Pehea ke ano o ka maʻi av Hippel-Lindau?

I ka maʻi, a e e ana no hope i loko o ka 'atikala, ana wahi a hiki i ka pae o ka luna' aaiaoe kāna hana. Kona papa kuhikuhiE hiʻona o ke kahua o ka mau hikoko i loko o ke kino loko mai o nā nā'aʻaʻa o nā nā loko. He aha ke ano o ke kino, e ke pa a ke ku ka paʻakikī, e hilinaʻi nui, i hoikeia mai av Hippel-Lindau maʻi pale.

Naʻe, i ka ike hihia 'ole i ewalu nā mea maʻi, ka hoʻomakaʻana, mai ka ia ohana, e like me ka mea wale symptom ua polycystic hakuʻala maʻi. Keʻike, i loko o keia hihia i ua i ka hoʻohana 'ana i ka' ike o ka aaiaoe kina (mutation).

Av Hippel-Lindau maʻi ua pili i ka ke nānā mai, a me ke alapine (frequency) o kona hanana - kekahi hihia no 36.000 hanau.

Keʻano papa hana o ka maʻi ulu

Ke kumu av Hippel Lindau maʻi mea hereditaryʻano generalized kāna hana wehewehe aku ma excessive ka hoʻonuiʻana o ka capillary ka lole, a me ka helehelena e like me ka hopena he nui helu o neoplastic hikoko.

I ka maʻi ua pū i hiki i ka aʻe hanauna i loko o ka autosomal Hoʻohaku ano, oa ma ke alo o ka'ōewe hoʻopā'ālua ka pathology, kekahi o na makua, i ka pahiki o ka morbidity keiki kakou, he 50%.

Ke kina (mutation) i nā hualoaʻa ma ka suppression o ka'ōewe, i mea he suppressor o kaʻeho ka ulu ana.

Hoikeana - Ka maʻi 'aʻai keʻano o ka hanaʻana

I ka papa kuhikuhiE hoikeana o av Hippel Lindau maʻi pale - pituitary hikoko, cerebellum, spinalʻaha, retina, na nā loko. Papa Inoa o ka hiki neoplasms:

• Hemangioblastoma - i IIeEeAIEUAA i loko o ka cerebellum a me ka retina; loko mai o ko mākou hopohopo'aʻaʻa. CNS neoplasms i ke alapine (frequency) o ka hanana o ka maʻi i loko o nā mea maʻi me av Hippel-Lindau ma ka liʻiliʻi 60%. Ka awelika makahiki o ka hoʻomanawanui iki mai ka poe i loaʻa i kēiaʻano o ka Central ko mākou hopohopo'ōnaehana hikoko, 42 makahiki.
• Angioma - he neoplasm o vascular ka molekumu o, me ka IIeEeAIEUAA i loko o ka spinal kaula.
• Cysts. Kēia neoplasm loaa i loko o ka lua piha me ka wai. Na oihana mua hiki ke hana ia ma ke akepaʻa, hakuʻala, testis a me ka pancreas. Ua hōʻike hewa, i loaʻa nā konāhua, a pancreatic cysts i loko o 33-54% o ka hihia.
• Pheochromocytoma -ʻeho loko mai o ka adrenal medulla keena. Mea hiki wale mai - e pili ana i 7% o ka hoʻomanawanui iki mai. Ka awelika makahiki o ka hoʻomanawanui iki mai me ka pheochromocytoma - ma kahi o 25 makahiki.

• Cell carcinoma. Ma 'ana, ka awelika makahiki o nā hihia me keiaʻeho - 43 makahiki.
• I na kanaka a me ka maʻi av Hippel-Lindau ke hoʻomōhala neoplasms benign ano i loko o ka hoʻi (e like, papillary cystadenoma).
• Women Ua loaʻaʻeho lesion o uterine ligaments.

Nā ana hoʻohālike e i kaʻike, ka

I kaʻike, o ka "maʻi av Hippel-Lindau" hiki e lako i ka hihia o ka loaʻaʻana o ka ma ka liʻiliʻi loa kekahi o ka hōʻike Ma ke kēia papa inoa o symptoms. Akā, he mea hiki ina oe i ka mōʻaukala o ma ka liʻiliʻi loa kekahi o na lala ohana o ka hoʻomanawanui ka Central ko mākou hopohopo'ōnaehana hemangioblastoma, retinal hemangioblastoma,ʻaʻa puʻuwai cystic me ka ho okumu a adenocarcinoma:

• hemangioblastoma ua like loa me ka na wahi e ae o ka Central ko mākou hopohopo'ōnaehana IIeEeAIEUAA ma cerebellar'aʻaʻa;
• hemangioblastoma o ka spinal ke kaula;
• hemangioblastoma me IIeEeAIEUAA i loko o ka iwi ka lolo;
• hemangioblastoma ma ka retina;
• cysticʻaʻa puʻuwai'aʻaʻa poho;
• ʻaʻa puʻuwai adenocarcinoma;
• pancreatic cysts;
• tsistadenokartsinoma;
• pheochromocytoma;
• epididymal adenoma.

It E e hoʻomaopopo i ka 'inikuaʻoihana kona mau lala ka i hana i loko o ka pilikia o ka maʻi pale av Hippel-Lindau - neurology, ophthalmology, urology, maloko lapaʻau.

CNS hemangioblastoma

Ka loa pinepine hoike symptoms maʻi pale av Hippel-Lindau hemangioblastoma o ka ulu ana o ka cerebellum, spinal kaula, a me ka retina. Ua ma luna o ka cerebellumʻeho i ka kēia symptoms:

• Headaches Expander ano.
• Nausea, ka luaʻi.
• Maopopo ma ka hele ana, ka hoʻopiʻi i hihia hoʻi ōhumu.
• Pōniuniu.
• Hoʻopilikia o ka ike paka i (ano o na kuʻina ma hope o ka maʻi).

I ka hemangioblastoma IIeEeAIEUAA ma spinal kaula, ka papa kuhikuhiE lapaʻau symptoms mea he emi ma ka pā lonoaʻili, paresis a me ka lōlō, micturition kāna hana a me ka defecation kaʻina. Pain maʻi pale ua kaulana wale kēlā wā i kēia wā.

Kēiaʻano o kaʻeho no a hiki i ka lohi e holumua ai. A loa informative no kaʻike, a me ka hooponopono ana o ka hana nana uaʻike,ʻume mākenēki Resonance kiʻi (MRI), Akä naÿe hoʻokāʻoi hāpana hapa.

Ka mea wale nō pono kaʻaoʻao, e hana i kekahi hemangioblastoma cerebellum keia meaʻoki lāʻau lapaʻau wehe '. Ke hoʻohana no ka pāhawewe a me ka 'inikua ano o ka hoomaopopo ana maikaʻi hualoaʻa mea i hōʻike' ia.

Ma hope o microsurgical wehe 'hemangioblastoma, e like me ka rula, ka mea aole e recur, akā, e hōʻike i nā hikoko. Ma kaʻoki lāʻau lapaʻau lapaau o kaʻeho mea e pono ke lawe i loko, no kona iiiaanoaa, i mea ano o ka maʻi av Hippel-Lindau.

retinal angiomatosis

No ka ophthalmic IIeEeAIEUAA o ka pathological kaʻina ano mea triad hiʻona:

• loaʻa nui o ka angioma;
• vasodilation i loko o ka fundus;
• i ke alo o subretinal exudation (hoʻomāhuahua o ka loli - li nui o ka hoonui - ma lalo o ka cornea), a hiki i ka hooulu ana o exudative retinal poe i loko o ka holomua ke kahua o ka maʻi.

No ka mea kuʻina ka loiloi mua o ka maʻi nō kāu emi iho pigmentation i loko o ka fundus, māhuahua tortuosity o na moku koko. I ka ikaika loa o Paulo ma ka'ōnohi a poʻopoʻo ka, a ua hoailono aku la pulsation o na arteries a me aa koko.

Ma nā kahua o ka hoʻonaʻauao ma hope o ka vasodilatation a me ka vascular tortuosity holomua, ke kahua o aneurysms a me angiomas, i ke kiʻiona Look a glomeruli - keia mea he maopopo hoailona o ka maʻi av Hippel-Lindau.

pheochromocytoma

Inā ka mea, e loli ai ka adrenal glands, av Hippel-Lindau maʻi; ua hoikeia mai ke ano o ka pheochromocytoma. Kēia neoplasm 'ia' ona o ka adrenal medulla mea IeAUPIIe, IAa IO benign ma ka ano. Ma ka hapanui o na hihia diagnosed ma ka makahiki o ma kahi o 30 makahiki, a ua wehewehe aku ma kekahi mau-kapakahi, mau huahelu, a me ka hiki, e neʻe i nā'aʻaʻa e pili.

Pheochromocytoma hoike aku i ke kēia mau hiʻona:

• Hoʻopale i antihypertensive Inc, arterial hypertension.
• Pili i ke koko, cranialgia.
• Pale ili.
• Ka welelau o ka hyperhidrosis.
• Tachycardia.

Cancer o ka pancreas pio

Pancreatic Ka maʻi 'aʻai pathology ee iaaanu aey: hikoko hiki e kekahi benign paha malignant ano. A 'ole e like me kekahi cysts a neuroendocrineʻeho.

Kula Kaiapuni 'o keʻano o ka hanaʻana o ka maʻi' aʻai ka heluna i ka pancreas, ua loaʻa i loko o ka hapalua o nā mea maʻi i ka maʻi av Hippel-Lindau. Symptoms pili me ka, nuiʻana me kaʻole pancreatic kuleana pili i.

Ka awelika makahiki o ka hoʻomanawanui iki mai i ka manawa o ka loaʻaʻana o ka hikoko o 33-35 makahiki. Ma ka hihia o malignant kaʻina metastatic kaʻina ua kuhikuhi 'ana i ke akepaʻa.

Endolymphatic sacʻeho

Hawaiʻi a me ke kapaʻia maʻi pale, i ka oncologic kaʻina mau mea i ka benign papa, a ua hoike mai ia penei:

• I ka lohe ana kāna hana, mai i ka huina lilo o ka hālāwai hoʻolohe manaʻo.
• Pōniuniu.
• Diskoordinatornye aʻe '.
• Tinnitus.
• Paresis o lehalehu t nāʻiʻo.

lapaʻau '

ʻO ka muaʻike, ua lawe mai ma luna o ka muli o kekahi mau a me ka ophthalmologic ninaninau o ka hoomanawanui, anamnesis, me hereditary ohana.

Loa informative o ke ao ana o ke koko, e hōʻike kona maʻiʻo o ka monakō koko, a me catecholamines.

Visualization o hikoko E e hana hoʻohanaʻume mākenēki resonance kiʻi (MRI).

Ke kumu o ka waiwai 'ike no ka kakahiaka ka loaʻaʻana o ka maka maʻi mea kukui hāweo angiography. Kēia PalekanaEND_LINKʻano hana kōkua ai i nā ka 'ike o ka loiloi mua hoikeana o ka maʻi i loko o ka koko fundus a i ole i palapalaia iloko o ophthalmoscopy (e like me telangiectasia, Puhi iho i hana na moku). Ma ka hihia o ka Holomua o ka pathological kaʻina, i kēiaʻano hana hōʻike i ka moku e hanai ana oia i kaʻeho nui mua ma mua o ophthalmoscopy.

Ke hoʻokō 'ia ka hulina ninaninau ninoieo o ke kēia PalekanaEND_LINK kaʻina hana:

1. Kamepiula a me kaʻume mākenēki resonance tomography. Ma ka hoʻohana ana i kēia mau 'ana i kūpono i me ka nui loa, a mahuahua nui o ka helu diagnosed i loko o nā kuʻina kakahiaka o ka maʻi. A ma keia hihia, ka mea, ua pinepine paʻakikī i ka pono.
2. Ultrasound kiʻi.
3. Angiography mea he kumu o Pahuhopu 'ike e pili ana i mea e hanaia mai ka hui, o kaʻeho.
4. Pneumoencephalography.

He nui i ka ike i ka hihia o ka loaʻaʻana o kaʻeho i loko o kekahi o nā kino, ia mea e pono ke alakai i ka piha keia hookolokolo ana, e hookaawale ia i kekahi mau mea o ka pathology.

lapaʻau

Common hoʻokokoke mai i ka lapaau ana i ka maʻi pale av Hippel-Lindau me na loina o ka maʻi 'aʻai Inc. Inc E e noniakahi a huiʻoki lāʻau lapaʻau a me ka pāhawewe 'ana i kūpono. Kēlā me kēia no ia ua hoʻohana pololei i kulike ai me na nāʻikepili, contraindications, a me nā hiʻona o ka mauʻeho hoʻomanawanui.

Lapaʻauʻia hoʻohanaʻano nui me ka symptomatic Inc no ka iaea o kekahi mau oihana o na kino kanaka a me ka nui ana o ka hoʻomanawanui.

Outlook

Me ka 'eleuʻike, a me ka pono i koho lapaʻau, Holomua o ka maʻi, e like me ka pono me ka hopena maikaʻi' ole ma ke kino e hiki i ka pāʻana. OAXA koi '- i kaʻike, a me he lālā o ka lapaau ana ma ke kakahiaka nui kahua o ka hoʻonaʻauao.

Eia naʻe, ma kekahi hihia, hoʻowahāwahā i ka hoʻoikaikaʻana i, ka prognosis koe ilihune no ka hoʻomanawanui, no o ke kamepiula kiʻekiʻe o complications.