I vunah hian hliam i nei em, i taksaah hian lumps i nei em? Hei hi Neurofibromatosis Type 1 (NF1) a ni thei!

Vawiin hian condition pakhat, a buaithlak deuh, mahse mi tam tak tan chuan a pawimawh thei tih kan sawi dawn a ni. Neurofibromatosis Type 1, a tawi zawngin (NF1) kan ti a. He hming hi i hre lo mai thei. Mahse, a awlsam zawngin, i hriatthiam theih dan tur angin sawi ila.

Neurofibromatosis Type 1 (NF1) hi eng nge ni?

A awlsam zawngin, (NF1) hi i vun leh i nervous system (chu chu i thluak , i spinal cord , leh nerve dang zawng zawng) tichhe thei natna a ni. Hei hi kan taksaa cell thenkhat than danah inthlak danglamna tlemte thlentu a ni. Hei hian non-cancerous (benign) tumor a siam thin. Chungte chu neurofibromas an ti a . Heng tumor te hi i thluak, i ruhro leh vun chhunga nerve-ah te a lo awm thin. (NF1) vei te zingah hian a lan chhuah dan ber pakhat chu an vunah café au lait spot tam tak an awm thin hi a ni. Hetiang dinhmun hian mit leh ruh a nghawng thei bawk. A châng chuan Doctor-te pawhin Von Recklinghausen natna an ti bawk a, chu chu i hre tawh mai thei.

Neurofibromatosis Type 1 (NF1) hi engtiang chiahin nge a awm?

Hei hi neurofibromatosis chi khat zingah chuan a tam ber a ni. Dik tak chuan neurofibromatosis vei zawng zawng zinga 96% chuan NF1 an nei a ni. Khawvel pumah kum tin naupang piang 3,000 zinga pakhat velin NF1 an nei nia chhut a ni.

NF1 natna lan chhuah dan chu engte nge ni?

(NF1) symptoms hi nerve compression vang a ni ber. Heng symptoms te hi i hriat lar angin a lang em tih en rawh:

• Café au lait spot paruk aia tam: Hengte hi vunah flat, light brown atanga dark brown spot angin a lang a, birthmarks nen a inang .
• Vun hnuaia neurofibromas pahnih emaw a aia tam emaw: Heng tumor te hi taksa khawi hmunah pawh a lo awm thei. Pea ang maia te emaw, a aia lian emaw pawh an ni thei. An khawih chuan a nem emaw, a khauh deuh emaw pawh a ni thei. Vawi tam tak chu heng tumor chhehvel vun hi i vun rawng pangngai aiin a dum zawk thin.
• Armpits, groin, leh a chang chuan hnute hnuaiah te te (freckles) ang mai a ni.
• Iris emaw, mit nerve tumor emaw (optic glioma)-a lump te te (Lisch nodules ) lo awm a, chuvangin mit a tichhe thei.
• Ruh thanna lama thil dik lo awmte: 1.1.Entirnan, scoliosis emaw, ke ruh bowing emaw, lu pangngai aia lian (macrocephaly), leh a sei tawi te ang chi thil a awm thei.
• Attention-deficit/ hyperactivity disorder ( ADHD ) a awm a, a rilru a hah lutuk a, a rilru a buai em em bawk a .
• Tumor siamna hmuna natna, kal kual harsa, leh a kaihhnawih taksa peng hrang hranga hnathawh harsa.

Heng symptoms te hi mi thenkhat tan chuan a na lo thei hle a, thenkhat tan chuan nasa takin a nghawng thei bawk. Tin, heng symptoms zawng zawng hi pian tirh atangin a awm vek lo. Nun chhung zawngin hun hrang hrangah an lo lang thin. Chuvangin mi tin a nghawng dan pawh a inang lo.

Eng thilin nge Neurofibromatosis Type 1 (NF1) hi a thlen?

NF1 awm chhan ber chu kan gene pakhat a inthlak danglam vang a ni a, chu chu mutation an ti a ni. He gene hi Neurofibromin 1 gene an ti a. He Neurofibromin 1 gene hian kan taksa chu protein pakhat neurofibromin an tih siam turin a hrilh a. He protein hi a pawimawh hle a, a chhan chu cell te hi vawi engzat nge an inthen tih leh copy engzat nge an siam tih a control a ni. A dik zawka sawi chuan hei hi protein a ni a, tumor lo awm thei lo (tumor suppressor) .

Chuvangin, taksain he neurofibromin protein siam dan tur dik tak a hmuh loh chuan cell thenkhat chu an inthen tha lo va, an insiam thar leh thin. Chu ai chuan an mamawh aia tam copy an siam zawk a ni. Chutah chuan tumor a lo awm ta a ni. Tumor chu tissue dense mass a ni a, chu chu cell pangngai lo tam tak atanga siam a ni.

He gene mutation hi i nu leh pa pakhat hnen atangin i rochun thei a ni. Hei hi autosomal dominant pattern of inheritance an ti a . Mahse, i chhungkuaa tumahin he natna hi nei lo mahse he gene mutation hi i nei thei a ni. NF1 vei zinga a chanve vel chuan anmahniin an vei thin. Chumi awmzia chu gene mutation hi thil thleng mai mai a ni a, tumah rochun a ni lo tihna a ni.

Neurofibromatosis Type 1 (NF1) avanga harsatna awm thei te chu engte nge ni?

(NF1) hian harsatna engemaw zat a thlen thei. Chung zinga ṭhenkhat chu hetiang hi a ni:

• I mit i hloh mai thei.
• Ruh thanna lama ruh tliak emaw, a danglamna (dysplasia) a awm thei.
• Nerve a chhiat phah thei.
• Thisen sang (hypertension) a awm thei.
• Enkawl leh lakchhuah hnuah pawh tumor hi a lo lang leh thei a ni.
• Mahni inngaihhlutna a tlahniam thei.

A pawimawh ber chu NF1-a tumor lo awm ṭhenkhat hi cancer a ni.Buaina a nih theihna a awm. Hemi chungchangah hian fimkhur a pawimawh hle a ni.

Engtin nge NF1 hi hmuhchhuah a nih?

Doctor chuan a enfiah ang che a, NF1 i neih leh neih loh enfiah nan a tul angin test a kalpui ang che. A taksa enfiahna a nei hmasa ang a, i natna lan chhuah dante a zawt ang a, engtin nge a nghawng che tih a zawng chhuak ang.

Chu bakah, hetiang test pawh i ti thei bawk:

• Imaging test: Entirnan, MRI, X-ray, emaw CT scan emaw.
• Genetic testing neih a ni.
• Mit enfiahna a ni.

Mi tam zawk chu puitlingah NF1 vei an ni tih hmuhchhuah a ni. Hei hi a chhan chu symptoms hi vawi khatah a lang vek lo va, hun kal zelah a lo thang zauh zauh thin. Entirnan, i pian tirh lamah café au lait spot i nei thei a, kum engemaw zat chhungin a lo lang thei bawk. Mahse, kum 3-5 vel a ngai thei a, chu chu armpits leh groin-ah freckles a lo lang thei a ni. Neurofibromas hi tumor chi khat a ni a, puitling laia hmuh tam ber a ni. Vawi tam tak chu miten mit hmuh dan inthlak danglamna atan damdawi lam panna an zawng thin.

Neurofibromatosis Type 1 (NF1) hi engtin nge enkawl a nih?

Tunah hian he natna (NF1) hi damdawi a la awm lo. Mahse, doctor chuan i natna lan chhuah dan tur a enkawl thei che a ni. I natna lan chhuah danin a nghawng dan azirin enkawl dan tur a inang lo. Enkawlna awm thei thenkhat chu hetiang hi a ni:

• Tumor lakchhuahna surgery neih a ni.
• Chemotherapy hi tumor cancer lo ni tawhte enkawlna a ni.
• Ruh than dan dik lo surgery emaw brace emaw ang chi thil.
• Tumor than control na damdawi: Entirnan, selumetinib an tih damdawi.
• Symptom dang control nana damdawi: Entirnan, ADHD damdawi.

NF1 i neih chuan kum khatah vawi khat tal doctor pan a, medical check-up kimchang neih a pawimawh hle. Kum tin mit lam enfiahna (ophthalmologic examination) pawh i nei tur a ni. Tin, kum khatah vawi khat tal i thisen sang enfiah a, harsatna a awm leh awm loh enfiah a pawimawh hle.

Rilru hriselna lam pawh ngaihtuah a ngai em?

Ni e, a dik tak zet a ni. Hetiang dinhmun hian rilru lamah nghawng a nei che hi thil awmze nei tak a ni. Mi tam tak chuan rilru lam hriselna lama mithiamte biak hian hahdamna an hmu thin . A nih loh leh, dinhmun inang nei mite inkhawmna support group-a tel pawh hi a ṭangkai hle thei bawk. A chhan chu a châng chuan, mi dangte hmuh theih hmuna heng ṭhang leh hnim hringte hi a lo lan hian i hmel lan dânah i lungkham thei a ni. Chuvang chuan hetiang thil hi doctor emaw mental health counselor emaw nena inbiak pawh a tha hle.

Enkawlna hian side effect a nei em?

Ni e, side effects hi enkawlna chi hrang hrang a zirin a danglam thei. I doctor chuan hengte hi enkawlna i tan hmain a hrilhfiah ang che. Entirnan, zai i neih chuan thisen chhuah leh natna hrik i nei thei. Chemotherapy i neih chuan sam tlakchhiatna leh hahna i nei thei.

Neurofibromatosis Type 1 (NF1) hi ven theih a ni em?

Tunah hian NF1 ven dan tur hriat a la ni lo. Mahse, chhungkaw din i tum a nih chuan, chu chu fa neih i beisei a nih chuan doctor nen inbiakna neiin genetic counseling chungchang i zawt thei ang . Hei hian NF1 ang chi genetic disease nei fa i neih theihna chance a hriatthiam tir thei che a ni.

NF1 vei dam rei zawng chu engzat nge ni ang?

A tlangpuiin i symptoms a nasat loh chuan NF1 hian i dam rei zawng direct-in a nghawng lo. Mi tam zawk chuan dam chhung pangngaiin an hmang thin. Mahse, harsatna a awm chuan (a tam lo hle nachungin), a bik takin operation hmanga him taka lak chhuah theih tur tumor a tam lutuk a nih chuan, emaw, tumor chu cancer a nih chuan i dam rei zawng (prognosis) a nghawng thei a ni.

NF1 diagnosis i hmuh chuan eng nge i beisei ang?

(NF1) hi mite a nghawng dan a inang lo hle. Mi thenkhat chuan nitin hnathawhna tibuai thei khawpa na tak symptom an nei lo mai thei. Mi dangte chuan mit lama harsatna emaw, chetsualna tichhe thei khawpa natna emaw an nei thei bawk.

Mi tam tak chuan an natna lan chhuah danin an rilru hriselna leh an taksa an ngaihtuah dan a nghawng lai hian rilru lam hriselna lama mithiamte biak chu a \angkai hle niin an hria. Chûng ṭhang lian, mole leh pian chhinchhiahna ang chite chu a châng chuan mi dangte hmuh theih a nih avângin, i hmel lan dân chu i zak emaw, i zak emaw a ni thei. Doctor chuan heng rilru natna leh i rilru ti na thei symptom eng pawh hi a enkawl thei che a ni.

Engtikah nge Doctor ka hmuh ang?

NF1 symptoms i neih chuan, a bik takin café au lait spot paruk aia tam i neih chuan, sawifiah theih loha vun inthlak danglam emaw, mitdel emaw i neih chuan doctor pan nghal rawh. NF1 enkawl mek i nih chuan natna lan chhuah dan, natna ang chi te a pung emaw a zual emaw chuan doctor hnenah hrilh rawh.

Chu bâkah, kum khatah vawi khat tal medical check-up neih a pawimawh hle bawk . Hei hian i taksa peng hrang hrang NF1-in a nghawng thei te hi a thawk tha em tih enfiah nan a pui thei a ni:

• Mit
• Nervous system a ni
• Vun
• Lung leh thisen kalna (Cardiovascular system) a ni
• Spine a ni

Doctor hnenah eng zawhna nge ka zawh ang?

Doctor hmu tura i kal hian hetiang zawhna hi i zawt thei ang:

• (NF1) diagnosis a nih chuan eng nge ka beisei ang?
• Ka fate hian he dinhmun hi an rochun thei ang em?
• Eng enkawlna nge i rawt? Side effect a awm em?
• Tumor te hi lakchhuah a nih hnuah a lo thang leh thei em?
• Follow-up appointment atan engzat nge ka lo haw a ngai?

A tawp berah chuan thil hriat reng tur (Take-Home Message) .

Neurofibromatosis Type 1 (NF1) hi a tam ber a ni. I taksa peng tam tak a nghawng thei a, a bik takin i vun leh nervous system a nghawng thei a ni. I natna lan chhuah dan hian i nitin nun leh i taksa i ngaih dan a nghawng thei a ni. Mahse, lungngai suh. I medical team chuan i dinhmun enkawlna tha ber zawng turin an pui thei che a ni. I natna lan chhuah dânin i inngaihhlutna a nghawng a nih chuan, rilru lam hrisêlna lama thurawn petu nêna inbiakna neih chu a ṭangkai hle mai thei . NF1 i vei tih i hriat tawh a, chhungkaw din i tum a nih chuan i doctor nen genetic counseling chungchang sawi dun theihnghilh suh.

Nangmah chauh i ni lo, he zinkawngah hian tanpui tur che mi tam tak an awm.