I vunah, a nih loh leh i naute taksaah hian light brown spot tam tak i hmu tawh em? Vun hnuaiah hian lump te te i hmu mai thei em? Mi tam takin hengte hi thil pangngaiah an ngai laiin, a chang chuan ‘Neurofibromatosis Type 1’, a tawi zawngin NF1 an tih, genetic condition chhinchhiahna a ni thei bawk. Ngaihtuah buai suh, a hming hi hlauhawm tak angin lang mahse, manageable condition a ni. Vawiin hian a zawng zawng hi a awlsam, hriatthiam awlsam takin kan sawi dawn a ni.
A awlsam zawngin NF1 hi eng nge ni?
NF1 hi genetic condition a ni a. Kan vun leh nervous system (chu chu thluak, ruhro leh taksa pum puia nerve te a nghawng ber a ni). Kan taksaa cells te than dan control tu process ah hian inthlak danglamna tlemte a thlen thin. Kan taksaa ‘switch’, cell inthen darhna thununtu ang maiin han ngaihtuah teh. NF1 vei chuan he switch-ah hian chhiatna tlem a nei a. Chumi avang chuan cell thenkhat chu an inthen chak lutuk a, tumor an siam thin.
Heng tumor te hi nerve-ah an lo awm tlangpui. Chuvangin neurofibromas kan ti a ni. A pawimawh ber chu heng tumor tam zawk hi benign an ni . Chu chu cancer an ni lo tihna a ni. Heng tumor te hi thluak, ruhro leh vun chhunga nerve-ah te a lo awm thei a ni. I doctor-in he natna hi ``Von Recklinghausen disease'' a tih i hre tawh mai thei.Chu chu a hming dang a ni.
NF1 hi engtiang chiahin nge a awm?
NF1 hi neurofibromatosis chi khat a ni ber a. Damlo zawng zawng zinga 96% chu hetiang chi-ah hian an tla niin an sawi. Khawvel pumah nau piang thar 3,000 zinga pakhat velin NF1 an nei thei niin an chhut. Chumi awmzia chu thil awm lo tak a ni lo tihna a ni.
NF1 natna lan chhuah dan berte chu engte nge ni?
NF1 symptoms hi a hmaa kan sawi tawh tumor te avanga lo awm a ni a, chu chuan nerve a press a ni. Mahse mi zawng zawng hian symptoms inang an nei vek lo. Mi thenkhat chuan symptoms an nei tlem hle a, thenkhat erawh chu tlemin a nghawng nasa zawk thei bawk. A lan chhuah dan bulpui te han thlir ila.
| Symptom a ni | A sawifiahna awlsam tak |
|---|---|
| Café au lait hmun hrang hrangah a awm | Hengte hi i coffee-a bawnghnute tlem i dah atanga lo chhuak light brown colour ang kha a ni. Vun chunglam a flat spot chi khat an ni. Diagnosis atan chuan heng spot paruk aia tam neih hi thil pawimawh takah ngaih a ni tlangpui. |
| Neurofibromas a awm thin | Vun hnuaia nerve tumor lo awm thin. Chungte chu taksa khawi hmunah pawh a lo thang lian thei a ni. Ṭhenkhat chu pea ang maia te an ni a, ṭhenkhat erawh chu a lian zawk. Khawih pawhin a nem emaw, a nghet deuh emaw pawh a ni thei. |
| Armpit leh groin-ah hmun a awm | He natna danglam bik chu hmeichhiate hnute hnuai, a kekawrte, leh a chang chuan hnute hnuaiah te te (freckles) a lo lang thin hi a ni. |
| Mit a inthlak danglam thin | Mit iris-ah hian brown lumps te te (Lisch nodules) a lo awm thei a. Tin, mit leh thluak inzawmkhawmtu nerve-ah hian tumor (optic gliomas) a lo awm thei bawk. Hei hian mit lamah harsatna a thlen thei a ni. |
| Ruh lama harsatna nei | Scoliosis, ke hrual, lu pangngai aia lian (macrocephaly), leh a sei tawi te ang chi thil hmuh tur a awm. |
| Ngaihtuahna lama harsatna a awm | Naupang leh puitling thenkhat chuan attention deficit hyperactivity disorder (ADHD) ang chi natna an nei thei bawk. |
| Na | Tumor siamna hmunah chuan nerve compression vangin natna a thlen thei a ni. Tin, taksa peng thenkhat hnathawh pawh a nghawng thei bawk. |
A pawimawh ber chu heng mizia zawng zawng hi mi pakhatah a thleng vek lo tih hi a ni. Tin, an pian tirh lamah hmuh theih vek an ni lo. A mizia thenkhat chu kum upat chhoh rualin a lo lang tan thin.
Eng thilin nge NF1 hi a thlen?
Hei hi kan genes inthlak danglamna tenau lutuk vang a ni. A dik tak chuan `neurofibromin 1` an tih gene-a mutation a ni. He gene hian kan taksa chu protein pakhat `neurofibromin` siam turin a hrilh a. He protein hi kan taksa chhunga cell te inthen darh dan tur control tu `brake` ang a ni. Hei hi `(tumor suppressor)` kan ti bawk.
NF1-ah hian he gene-a thupek dik lo a awm avangin neurofibromin protein hi a siam tha lo hle. Chumi awmzia chu ‘brake’ a thawk tha lo tihna a ni. Chumi avang chuan cell thenkhat chu thunun theih lohvin an inthen a, tumor an siam thin.
He genetic change hi a awm theih dan kawng hnih a awm a:
1. Nu leh pa atanga rochan: Nu leh pa pakhatin NF1 a neih chuan naupangin he natna hi a rochun theihna chance 50% a nei a ni.
2. Random occurrence: NF1 vei zinga a chanve vel chuan chhungkaw history an nei lo. Hei hian a awmzia chu nu leh pate hian he natna hi an nei lo a nih pawhin naupang taksaa gene siam chhuah chhung hian a inthlak danglamna chu random takin a thleng thei tihna a ni.
NF1 hian eng harsatna nge a thlen theih?
Mi tam zawk chuan harsatna lian tham tak nei lo mah se, a hnuaia mite hi a chang chuan a thleng thei a ni:
- Mitdelna (optic glioma tumor vang) .
- Fracture emaw ruh deformities emaw a awm thei
- Nerve tihchhiat a ni
- Thisen sang (hypertension) 1.1.
- Enkawl hnuah pawh tumor a lo lang leh thin
- Mihring hmel lan dan ngaihtuah avanga mahni inngaihhlutna hniam
Heng tumor te hi cancer ni lo mahse, a tlem hle a, neurofibroma thenkhat chu cancer a ni thei. Chuvangin doctor hnen atanga check out fo a pawimawh hle.
Engtin nge NF1 hi hmuhchhuah a nih?
Doctor chuan natna chungchang ngaihtuahna a neih theih nan a enfiah hmasa ang che. Doctor chuan i vun chu uluk takin a enfiah ang a, i vunah hian a hring, a hring, etc. a awm leh awm loh a enfiah ang a, chubakah, a natna hriat chian nan test an nei thei bawk.
- Imaging test: Taksa chhungah tumor a awm em tih hriat nan `(MRI)`, `(X-ray)` emaw `(CT scan)` ang chi test te hi a ni.
- Genetic testing: `NF1` gene-ah hian mutation a awm leh awm loh finfiah nan thisen test a ni.
- Mit enfiah: Lisch nodules awm leh awm loh enfiah turin specialist hnenah i mit enfiah tir.
He natna hi puitlingah hmuh chhuah a ni fo. Hei hi a chhan chu, kan sawi tawh ang khan, natna lan chhuah dan thenkhat (entir nan, vun hnuaia lumps) chu kum upat rualin a lo lang tan thin a ni.
NF1 enkawlna tur engte nge ni?
Sawi hmasa tur pawimawh ber chu, .NF1 hi damdawi a la awm lo. Mahse, hlau suh. I natna lan chhuah dan enkawl thei tur leh nun hlawhtling leh hlim zawka i nun theih nan enkawlna tam tak a awm a. Enkawlna chu i natna lan chhuah dan chi hrang hrangah a innghat a ni.
- Surgery: Tumor na tak tak, nerve compress emaw, hmel tibuaitu emaw te chu surgery hmangin lakchhuah theih a ni.
- Cancer enkawlna: Tumor chu cancer a nih chuan ``chemotherapy`` tih ang chi enkawlna pek thin a ni.
- Ruh enkawlna: Surgery emaw braces emaw hi spinal fusion ang chi thil atan hman a ni.
- Damdawi: Tunah chuan tumor thanna control nan damdawi bik, selumetinib ang chi a awm ta. Damdawi hi ADHD ang chi natna enkawlna atan pawh hman a ni.
Medical checkup neih fo a pawimawhzia
NF1 vei tan chuan kum khatah vawi khat tal doctor pan a, check-up kimchang neih a pawimawh. Tin, kum tin an mit leh thisen sang endik tir thin tur a ni. Hei hian harsatna thar an neih chuan a hmaa an hriatchhuah theih nan leh enkawlna an tan tir thei dawn a ni.
Engtikah nge Doctor i hmuh ang?
Nangmah emaw i fa emaw hian NF1 symptoms i neih i rinhlelh chuan, a bik takin a hnuaia mite hi i hriat chuan doctor pan ngei ngei tur a ni.
- Café-au-lait (coffee rawng) hmun paruk aia tam neih.
- Vun a inthlak emaw, chhan awm lovin a lum emaw.
- Mit hmuhna lama inthlak danglamna.
NF1 i vei nia hriat tawh i nih chuan, natna zual, tumor lian chak tak, symptom thar i neih chuan doctor hnenah hriattir nghal rawh.
Take-Home Thuchah a ni
- NF1 hi genetic condition a ni a, vun natna leh nerve-ah tumor a siam thin. Heng tumor tam zawk hi chu cancer a ni lo.
- Hetiang dinhmun hi nu leh pa atanga rochun a ni thei a, chhungkuaa tumah awm lovin random takin a thleng thei bawk.
- Mi tin hian natna lan chhuah dan a inang lo hle. Tin, symptom zawng zawng hi vawi khatah a lang vek lo va, hun kal zelah a lo lang chhuak thin.
- NF1 hi tihdam kim theih ni lo mah se, natna lan chhuah dan control thei tur leh nun tha tak nei thei tur enkawlna tam tak a awm. Kum tin medical checkup neih a pawimawh hle.
- Skin tag leh bumps avanga lungngaihna leh lungngaihna hi thil pangngai tak a ni. Hemi chungchang hi i doctor emaw mental health counselor emaw hnenah sawi hreh ngai suh.











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