I hriatna a hloh nghal a, i lu a buai mai mai em ni? I beng bulah thawm i hre mai mai em ni? Hengte hi thil pangngaiah i ngai a, mahse a chang chuan heng thil hnung lamah hian kan hriat tam loh natna a awm thei a, mahse kan fimkhur tur a ni. Vawiin hian chutiang natna chungchang kan sawi a ni. He natna hi Neurofibromatosis Type 2, a tawi zawngin NF2 a ni.
A awlsam zawngin sawi ila, Neurofibromatosis Type 2 (NF2) hi eng nge ni?
NF2 hi genetic condition a ni a, kan nervous system a nghawng a ni. Kan taksaa cell thang chakna control tu gene inthlak danglamna avanga lo awm a ni. He genetic change hian taksain cell a siam tam lutuk a. Heng cell tam lutuk te hi an pungkhawm thei a, tumor an siam thei bawk.
Mahse lungngai suh, heng lumps tam zawk hi benign , cancer a ni lo tihna a ni. Heng lumps te hi a bulpui ber chu:
- Peripheral nerve hi kan taksa peng hrang hrang, ke leh ke leh ke peng hrang hranga inzar pharh nerve a ni.
- Kan luruh leh thluak inkar (meninges) inkar membrane.
- Spine a ni.
- Thluak leh beng chhung lam thlunzawmtu nerve te, hriatna leh taksa inthlauhna atana puitu te.
NF2 hi natna chi khat Neurofibromatosis an tih zingah a tel a ni. He natna pawl hian kan vun leh nervous system a nghawng ber a ni.
Khawtlang nunah he NF2 dinhmun hi engtiang chiahin nge a hluar?
Hei hi thil awm lo tak a ni. Statistics atanga a lan dan chuan NF2 hian khawvel puma nausen piang 33,000 zinga pakhat vel a nghawng a ni. Neurofibromatosis vei hmuhchhuah zawng zawng zinga 3% vel chu NF2 vei an ni.
NF2 hi eng symptoms nge ni?
NF2 natna lan chhuah dan hi mi hrang hrangah a inang lo thei. I taksaa tumor awmna hmun leh a lian ziaah a innghat a ni. Mi tam zawk chuan beng leh thluak inzawmkhawmtu nerve-a tumor awm vangin symptoms an nei hmasa thin.
Heng symptoms te hi chiang taka hrethiam turin i en ang u:
| Mihring ziarang category | A lan chhuah dan tlangpui |
|---|---|
| Auditory nerve nena inzawm symptom hmasa ber | |
| Balance lama harsatna a awm | Sudden luak chhuak |
| Hriatna lama harsatna nei | Zawi zawiin hriatna a hloh |
| Beng ah chuan a ri ta | Beng chhunga ri ri hriat (Tinnitus) . |
| Neurological symptom dangte pawh a awm bawk | |
| Thil dang pawh a awm |
|
Naupan lai atanga kum 30 inkar inkarah hian symptoms hi a lang tan fo thin a, mahse he natna hian kum eng pawhah tu pawh a nghawng thei a ni. Puitlingah chuan beng leh thluak thluak thlunzawmtu nerve te hi a nghawng hmasa fo thin. Chuvang chuan hriatna lama harsatna lo lang hmasa ber a ni. Naupangah erawh chuan thluak emaw, ruhro emaw-ah tumor a lo awm fo thin. Naupan lai emaw, tleirawl lai emawa symptoms a lan chuan natna hi a na zawk tih a tilang thei a ni.
NF2 ah hian eng chi tumor nge lo awm?
NF2 ah hian tumor chi hrang hrang a lo awm thei a. Anni hi han thlir thuai teh ang u.
| Tumor chi hrang hrang | A awlsam zawngin... |
|---|---|
| Schwannomas a ni | Hengte hi nervous system-a tumor, Schwann an tih cell atanga lo piang chhuak an ni. Thluak leh beng chhung lam inzawmkhawmtu nerve (vestibular schwannomas)-ah an lo awm tam ber. Mit che vel, lei inher leh ei leh in control tu nerve-ah te pawh an lo thang lian thei bawk. |
| Meningioma natna (meningiomas) a awm | Tumor chi khat, thluak leh luruh inkar venhimna (meninges)-a lo awm. |
| Gliomas (Gliomas) a awm | Hengte hi thluaka tumor chi khat, glial cell an tih cell atanga lo piang chhuak an ni. Optic nerve vel ah an lo piang fo thin. |
| Ependymomas natna (Ependymomas) te | Hei hi glioma chi khat a ni bawk. Thluak leh ruhroah a lo thang lian thin. Thluak leh ruhro chhunga cerebrospinal fluid (CSF) siamtu cell atanga lo chhuak a ni. |
Engvangin nge NF2 hi a lo awm? Eng nge a chhan?
A chhan ber chu kan taksaa Neurofibromin 2 (NF2) an tih gene inthlak danglam vang a ni. He gene hian protein ‘merlin’ an tih siam nan a pui a ni. He protein hnathawh ber chu tumor lian zel tur tihtawp a ni. Chu chu tumor-suppressor a ni tihna a ni .
Chuvangin, he NF2 gene-ah hian chhiatna a awm chuan ‘Merlin’ protein hi a siam tha lo hle a ni. Tichuan kan taksaa cell ṭhenkhat chu thunun theih lohvin an inṭhen a, an pung a, an inzawm khawm a, tumor an siam a ni.
He genetic variation hi nu leh pa atanga fate atanga rochun theih a ni. Hei hi autosomal dominant pattern an ti a. Mahse mi tam tak tan chuan rochun a ni lo. Hei hian he genetic variation hi naupai lai pawhin a awm thei tihna a ni a, chhungkaw history engmah a awm lo.
Hetiang dinhmun avang hian eng harsatna nge awm thei?
NF2 hi control a nih loh chuan complication thenkhat a awm thei. Chung zingah chuan:
- Hriatna hloh vek
- Mit hmuhna hloh
- Nerve chhiatna awm reng
- Thluak a tui awm zat (hydrocephalus) .
Heng tumor te hi cancer ni lo mahse, NF2 tumor thenkhat chu cancer a nih theihna chance a tlem hle a, a tlem hle. Chuvangin regular medical checkup neih hi a pawimawh hle a ni.
Engtin nge NF2 hi hmuhchhuah a nih?
Doctor i pan hunah uluk takin a enfiah hmasa ang che a, i natna lan chhuah dan leh i chhungte zingah he natna hi tu emaw chuan an vei tawh em tih te a zawt hmasa ang che. Tichuan, a natna hriat chian nan test eng emaw zat a ti thei ang:
- MRI (Magnetic Resonance Imaging) scan: Hei hian nervous system leh vunah tumor awmte chu chiang takin a hmu thei a.
- Hearing test: I hriatna level enfiah rawh.
- Mit enfiah: Mit (cataract) emaw, mit lama harsatna dang emaw a awm leh awm loh enfiah thin ang che.
- Genetic testing: NF2 gene-ah hian chhiatna a awm leh awm loh finfiah nan.
NF2 enkawl dan tur chu engte nge ni?
Hei hi damdawi a la awm lo. Mahse, lungngai suh, tunah chuan heng tumor te hi hriatchhuah, enkawl leh enkawl dan hmasawn tak tak a awm ta. I doctor chuan i tana tha ber tur enkawlna a thlang ang. Enkawl dan hi mi hrang hrangah a inang lo thei.
Enkawl dan tlangpui thenkhat chu hetiang hi a ni:
- Surgery: Surgery hmanga symptomatic tumor te lakchhuah. Surgery hmanga mittui tlakna (cataract) tihbo nan pawh hman a ni.
- Chemotherapy emaw Radiation Therapy: Heng enkawlnate hi tumor thenkhat lian tham tihziaawm nan an hmang thin. Chutiang radiation therapy hmanraw hmasawn tak pakhat chu Stereotactic radiotherapy hi a ni.
- Hriatna leh mit hmuhna hmanrua: Hriatna lama harsatna nei te chuan `Hearing aids`, `cochlear implants` emaw `auditory brainstem implants` ang chi hmanrua an hmang thei a ni. Mitmeng ang chi thilte hian mitdelna kawngah a pui thei a ni.
- Aids dang: Balance leh kea kal harsatna i neih chuan mobility devices i hmang thei ang.
- Damdawi: Natna ang chi symptoms control nan doctor chuan damdawi a pe thin.
A pawimawh ber chu a chang chuan, cyst te hian harsatna lian tham a thlen loh chuan i doctor chuan enkawl loh a rel thei a, a enfiah reng mai thei. Chuvangin kum khatah vawi khat tal taksa enfiahna, MRI scan, beng leh mit enfiahna neih a pawimawh hle.
Engtik hunah nge doctor ka hmuh ang?
A hnuaia symptoms pakhat emaw a aia tam emaw i neih chuan doctor pan ngei ngei tur a ni.
- Mit hmuh dan inthlak danglam.
- Hriatna a inthlak emaw, beng a ri emaw.
- Muscle chak lohna emaw, hmai tlahniam emaw.
- Skin bumps emaw spot thar emaw a awm.
- Lu na reng reng.
- Chhan awm lovin taksa peng hrang hrangah natna.
- Fit (seizures) neih.
Vawi tam tak chu hriatna emaw, mit hmuhna lama inthlak danglamna hi he natna chhinchhiahna hmasa ber a ni. Chuvangin, heng symptoms zinga pakhat tal i neih chuan, tihkhawtlai lohvin damdawi lam thurawn zawng la.
Take-Home Thuchah a ni
- Neurofibromatosis Type 2 (NF2) hi genetic condition a ni a, nervous system-ah cancer ni lo tumor a lo awm a.
- Hriatna hloh, luak chhuak, beng ri (tinnitus) te hi a hmaa a lan chhuah tam ber a ni.
- He natna hi damdawi la awm lo mah se, a natna lan chhuah dan control dan leh tumor enkawl dan hmasawn tak tak tam tak a awm a ni.
- Natna hriat chhuah a nih hnuah doctor enkawlna hnuaia awm reng a pawimawh hle a, chutah chuan MRI scan te, beng leh mit enfiah te, kum khatah vawi khat tal checkup neih reng a pawimawh hle.
- Nangmah emaw, i chhungte zinga tu emaw hian heng symptoms hi i neih chuan doctor thiam tak hnenah thurawn dil nghal rawh.











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