplastic.

cudurka waxaa soo gudbiyo si jiilka soo socda ee autosomal si adag, taas macnaheedu waa in ay goobjoog ka ahaayeen hidda habaynteeda cudurada ah, mid ka mid ah waalidiinta, jaaniska of farcankaaga silica waa 50%.

iin The (isbedel) natiijooyinka xakamaynta of hiddo, taas oo suppressor ah ee koritaanka buro.

Eebbe - geeddi socodka kansarka

The daliilka ugu weyn ee von Hippel Lindau syndrome - burooyin pituitary, cerebellum, laf-dhabarta, ileyska, xubnaha gudaha. List of neoplasms suurto gal ah:

• Hemangioblastoma - leedahay deegaamaynta ee cerebellum iyo retina; oo ka soo jeeda unugyada neerfaha. neoplasms habdhiska leeyihiin inta jeer ah dhacdo cudurka bukaanka qaba von Hippel-Lindau ugu yaraan 60%. Celceliska da'da bukaanka helay noocan oo kale ah burooyinka habdhiska dhexe, 42 sano jir ah.
• Angioma - neoplasm ah oo asal ahaan vascular, la deegaamaynta ee laf dhabarta.
• Fiix. neoplasm Tani isagoo gudaha saabka ah oo dareere. xubno noocan oo kale ah la abuuri karaa in beerka, kelyaha, testis iyo beeryarada. Gaar ahaan, kelyaha iyo ganaca fiix waxaa la helay in 33-54% ee kiisaska.
• Pheochromocytoma - buro ka soo jeeda unugyada medulla adrenal. Dhacdooyinka - oo ku saabsan 7% bukaanka. Celceliska da'da bukaanka qaba pheochromocytoma - oo ku saabsan 25 sano.

• carcinoma Cell. Gaar ahaan, celceliska da'da kiisaska la buro this - 43 sano.
• In ragga qaba cudurka Von Hippel-Lindau horumarin karaan neoplasms nooca aan xumayn ee xiniinyaha (tusaale ahaan, cystadenoma papillary).
• Haweenka helay nabar buro seedaha makaanka.

Shuruudaha by kaas oo cudurka waa

ogaanshaha ah "cudurka von Hippel-Lindau" la bixisey Karin haddii la ogaado oo ugu yaraan mid ka mid ah lagu soo bandhigay Liiska soo socda calaamadaha. Laakiin waxaa suurto gal ah haddii aad leedahay taariikh ah oo ugu yaraan mid ka mid ah xubnaha qoyska ee hemangioblastoma bukaanka dhexe ee habdhiska, hemangioblastoma isha, qaababka fibrosis kelyaha ama adenocarcinoma ah:

• hemangioblastoma isagoo deegaamaynta in unugyada cerebellar iyo sidoo kale qaybaha kale ee habka dhexe ee neerfayaasha,
• hemangioblastoma ee laf-dhabarta,
• hemangioblastoma la deegaamaynta dhuuxa lafta,
• hemangioblastoma on ileyska;
• dhaawac jirka kelyaha fibrosis;
• adenocarcinoma kelyaha,
• fiix ganaca,
• tsistadenokartsinoma;
• pheochromocytoma;
• adenoma epididymal.

Waa in la ogaadaa in xirfadda caafimaadka kuwaas oo xubnaha ku hawlan yihiin dhibaatada syndrome von Hippel-Lindau - neurolojiga, indhaha, urology, daawada gudaha.

habdhiska hemangioblastoma

Inta badan la sheegay calaamadaha syndrome von Hippel-Lindau hemangioblastoma waa horumarinta cerebellum ah, laf-dhabarta, ama ileyska. Ma on buro cerebellum ayaa calaamadaha soo socda:

• Madax Expander dabiiciga ah.
• Lallabo, matag.
• Lama hubo in socodka, ay sabab u tahay isuduwidda si daciif ah.
• Dawakh.
• Dhimantahay miyir (caan ku tahay marxaladaha dambe ee cudurka).

Marka deegaamaynta hemangioblastoma ee laf-dhabarta, calaamadaha ugu muhiimsan ee lagu daawayn karo waa hoos u dhac ku dareen, paresis oo curyaannimo, xanuunada muqayiliinta iyo nidaamka ku saxaroodaan. Xanuun syndrome waa xusay mar mar kaliya.

Noocan ah buro iska leh a si tartiib tartiib. A ugu wargelin ah habka cudurka iyo xakameynta kormeerka la aqoonsan yahay, sawirka resonance magnetic (MRI), iska qayb muunad wanaajiyey.

Sida kaliya ee wax ku ool ah si loo daaweeyo cerebellum hemangioblastoma maanta waa ka saarida qalliinka. Isticmaalka shucaac iyo hababka caafimaad ee natiijo wanaagsan qanci aan la tusay.

Ka dib markii hemangioblastoma saarista microsurgical, sida caadiga ah, waxa aanu soo noqnoqon, laakiin u muuqan kara in burooyinka kale. In daaweynta qalliin ay buradu ku lagama maarmaan ah inay tixgeliyaan ay hannaanka, oo waa dabeecadda cudurka Von Hippel-Lindau.

angiomatosis isha

Waayo, deegaamaynta indhaha of sifo nidaamka bahal waa sifooyinka triad:

• helitaanka angioma;
• vasodilation in fundus ah;
• joogitaanka maadoyinka subretinal (raasamaal ah ee dareeraha - barar ah sheyga - hoos wilka), taasoo keeni karta horumarinta go'idda exudative yada sare ee cudurka.

Waayo, marxaladaha hore ee cudurka sida caadiga ah hoos midabka ee fundus ah, kor u tortuosity marinnada dhiigga. Marka cadaadisay on bu'da iyo sida calaamad oo pulsation oo ka mid ah halbowlayaasha iyo xididada.

In marxaladaha ugu dambeeya ee ku vasodilatation iyo horumarka tortuosity vascular, formation of aneurysms iyo angiomas, waxay leeyihiin a glomeruli wareega eegno kala - taasi waa calaamad gaar ah oo xanuun Von Hippel-Lindau.

pheochromocytoma

Haddii ay saamayn ku qanjidhada adrenal, cudurka von Hippel-Lindau waa nooc muujiyey of pheochromocytoma. neoplasm Tani dhaca walxaha of medulla ee adrenal inta badan waa benign ee dabiiciga ah. Xaaladaha intooda badan laga helay at da'da 30 sano iyo waxa lagu gartaa laba dhinac, unugyo badan oo awood u leh inay u haajiraan si ay unugyada ku xeeran.

Pheochromocytoma muujiyey sifooyinka soo socda:

• Adkaysi u therapy antihypertensive, hypertension halbowlaha.
• La xiriira cranialgia cadaadiska dhiigga.
• jirka oo caddaado.
• Ujeedo in uu hyperhidrosis.
• Tachycardia.

Cancer of guuldaradii ganaca

Ganaca variable cudurada kansarka: burooyinka noqon karaa nooca aan xumayn ama malignant. Qaab dhismeedka A sida uu sheegay mid ka mid fiix ama buro neuroendocrine.

hababka kala duwan ee kansarka saameeya beeryarada, waxaa la helay in kala badh dadka qaba cudurka Von Hippel-Lindau. Astaamaha la xidhiidha, guud ahaan la shaqayn ganaca oo yaraada.

Celceliska da'da bukaanka waqtiga la ogaado burooyinka of 33-35 sano. In the case of habka malignant habka xadka- la faray in beerka.

buro koleyga Endolymphatic

Guuxayay la syndrome la odhan jiray, habka oncologic had iyo jeer waxay leedahay kooras benign iyo waxa muujiyay sida soo socota:

• xanuunada Maqalka, ilaa khasaare guud ee maqalka.
• Dawakh.
• xadgudubyada Diskoordinatornye.
• Tinnitus.
• Paresis oo ka mid ah muruqyada wejiga.

Baadhista xanuunada

Waxaa cudurka hordhaca ah waxaa lagu fuliyaa ku salaysan baaritaan guud oo ophthalmologic bukaanka, anamnesis ah, oo ay ku jiraan qoyska la iska dhaxlo.

Aad wargelin ah waa waxbarasho dhiigga, si ay u aqoonsadaan ay ka kooban of glucose, iyo catecholamines.

Muuqaalaynta of burooyinka waa in la sameeyaa iyadoo la isticmaalayo imaging resonance magnetic (MRI).

Isha of warbixin qiimo leh, waayo, goor hore la ogaado cudurka indhaha waa ANGIOGRAFI dhaadheer. Farsamadan ogaanshaha fududeeyaa aqoonsiga ee daliilka hore ee cudur ee fundus ee dhiigga, taas oo aan la diiwaan inta lagu jiro ophthalmoscopy (sida telangiectasia, weelasha dhowaan la sameeyey). In the case of horumarka geedi socodka bahal, farsamadaa waxa ay shaaca ka qaaday weel quudinta burada ka badan sidii hore ka badan ophthalmoscopy.

Buuxi baaritaan dhamaystiran oo ka kooban oo ka mid ah hababka ogaanshaha soo socda:

1. Computer iyo raajito loogu resonance magnetic. Iyada oo la adeegsanayo hababkan ayaa si weyn u kordhay tirada cudurka ku jira marxaladaha hore ee cudurka. Oo ay dhacdo in this, waxay inta badan waa adag tahay in la daweeyo.
2. Ultrasound imaging.
3. ANGIOGRAFI waa il macluumaad Ujeedada ku saabsan waxa dhacaya waxa xubin burada.
4. Pneumoencephalography.

Waxaa muhiim ah in la ogaado in ay dhacdo in la ogaado burada in mid ka mid ah meydadka, waxaa lagama maarmaan ah si uu u hoggaamiyo baaritaan buuxa si looga saaro goobaha kale ee cudurada.

daaweynta

habab caadi ah in daaweyn jiray syndrome ee von Hippel-Lindau ay la socotaa mabaadi'da therapy kansarka. Therapy waa in lagu daro oo isku daro farsamooyinka qalliinka iyo shucaaca. Mid kasta oo iyaga ka mid ah waxaa loo isticmaalaa si adag iyadoo la raacayo muujinaya, sababo, iyo muuqaalada bukaanka burada gaar ah.

Daawooyinka loo isticmaalo inta badan sida therapy ah astaamaha iyo hagaajinta ee hawlaha qaar ka mid ah meydadka qofka iyo xaalada guud ee bukaan-socodka.

Odoroska

Iyada oo ogaanshaha waqtiga iyo daaweyn si sax ah loo doortay, horumarka cudurka, iyo sidoo kale saamaynta ay xun ee jirka oo aan awoodin in la xakameeyo. looga baahan yahay qasabka ah - baaritaan iyo bilaabidda daawada ku jira marxaladaha hore.

Si kastaba ha ahaatee, mararka qaarkood, inkastoo dadaalka sameeyey, saadaasha weli masaakiinta bukaanka sababtoo ah khatarta sare ee dhibaatooyinka.