I vun hi ni êng lakah a sensitive hle em? Hei hi (Xeroderma Pigmentosum - XP) pawh a ni thei!

Vawiin hian hriselna dinhmun hriat tlem, mahse pawimawh tak tak kan sawi dawn a ni. Mi thenkhat chu ni chhuahna tlemte hnuah pawh ni zung an kang a, an sen thin tih i hmu tawh em? A châng chuan naupang tê tête chungah pawh hetiang hi a thleng ṭhin. Hei hi a chhan awm thei pakhat chu Xeroderma Pigmentosum , a tawi zawngin XP an tih hi a ni. Vawiin hian eng nge a nih, engvangin nge a lo awm tih leh engtin nge kan nunpui theih ang tih kan sawi mai dawn a ni.

Hei (XP) hian eng nge a awmzia? I hrethiam ang u!

A awlsam zawngin, Xeroderma Pigmentosum (XP) hi genetic condition hmuh tur awm lo tak a ni . "Genetic" tih thumal i hriat hian hetiang hi i ngaihtuah mai thei. Chu chu kan nu leh pate hnen atanga kan rochun, a nih loh leh an pahnih atanga kan rochun thil a ni tihna a ni. Hetiang natna vei te hian ni atanga ultraviolet (UV) rays lakah vun an sensitive hle. A dik tak chuan an vun hian heng (UV) rays te hi a hip harsa hle.

A tlangpuiin he (XP) natna lan chhuahna hi taksa peng ni chhuahna hmuna awm fo thinah a lang thin. Entîr nân, hmai, kut, leh hmuiah . Vawi tam tak chu, heng chhinchhiahnate hi kum naupang tê aṭangin a lang ṭan a, chu chu nausen lai a ni . Ngaihtuah teh, naupang tê tê pawh minute tlemte chauh ni chhuahnaah an awm thei a, an vun chu a hring chhuak thei a, a kang thei bawk (blistering sunburns).

(XP) hi cancer a ni em? Hei hi mi tam takin zawhna an neih a ni!

Ni lo, XP hi direct cancer a ni lo . Mahse hetah hian a pawimawh ber chu a awm. XP vei hian midang aiin vun cancer a vei theihna a sang zawk hle .

Hei hi thawnthu hlauhawm deuh a nih laiin, fimkhur a pawimawh hle. Heng statistics te hi lo en ve teh:

• XP vei chu mi pangngai aiin nonmelanoma skin cancer , basal cell carcinoma emaw squamous cell carcinoma emaw ang chi a vei theihna chu a let 10,000 velin a tam zawk a ni .
• Tin, vun cancer na tak melanoma vei theihna pawh a let 2,000 velin a sang zawk bawk .

Heng vun cancer bakah hian zirchianna thenkhatah chuan XP vei te hian cancer chi dang an vei theihna chance a sang zawk tih hmuhchhuah a ni. Entir nan:

• Thluak natna (Astrocytoma) , (Glioblastoma) te hi a awm a.
• Hnute cancer a ni
• Kidney cancer a ni
• Leukemia natna (Leukemia) a ni
• Lung cancer a ni
• Pancreatic cancer natna a ni
• Pum cancer a ni
• Testicular cancer a ni
• Thyroid cancer a ni
• Uterine cancer a ni

He list hian hlauthawng suh. Heng cancer zawng zawng hi (XP) damlo zawng zawngah a lo awm vek dawn lo. Mahse, a hlauhawmna hriat chian a pawimawh hle.

Hetiang dinhmun hi engtiang chiahin nge a awm?

Xeroderma Pigmentosum hi a awm lo hle a ni . United States leh Europe ang ramah chuan mi maktaduai khat zinga pakhat chauhvin he natna hi an vei nia chhut a ni (XP).

Mahse, khawvel hmun thenkhatah chuan a hluar zawk. Entîr nân, Japan ramah chuan he natna hian mi 22,000 zînga pakhat vêl a nghawng a ni. North Africa leh Middle East-ah pawh a awm ve deuh hlek a ni. Sri Lanka-a a hluar dan data chiang tak awm lo mah se, natna hmuh tur awm lo anga ngaih a ni.

(XP) natna lan chhuah dan chu engte nge ni? I ti chhe vek ang u!

(XP) Mi hrang hrangah natna lan chhuah dan a inang lo thei a, mahse vun, mit leh nervous system a nghawng ber a ni .

Vun natna lan chhuah dan

Hengte hi a lan chhuah tam berte chu an ni:

• Blistering sunburns: Hun rei vak lo ni a kan awm hnuah pawh vun a blister a, a kang bawk.
• Dry, thin skin (Xerosis): Vun hi a vawt hle a, parchment paper ang maiin a hring thei bawk.
• Kum 2 an tlin hmaa freckles (Lentigos): A tlangpuiin naupang te te hian hetiang hian freckles an nei hma lo.
• Patches of increased and decreased skin pigment (Poikiloderma): A hmun thenkhat chu a sen a ni thei a, a then chu a var a ni thei bawk.
• Vun a ti hring (Atrophy).
• Thisen kalna kawng zau lutuk avanga vunah line sen - Telangiectasia: A chung lamah thisen kalna kawng te te a hmuh theih avangin line sen angin a lang.

Chu bâkah, XP vei te chu vun natna chi hrang hrang actinic keratoses an tih chu an vei theihna a sang hle a, chu chu cancer a ni thei bawk .

Mit natna lan chhuah dan

Vawi tam tak chu XP vei te hian kum 10 an tlin hmain mit lam natna an nei thei :

• Mit a vawt.
• Mitmeng a chhe/atrophy.
• I mit (cornea) a inflammation (Keratitis) a awm.
• I mit pawn lam layer-a transparency awm lohna - Cornea opacification: Hei hian mit darthlalang ang chi chu a thim tihna a ni.
• Light sensitivity (Photophobia): Eng tlemte pawh tuar harsa.
• Mitmeng hloh.

Hun a kal zel a, heng chhinchhiahnate hian mitdelna a thlen thei a, mitdelna pawh a thlen thei bawk . XP vei te pawh mit cancer vei an hlauhawm hle .

Neurological symptoms a awm thei

XP hian i nervous system a nghawng thei bawk. XP vei zinga hmun li a\anga hmun khat vel chuan nervous system nena inzawm symptoms an nei ang. Entirnan:

• Dysphagia - ei harsat.
• Reflexes a hloh.
• Muscle control tha lo (Ataxia) leh stiffness (Spasticity) te a awm bawk.
• Ngaihtuahna (cognitive) thiamna hloh zel.
• Inner ear nerve chhiatna avanga hriatna hloh zual zel.
• Lu lian lo zawk (Microcephaly).
• Vocal cord a tichhe thei.

Mithiamte chuan heng symptoms te hi thluaka nerve cells te tihchhiat avanga lo awm niin an ngai.

(XP) siam chhan hi eng nge ni?

Kan sawi tawh ang khan Xeroderma Pigmentosum hi genetic disease a ni . Chumi awmzia chu he dinhmun hi kan taksa chhunga genes-a inthlak danglamna (mutations) danglam tak tak thenkhat avanga lo awm a ni tihna a ni. Heng genetic mutations te hi i nu emaw, i pa emaw, an pahnih atanga rochun theih a ni. A awlsam zawngin sawi ila, UV rays avanga kan vun chhiatna siamthatna tura puitu genes chak lohna avanga lo awm a ni.

Engtin nge (XP) ka neih leh neih loh ka hriat theih ang?

I doctor chuan i symptoms a zirin XP i nei tih a ringhlel mai thei. An rinhlelhna finfiah nan thisen test an nei ang . He thisen test hian Xeroderma Pigmentosum thlentu gene mutations a awm leh awm loh a enfiah thin.

(XP) damdawi kimchang a awm em?

Vanduaithlak takin tunah hian Xeroderma Pigmentosum hi damdawi a la awm lo . Mahse, i doctor chuan natna lan chhuahna tihziaawmna tur leh i nun awlsam zawka siam turin enkawlna chi hrang hrang a rawt thei ang:

• Eye drops: Mit var a inflammation tihziaawm nan lubricating eye drops pek theih a ni.
• Hearing aids: Hriatna lama harsatna a awm chuan hearing aids pek theih a ni. In some severe cases , cochlear implants pawh hman a tha thei.
• Surgery: Vun cancer i vei chuan a paih chhuah theih nan surgery a ngai mai thei. A nih loh leh mit natna thenkhat, mitmeng tlak (ptosis) emaw, mit var lama harsatna emaw enkawl nan operation i mamawh thei bawk. In some severe cases , mit (cornea transplant) a ngai mai thei.
• Vitamin D tihpunna tur:Mi tam zawk chuan ni eng leh ei leh in atanga vitamin D an dawng thin. XP vei te hian ni êng hi an pumpelh vek a ngai a, chuvangin vitamin D supplement an ei a ngai a ni.

XP hi ven theih a ni em?

XP hi genetic condition a nih avangin a lo awm tur venna tur a awm lo . I doctor-in XP thlentu gene mutation i neih a rinhlelh chuan genetic testing i nei thei ang .

Genetic test hmangin gene mutation i neih leh neih loh a hriat theih. Genetic counselor chuan a result te, a hlauhawmna te, leh XP nei fa neih theihna chance te a hrilhfiah ang che.

(XP) nen kan awm dun hian eng nge i ngaihven tur?

Xeroderma Pigmentosum i neih chuan i hriselna zawng zawng vawng reng turin thil tlemte i ngaihven ngei ngei tur a awm. Hengte hi a pawimawh ber a ni:

• Sun protection: Hei hi XP enkawlna atana pawimawh ber a ni . UV rays laka i vun venhim nan a pawimawh hle.
• Ni laka invenna thawmhnaw ha rawh . Hei hian kawr kawr sei, kawr sei, socks, leh lukhum a kawk a ni.
• UV-blocking sunglass vuah rawh .
• Nitin SPF 35+ aia sang nei broad-spectrum sunscreen hmang thin ang che. Ni chhuak angin lang lo mahse, UV rays hian chhum kaltlangin a la thleng thei tho.
• Mithiamte chuan UV light meter hmangin i chhehvel khawiah nge UV rays a tlakbuak ber tih hriat a, pumpelh turin an rawt a ni. I in chhunga light thenkhat pawhin UV rays a pe chhuak thei bawk a, chuvangin chu pawh chu hre reng ang che.

• Mit enfiah: Mit lam doctor pan la , i mit enfiah fo ang che. Mitmeng tlak leh mit hring ang chi thil a awm leh awm loh a enfiah ang. Kum khatah vawi khat tal i mit enfiah tir la, a nih loh leh doctor-in a rawt angin a tam zawk.

• Skin check: Thla 6 atanga 12 danah dermatologist hmu thin ang che, a nih loh leh i doctor-in a rawt angin a aia tam. Cancer emaw, cancer lo ni thei moles emaw a awm leh awm loh an enfiah ang. Doctor office-a i awm lai hian thla khatah vawi khat tal i vun enfiah thin ang che . Spot thar emaw, lump thar emaw i hmuh chuan dermatologist pan nghal rawh.

• Neurological care: (XP) vei te chuan neurologist an pan tur a ni.An reflexes, hearing, etc. enfiah turin i hmu fo tur a ni a, doctor chuan heng hnathawhnaah hian inthlak danglamna a hriat chuan, an natna zual zel dan tur control emaw, tihkhawtlai emaw nan enkawlna a rawt ang.

Heng zawng zawng hi mahni invenna tur a ni tih hre reng ang che. Hengte hi uluk taka i zawm chuan (XP) nen pawh nun hlawhtling takin i nung thei ang.

(XP) vei dam chhung hi engtin nge i sawi theih ang?

Xeroderma Pigmentosum vei dam rei zawng hi mi hrang hrangah a inang lo thei. Thil tam takah chuan vun cancer hi kum naupang tak atangin hriat theih a ni a, chu chuan dam chhung a ti tawi thei a ni. Mahse, naupan lai aṭanga ni zungin vun a tihchhiat loh nan hma i lak chuan dam rei theihna chance a tipung thei a ni . Chuvangin a chunga kan sawi tak himna dan zawm hi a pawimawh hle a ni.

I doctor zawh tur zawhna

Nang emaw i fa emaw hian Xeroderma Pigmentosum i vei a nih chuan, emaw, i vei nia i rinhlelh chuan i doctor hnenah heng zawhnate hi i zawt thei ang:

• Skin cancer ka vei theihna tur tihtlem nan eng nge ka tih theih ang?
• Eng ang cancer screening nge tih tur?
• Vun cancer chhinchhiahna hmasa berte chu engte nge ni?
• He diagnosis hian engtin nge ka chhungkaw dangte a nghawng ang?
• Genetic test hi ka ngaihtuah tur em ni?
• (Xeroderma Pigmentosum) nei fa dang ka neih theihna chance engzat nge awm?

A tawp berah chuan thil hriat reng tur

Okay, chuti a nih chuan kan sawi thin natna (Xeroderma Pigmentosum - XP) hi a awm lo, genetic condition a ni. Hei hi a langsar ber chu (UV) rays laka sensitivity nasa tak a ni. Mi tam tak chuan mit leh nervous system hmasawnna lama harsatna an nei bawk. (XP) i neih chuan ni êng tam thei ang ber, chu chu (UV) êng, a theih ang anga i pumpelh tur a ni . Ni laka i vun venhim leh vun cancer screening neih fo hi i hriselna atana i chance tha ber a ni.

He thu hi in tan a tangkai ngei ka beisei. Zawhna i neih chuan doctor zawh hreh suh. An pui ang che.

` Xeroderma Pigmentosum, XP, vun natna, genetic natna, ni sensitivity, vun cancer, UV rays