Ta yaya cutar Von Hippel-Lindau?

Da cutar, wanda za a tattauna daga baya a cikin labarin, yawanci dangana ga kungiyar na hadaddun kwayoyin cuta. Its main alama ne da samuwar mahara marurai a cikin jiki samu daga kyallen takarda daban-daban gabobin. Wane irin jiki za a buga wuya, shi ya dogara, kamar yadda bayyana von Hippel-Lindau ciwo.

Duk da haka, da aka sani da hali shafe takwas marasa lafiya, yana da asali daga wannan iyali a matsayin kawai alama ya polycystic koda cuta. A ganewar asali a cikin wannan harka da aka kafa ta amfani da katin shaida na kwayoyin aibi (maye gurbi).

Von Hippel-Lindau cutar da ake alaka da rare cuta, da kuma mita na da ya faru - daya hali da 36,000 kowacce haihuwa.

The inji na cuta ci gaba

Dalili von Hippel Lindau cuta ne hereditary yanayi jimlace cuta halin da ya wuce kima yaduwa na capillary zane da kuma bayyanar a sakamakon babban adadin neoplastic siffofin maruran.

Cutar da ake daukar kwayar cutar zuwa ƙarni na gaba a wani autosomal rinjaye iri, watau a gaban wani gene shigar a Pathology, daya daga cikin iyaye, yiwuwar wajen kisa da zũriyarta ne 50%.

A aibi (maye gurbi) sakamakon a danniya na gene, wanda shi ne ta haɗẽwar haushi na ƙari girma.

Bayyananen - ciwon daji matakai

Babban manifestations von Hippel Lindau ciwo - pituitary marurai, cerebellum, laka, akan tantanin ido, ciki gabobin. List of yiwu neoplasms:

• Hemangioblastoma - da sarrafawa a cikin cerebellum kuma akan tantanin ido. samu daga m nama. CNS neoplasms da aukuwar cuta a cikin marasa lafiya da von Hippel-Lindau akalla 60%. A talakawan shekaru marasa lafiya suka sami irin wannan tsakiya m tsarin marurai, 42 years old.
• Angioma - a neoplasm na jijiyoyin bugun gini asalin, da sarrafawa a cikin laka.
• Cysts. Wannan neoplasm da ciwon ciki a rami cike da ruwa. Irin wannan abubuwa za a iya kafa a cikin hanta, koda, testis da kuma pancreas. Musamman, koda da kuma pancreatic cysts da ake samu a 33-54% na lokuta.
• Pheochromocytoma - ƙari samu daga adrenal medulla Kwayoyin. Abin da ya faru - game 7% na marasa lafiya. A talakawan shekaru marasa lafiya da pheochromocytoma - game da shekaru 25.

• Cell sankara. A musamman, da talakawan shekaru lokuta da wannan ƙari - 43 years.
• A maza da cutar Von Hippel-Lindau zai iya ci gaba neoplasms benign halitta a cikin golaye (misali, papillary cystadenoma).
• Mata sun sami ƙari rauni na igiyar ciki jijiyoyin.

A sharudda da abin da ganewar asali ne

A ganewar asali da "cuta von Hippel-Lindau" za a iya kawota a hali na ganewa na akalla daya daga cikin gabatar A cikin wadannan jerin cututtuka. Amma yana yiwuwa idan kana da wani tarihi na akalla daya daga cikin 'yan uwa na haƙuri ta tsakiya m tsarin hemangioblastoma, retinal hemangioblastoma, koda cystic tsarin ko adenocarcinoma:

• hemangioblastoma da ciwon sarrafawa a cerebellar nama, kazalika da a wasu sassa na tsakiya m tsarin.
• hemangioblastoma na laka.
• hemangioblastoma da sarrafawa a cikin bargo.
• hemangioblastoma a kan haske a jikin.
• cystic koda nama lalacewa.
• koda adenocarcinoma.
• pancreatic cysts.
• tsistadenokartsinoma.
• pheochromocytoma.
• epididymal adenoma.

Ya kamata a lura da cewa likita sana'a wanda mambobi tsunduma a matsalar na ciwo von Hippel-Lindau - ilimin tsarin jijiyoyi, ophthalmology, urology, ciki magani.

CNS hemangioblastoma

A mafi akai-akai ruwaito cututtuka ciwo von Hippel-Lindau hemangioblastoma ne da ci gaban da cerebellum, laka, ko akan tantanin ido. Shin a kan cerebellum ƙari ya da wadannan cututtuka:

• Ciwon kai Expander yanayi.
• Tashin zuciya, amai.
• Rashin tabbas a tafiya, saboda sosai daidaituwa.
• Juwa.
• Hanawa na sani (halayyar da daga baya, saukarwa da cutar).

Lokacin da hemangioblastoma sarrafawa a laka, babban asibiti cututtuka su ne a karu a ji na ƙwarai, paresis kuma inna, micturition cuta da kuma defecation tsari. Pain ciwo aka lura kawai lokaci-lokaci.

Wannan irin ƙari nasa ne a sannu a hankali m. A mafi m ga bincike da kuma iko Hanyar dubawa aka gane, Magnetic rawa Hoto (MRI), bambanci inganta samfurin rabo.

The kawai tasiri hanyar yi wa wani hemangioblastoma cerebellum yau ne m kau. A amfani da radiation da kuma kiwon lafiya da hanyoyin da ake bautawar tabbatacce sakamakon da aka ba da aka nuna.

Bayan microsurgical kau hemangioblastoma, kamar yadda mai mulkin, ba sake aukuwa ba, amma za su iya bayyana a wasu siffofin maruran. A m magani na ciwon daji wajibi ne a yi la'akari da ta jam'i, wanda shi ne halayyar da cutar Von Hippel-Lindau.

retinal angiomatosis

Domin ophthalmic sarrafawa na pathological aiwatar halayyar ne triad fasali:

• samuwan angioma.
• vasodilation a fundus.
• gaban subretinal exudation (jari na ruwa - kumburi daga cikin samfurin - karkashin cornea), wanda zai iya sa da ci gaban exudative retinal detachment a wani ci-gaba mataki da cutar.

Domin da farko, saukarwa da cutar yawanci rage pigmentation a fundus, ta ƙara tortuosity na jini. Lokacin da guga man a kan eyeball, kuma an alama pulsation na arteries da jijiyoyinmu.

A baya matakai na vasodilatation da jijiyoyin bugun gini tortuosity ci gaba, da samuwar aneurysms da angiomas, da rarrabe look zagaye glomeruli - wannan shi ne wani takamaiman alamar rashin lafiya Von Hippel-Lindau.

pheochromocytoma

Idan rinjayar da adrenal gland, von Hippel-Lindau cuta ne bayyana irin pheochromocytoma. Wannan neoplasm faruwa abubuwa na adrenal medulla ne yawanci benign a yanayi. A mafi yawan lokuta kamu da shekaru game da 30 shekaru da kuma halin da biyu mai gefe, mahara raka'a da kuma ikon yin hijira zuwa kyallen takarda m.

Pheochromocytoma bayyana da wadannan siffofin:

• Resistant zuwa antihypertensive far, jijiya hauhawar jini.
• Dangantawa da jini cranialgia.
• Kodadde fata.
• Hali ya hyperhidrosis.
• Tachycardia.

Ciwon daji na pancreas kashi

Pancreatic ciwon daji Pathology m: marurai na iya zama ko dai benign ko m yanayi. A tsarin bisa ga ko dai cysts ko neuroendocrine ƙari.

Daban-daban matakai na ciwon daji shafi cikin pancreas, an samu a cikin rabin da marasa lafiya da cutar Von Hippel-Lindau. Alamun dangantawa da, kullum tare da mai illa pancreatic aiki.

A talakawan shekaru na marasa lafiya a lokacin ganewa na marurai na 33-35 shekaru. A cikin hali na m aiwatar metastatic tsari ne directed zuwa hanta.

Endolymphatic jakar kwai ƙari

Gwamnatin tare da mai suna ciwo, da oncologic tsari ko da yaushe yana mai kirki hanya da aka nuna kamar haka:

• Ji cuta, har zuwa a total asarar ji.
• Juwa.
• Diskoordinatornye take hakki.
• Tinnitus.
• Paresis na gyara man fuska tsokoki.

bincikowa da

A na farko ganewar asali ne da za'ayi a kan tushen da janar da kuma ophthalmologic jarrabawa na haƙuri, anamnesis, ciki har da hereditary iyali.

Very m ne nazari na jini, don gane da abun ciki na glucose, da kuma catecholamines.

Na gani daga marurai ya kamata a yi amfani da Magnetic rawa Dabarar (MRI).

A tushen muhimmanci da bayanai ga farkon ganewa na ido cuta ne mai kyalli angiography. Wannan bincike dabara facilitates da katin shaida na farko manifestations cuta a cikin jini fundus wanda ba a lokacin rubuta ophthalmoscopy (kamar telangiectasia, sabuwar kafa tasoshin). A cikin hali na ci gaban da pathological tsari, wannan dabara ta bayyana wani jirgin ruwa da ciyar da ƙari da yawa a baya fiye da ophthalmoscopy.

Kammala wani m jarrabawa kunshi wadannan bincike hanyoyin:

1. Computer da kuma Magnetic rawa tomography. Ta hanyar amfani da wadannan dabaru na da muhimmanci kara yawan kamu a farkon matakai da cutar. Kuma a cikin wannan harka, su ne sau da yawa wuya su bi.
2. Duban Dabarar.
3. Angiography ne tushen haƙiƙa bayani game da abin da ke faruwa daga abin da sashin jiki da ƙari.
4. Pneumoencephalography.

Yana da muhimmanci a san cewa idan na ganewa na ƙari a daya daga cikin jikinsu, shi wajibi ne don kai cikakken jarrabawa ware wasu yankunan Pathology.

magani

Common hanyoyin da magani na ciwo von Hippel-Lindau tare da ka'idodinta ciwon daji far. Far ya kamata a hadedde kuma hada m, kuma radiation dabaru. Kowane daga cikinsu yana amfani da tsananin daidai da alamomi, contraindications, kuma fasali na musamman ƙari haƙuri.

Magunguna amfani da yafi a matsayin symptomatic far ga gyara na wasu ayyuka da mutum jikinsu da kuma janar yanayin haƙuri.

Outlook

Tare dace ganewar asali da kuma daidai zaba magani, ci gaban da cutar, kazalika da ta korau effects a jiki iya sarrafa. M da ake bukata - da bincike da kuma qaddamarwa magani a farkon matakai.

Duk da haka, a wasu lokuta, duk da kokarin da ya yi, da hangen nesa da ya rage matalauta ga mãsu haƙuri saboda babban hadarin da rikitarwa.