I taksaah hian hmun mak tak tak emaw, lumps emaw i nei em? Neurofibromatosis (NF) chungchang hi han sawi ila!

I naupan lai atanga i hmuh tawh ngei tur chu i vunah coffee rawng chi hrang hrang i nei em? A nih loh leh, a châng chuan i taksa hmun hrang hranga thil tê tê, hrual hrual lo lang chhuak i nei ṭhin em? Hengte hi spot emaw lumps emaw chauh a ni lo mai thei. Vawiin hian chutiang dinhmun chu kan sawi dawn a, a buaithlak deuh a, mahse hriatthiam theih a ni. Chu chu Neurofibromatosis , a tawi zawngin NF a ni.

Neurofibromatosis (NF) hi eng nge ni?

A awlsam zawngin, Neurofibromatosis (NF) hi kan nervous system leh genes tichhe thei natna chi khat a ni . Kan thluak, ruhro, nerve leh vun a nghawng a ni. I natna lan chhuah dan hi mi hrang hrangah a inang lo thei a, NF i neih chi hrang hrang a zirin a inang lo bawk. Mahse a lan chhuah tam ber chu birthmark leh tumor te hi a ni a, a tlangpuiin cancer ni lo (benign) a ni. I chhungte hnen atangin he natna hi i rochun thei bawk a, chu chu i genes a ni. Mahse mak tak maiin 50% vel ah chuan random takin a thleng thei a, chhungkaw history engmah a awm lo.

Neurofibromatosis (NF) chi hrang hrangte chu engte nge ni?

Heng NF te hi chi thum a awm a. Eng nge an nih i en ang u.

1. Neurofibromatosis chi khat (NF1) .

Hei hi NF chi khat a ni ber . A lo awm a, chu chu:

• Café au lait spots: Hengte hi coffee rawng spot ang mai a ni. Taksa khawi hmunah pawh an lang thei.
• Neurofibromas: Nerve-a tumor te tak te lo awm thin.
• Armpit leh groin-a freckles: Dot te tak te ang maia lang.
• Optic nerve tumors: Hengte hi mit nena inzawm nerve-ah a lo awm thei a.
• Bone deformities: Entirnan, scoliosis ang chi thil.

Ngaihtuah teh, hmeichhe naupang pakhat Nimali tih hming pu a awm a. A pian tirh khan a taksaah hian milky coffee rawng chi hrang hrang a nei a. Tlem a lo puitlin deuh deuh chuan a kutphah ah pawh hmun te te a lo lang bawk. Doctor hnena a entir hnuah chauh NF1 a vei tih a hre ta a ni.

2. NF2 nena inzawm Schwannomatosis (hmanlai chuan Neurofibromatosis type 2 (NF2) tia koh thin) .

Hetiang chi hi a thleng thin:

• Neuromas zawi zawia lo thang lian: Hengte pawh hi nerve zawh rualin an lo awm bawk.
• Hriatna lama harsatna: Hriatna lama harsatna a awm thei.
• Mit a inthlak danglam: Mit (cataract) ang chi thil a awm thei.
• Numbness emaw chak lohna emaw: I ke ruhte chu a hring (peripheral neuropathy) angin i inhria thei.

3. Schwannomatosis (SWN) natna a awm thei.

Hei hi a rarest type a ni . Hei hi subtype engemaw zat a awm a, chu chu genetic mutation a zirin a ni. A châng chuan symptoms a awm lo thei bawk. Mahse symptoms a lo lang tak tak a nih chuan thil thleng thei thenkhat chu hetiang hi a ni:

• Nerve tumor zawi zawia lo thang lian (Schwannomas):Chungte chu taksa peng pakhatah chauh a thleng thei.
• Natna rei tak a awm.
• Kut zungtang ah natna leh inflammation a awm.

Neurofibromatosis (NF) hi engtiang chiahin nge a awm?

A tlangpuiin NF1 hi NF case zawng zawng zinga 96% vel a ni. Chu chu khawvêl puma kum tin nau piang 3,000 zînga pakhat vêl chuan a nei tihna a ni. NF2 hian 3% vel a luah a, chu chu naute piang 33,000 zinga pakhat vel a ni. Schwannomatosis (SWN) hi a tam lo zawk a, 1% velin a awm thin.

Neurofibromatosis (NF) natna lan chhuah dan chu engte nge ni?

Kan sawi tawh ang khan NF chi hrang hrang a zirin symptoms a inang lo. Mi thenkhat chuan symptoms an nei lo tak tak thei a, thenkhat erawh chuan symptom na tak tak an nei thei thung. Mahse, chi thum zinga thil langsar tak pahnih a awm a, chungte chu:

• Tumors: Hei hi cell inzawmkhawm atanga lo piang chhuak tissue lumps a ni. NF tumor chi hrang hrang hi cell chi hrang hrang atanga siam a ni. Heng tumor te hi a lo thang zawi zawi fo a, cancer (benign) an ni lo . Mahse, a tlem hle a, tumor thenkhat chu cancer a ni thei. Heng tumor te hi nerve a lo awm thin te hi Neurofibromas an ti a .

• Vun thanna: Chung zingah chuan pianpui chhinchhiahna, kan sawi tawh ang chi café au lait spot te, chubakah armpit leh groin area-a moles lo awm te pawh a tel. A châng chuan vun chung lamah hian lump te tak te, nêm, pea ang maia lian (cutaneous neurofibromas) a lo awm thei bawk.

A pawimawh ber chu hetiang spot pahnih khat i neih vang mai maia NF i nei tih hlau loh hi a ni. Amaherawhchu, rinhlelhna i neih chuan damdawi lam thurawn lak a tha ber.

Heng symptom lian ber berte bakah hian symptom dang a lo lang thei bawk:

• Hriatna emaw, mit hmuhna emaw a hloh.
• Scoliosis natna (Scoliosis) a ni.
• Muscle chak lohna.
• Ke ruh leh ke ruh (limbs) emaw, tingling emaw.
• Taksa na leh lu na.
• Nungchang inthlak danglamna, Attention-Deficit/Hyperactivity Disorder (ADHD) ang chi te.
• Zirna kawngah harsatna a awm.
• Seizures ang chi condition te.

Neurofibromatosis (NF) vei chu eng ang nge a nih?

Hei hi mi hrang hrangah a inang lo tak zet a ni. Mi thenkhat chuan an vunah hnah te te, zungbun (centimeter khat vel a sei, pea ang vel) an hmu thei. Chungte chu neurofibromas an ni. Mi thenkhat chuan heng lump pahnih aia tam an nei thei a, a nih loh leh vun hnuaia nerve engemaw zat atanga siam lump lian tak (plexiform neurofibroma) an nei thei bawk.

Café au lait spot chungchang kan sawi dun a. Hengte hi a lian leh a pianziaah chuan flat, light brown atanga dark brown thlengin a awm thei. Heng hmun paruk emaw a aia tam emaw hi i hmu tlangpui ang.

Kan hmaiah hian spot a awm hi thil pangngai a ni. Mahse NF ah chuan heng spot te hi armpits leh groin area ah a lang thin.Hengte hi a tam ber a ni. Dot te te, sen hring hring ang maiin an lang.

Kum engzat nge Neurofibromatosis (NF) symptoms hi a lo lang thin?

Hei pawh hi mi hrang hrangah a inang lo. Nau piang lai hian symptom thenkhat a awm thei. Ṭhenkhat chu i lo upat deuh deuh emaw, i tleirâwl lai emaw, i puitlin hunah emaw an lo lang thei a ni. Entirnan, naute thenkhat pian tirh lamah an vunah neurofibromas a awm thei. Mahse a tam zawk chu tleirawl laiah an lang thin. Café au lait spot hi naupang dam chhung kum hmasa lamah chuan a awm fo thin. Kum 3 leh 5 inkar velah hian groin leh armpit-ah freckles a lang thei a, schwannomatosis symptoms hi puitling atanga tan a ni tlangpui a, kum 30 vel a ni.

Eng thilin nge Neurofibromatosis (NF) hi a thlen?

Hei hi a chhan ber chu kan genes inthlak danglamna (mutation) vang a ni . Type tin hi eng thilin nge a chhan hi han en ila:

• Neurofibromatosis type 1 (NF1): Kan taksaah hian NF1 an tih gene kan nei a. Hei hian protein pakhat neurofibromin tih chu a control a ni. He protein hnathawh hi tumor siam chhuah tihtawp a ni.
• NF2-associated schwannomatosis/Neurofibromatosis type 2 (NF2): Hei hi NF2 (merlin) tih gene atanga lo chhuak a ni. Hei hian protein dang neurofibromin tih chu a control bawk. He protein hian tumor siamna pawh a titawp bawk.
• Schwannomatosis: Hei hi gene SMARCB1 emaw LZTR1 emaw a mutations vang a ni thei. Mahse, thil tam takah chuan he natna thlentu gene dik tak hi hriat theih a ni lo.

A awlsam zawngin, heng gene pali zinga pakhatah hian mutation a awm a nih chuan, protein-te hian kan cell-te thanlenna control nan a mamawh thupek an dawng lo. Chutah chuan taksaah tumor a lo awm tan ta a ni.

I nu leh pate hnen atangin NF1 emaw NF2 emaw i rochun thei a, chu chu autosomal dominant pattern an ti a ni. Hei hian a awmzia chu he natna hi i neih theih nan i nu leh pa pakhat hnen atangin altered gene copy chauh i lak a ngai tihna a ni. Mahse, NF1 vei zinga a chanve vel chuan anmahniin an vei a, chhungkaw history engmah an nei lo. Hei hi NF2 vei te chungah pawh a thleng thei bawk. Schwannomatosis vei zinga 85% vel chuan anmahniin he natna hi an nei a, chhungkaw history engmah an nei lo.

Neurofibromatosis (NF) vei theihna tur risk factors te chu engte nge ni?

Hetiang dinhmun hi tu chungah pawh a awm thei a, mahse i chhungkuaa mi tu emaw hian heng NF natna zinga pakhat a neih chuan nang pawhin i vei theihna a sang zawk.

Neurofibromatosis (NF) natna avanga harsatna awm thei te chu engte nge ni?

NF hian harsatna engemaw zat a thlen thei. Chung zinga ṭhenkhat chu:

• Hriatna hloh.
• Mit hmuh a tlahniam.
• Natna awm reng.
• Zirna leh nungchang lama harsatna a awm.
• Cardiovascular conditions - entirnan, hypertension, pianpui thinlung natna.
• Vun natna avanga mahni inngaihsakna (self-esteem issues) a awm.
• Mipui zawng zawng aiin cancer vei theihna a sang zawk a, chutah chuan hnute cancer leh soft tissue cancer (sarcoma) te pawh a tel.

Pawimawh: NF tumor tam zawk hi cancer a ni lo. Mahse, a tlem hle a, NF tumor thenkhat chu cancer a ni thei. Chuvangin medical checkup neih reng a pawimawh hle.

Engtin nge Neurofibromatosis (NF) hi an hriat theih?

Doctor in a enfiah ang che a, NF hriat theihna tur test tulte a ti ang che. I vun an enfiah hmasa ang a, café au lait spot emaw neurofibromas emaw a awm leh awm loh an enfiah ang. Tin, scoliosis, thisen sang, mit leh hriatna te an enfiah bawk ang. I hriselna leh chhungkaw medical history te pawh an zawt ang. Chuvangin, i chhungkuaa mi tu emaw chuan NF an nei tih i hriat chuan i doctor hnenah hrilh ngei ang che.

NF chi hrang hrang hriat theihna tur tehfung hi a inang lo hle. Clinical examination hmangin i natna hriat theih fo mah se, test ṭhenkhat chuan i natna lan chhuah chhan hriat theih nân a ṭanpui thei a ni. Imaging test, MRI, X-ray, emaw CT scan emaw hmangin he natna hian i nervous system a nghawng dan a tarlang thei a ni. Genetic testing hian i symptoms thlentu gene mutation hriatchhuahna kawngah pawh a pui thei bawk. Mahse, doctor-te chuan he natna thlentu gene zawng zawng hi an la hmuchhuak vek lo.

A châng chuan, a lan chhuah hnu kum tam tak hnuah chauh symptoms hi hriat theih a ni lo. Mi thenkhat chu puitling anga hmuhchhuah an ni. Hei hi a chhan chu NF symptoms hi hun kal zelah, stage hrang hrangah a lo awm thin a ni.

Neurofibromatosis (NF) hi eng enkawlna nge ni?

Hei hi a pawimawh hle: I NF enkawlna chu NF damlote chungchanga thiamna nei doctor-te multidisciplinary team kaihhruaina hnuaiah a ni tur a ni . He team-ah hian a tlangpuiin:

• Neuro-oncologist te an ni.
• Neurosurgeon te an ni.
• Beng, hnar leh ril surgeon (ENT surgeon) te.
• Plastic surgeon te an ni.
• Psychotherapist te pawh an awm.
• Audiologist te an ni.
• Genetic counselor te an ni.

Tunah hian NF damdawi awm lo mah se, NF kaihhnawih tumor hmuhchhuah leh enkawl kawngah hmasawnna tam tak a awm a ni. I doctor chuan i dinhmun enkawlna tha ber tur a kaihruai ang che.

I natna lanchhuahna i neih loh chuan, emaw, i natna lan chhuah danin i nitin nun a tibuai lo a nih chuan enkawlna engmah i mamawh lo mai thei. Chutiang a nih chuan i doctor chuan kum khatah vawi khat emaw vawi hnih emaw checkup turin a rawn lut ang a, natna kal zel dan enfiah turin a ti ang che.

Doctor chuan enkawlna hrang hrang a rawt thei a, chungte chu:

• Tumor lakchhuah dan:Surgery hian vun leh taksa hmun danga tumor awmte chu a paih chhuak thei a ni.
• Damdawi: US Food and Drug Administration (FDA) chuan US Food and Drug Administration (FDA) chuan naupang kum 2 leh 18 inkar NF1 vei, plexiform neurofibroma tumor nei, operation hmanga lakchhuah theih loh, emaw, an tumor avanga rualbanlo emaw, pianphung tichhe thei emaw, tumor cells thanna tihtawp nan a pawm a ni.
• Ruh than dan dik lo siamthatna tur operation: Scoliosis emaw, ruh than dan dik lo dang emaw i neih chuan i doctor chuan brace emaw, a nasat chuan operation emaw a rawt thei ang.
• Chemotherapy: He enkawlna hi malignant tumor tan pek thin a ni. Chemotherapy hian cancer cell a tichhia a.
• Radiation therapy: Radiation therapy hmang hian cancer, hnute cancer, soft tissue cancer sarcoma an tih, malignant peripheral nerve sheath tumor (MPNST), emaw glioma (thluak tumor) te tumor thanna control theih a ni.

I hriatna emaw, i mit hmuhna emaw NF-in a nghawng a nih chuan i doctor chuan assistive devices, hearing aids emaw corrective lens emaw a rawn rawt thei ang.

Enkawlna hian side effect a nei em?

Enkawlna zawng zawng hian side effect awm thei a nei vek. I doctor chuan enkawlna i tan hmain hengte hi a sawipui ang che.

Surgery avanga side effect awm thei te:

• Thisen chhuak.
• Kaichhawng.
• Neurofibromas te chu lakchhuah hnuah a lo let leh thin.
• Nerve tihchhiat a ni.

Chemotherapy hmanga enkawlna tha lo (side effects) te:

• Chau.
• Diarrhea emaw constipation emaw a awm thei.
• Sam tlakchhamna.
• Chaw chu a thlum lo.
• Nausea leh luak chhuak.

Neurofibromatosis (NF) vei dam rei zawng chu engzat nge ni ang?

Neurofibromatosis (NF) hian i dam rei zawngah nghawng lian tham a nei lo tlangpui. Mahse, tumor awmna hmun azirin nitin i thiltih thenkhat, hriatna leh mit hmuhna te chu tanpuina tel lo chuan tih harsa tak a ni thei. Enkawl loh chuan harsatna thenkhat, cancer ang chi te chu nunna atana hlauhawm tak a ni thei.

Neurofibromatosis (NF) hi a veng thei em?

Tunah hian NF ven dan tur hriat a la awm lo. Chhungkaw din i tum a nih chuan genetic condition nei fa neih a hlauhawmzia hriat belh duh chuan genetic counselor nen inbiakna neih a tha.

Engtikah nge Doctor ka hmuh ang?

Nangmah emaw i fain emaw an vunah heng NF symptoms te hi i hmuh chuan doctor pan rawh:

• Café au lait spot paruk emaw a aia tam emaw neih.
• Skin lumps hi chhan awm lovin a lo lang thin.
• Armpit emaw groin emaw-ah spot awm.

Doctor hmuh ngai symptom dang:

• I hriatna leh/ emaw i mit hmuhna lama inthlak danglamna.
• Chhan awm lova hrehawmna.
• Muscle chak lohna.
• Kut zungtang leh ke ruhte a hring emaw, a hrual emaw.

NF i neih chuan i doctor chuan i natna lan chhuah dan enfiah turin regular checkup (a tlangpuiin thla 6 atanga thla 12 danah) nei turin a rawn rawt mai thei. Symptom thar i neih chuan emaw, symptom awm tawh chu a na zual emaw a nih chuan appointment dang siam belh ngei ngei tur a ni.

Doctor hnenah eng zawhna nge ka zawh ang?

• Eng thilin nge ka natna lan chhuah chhan?
• Ka nakin lawka ka fate’n he dinhmun hi an rochun theihna tur hlauhawmna chu eng nge ni?
• Eng ang enkawlna nge i rawt?
• Enkawlna hian side effect a nei em?
• Follow-up test atan engzat nge ka lo kal ang?
• Clinical trial ka hmuh theih tur a awm em?
• Fertility specialist ka hmuh tur em ni?

Neurofibromatosis hi i nervous system leh vun tichhe thei natna a ni. Kawng hrang hrangin a nghawng thei che a ni. Nitin hnathawh tibuaitu symptom i nei thei a, symptom i nei lo thei bawk. Vawi tam tak chu, i kum upat deuh deuha i natna lan chhuah dan azirin, official diagnosis hmuh theih nan kum tam tak a ngai thei. He diagnosis hi i hmuh hnu chuan thil awmdan dik tak hriat chu hahdamna nasa tak a ni thei. I medical team chuan he natna hian nangmah leh i chhungkaw hmalam hun tur a nghawng theih dan tur hriat belh turin an pui thei che a ni. Damdawi awm lo mah se, enkawl dan tur a awm.

Hriat reng tur pawimawh ber (Take-Home Message) .

Neurofibromatosis (NF) hi hlauh tur a ni lova, fimkhur tur a ni.

• I vunah hmun danglam tak tak, i vunah lumps, emaw i armpits/groin-a spot tam tak i neih chuan doctor pan rawh .
• NF chi hrang hrang a awm a, a lan chhuah dan pawh a inang lo vek.
• Hei hi genetic condition a ni a, mahse chhungkuaah a kal ngai lo.
• Damdawi awm lo mah se, natna lan chhuah dan tihtlem leh nun ṭha zawk neih theihna tura enkawlna tam tak a awm a ni .
• Specialist medical team enkawlna hnuaia enkawl hi a pawimawh hle.
• Medical checkup neih fo a pawimawh a, natna lanchhuahna thar laka fimkhur a pawimawh hle.

Hemi chungchanga zawhna dang i neih chuan doctor nen inbiakna neih hreh suh. An lawm hle ang che.

` Neurofibromatosis, NF, nerve tumor, café-au-lait spot, genetic natna, vun spot, nervous system