Hnar thisen pawh i nei fo em? HHT (Hereditary Hemorrhagic Telangiectasia) a ni thei!

Hnar thisen i nei fo em? A nih loh leh, i taksa peng ṭhenkhatah, entîr nân, i hmaiah, i hmuiah, a nih loh leh i kut zungtang-ah te, a sen te te i hmu em? A châng chuan hengte hi thil pangngai angin kan ngai lo va, mahse a hnung lamah hian damdawi lam thil engemaw, ngaihven ngai tak a awm thei a ni. Chu chu HHT chu a ni a, mahse mi tam takin an hre lo. Vawiin hian he HHT, a nih loh leh damdawi lam atanga thlir chuan `(Hereditary Hemorrhagic Telangiectasia)`, thlahtu natna hi kan sawi dawn a ni. Ṭhenkhat chuan `(Osler-Weber-Rendu syndrome)` an ti bawk.

HHT hi eng nge ni tak tak?

A awlsam zawngin sawi ila, HHT hi genetic disease a ni a, kan taksa chhunga thisen kalna kawngte a nghawng a ni. I hria em, kan taksa pum puia thisen phurtu heng tube te te hi kan nei a, chu chu thisen kalna kawng kan ti a ni. Hetiang dinhmunah hian thil thleng chu heng thisen kalna kawngte, a bik takin capillaries an tih te hi a tha lo hle a ni. Heng capillary siam danglam tak takte hi ``telangiectasias'' kan ti thin.

Ngaihtuah teh, kan thinlung atanga kan taksa peng dang thlenga thisen phur thei thisen kalna kawng lian tak tak kan nei a, chu thisen chu thinlung lam pan lehtu vein kan nei bawk. Heng pahnih inzawmna hi capillaries an tih hian a siam a ni. HHT-ah chuan a chang chuan heng arteries leh veins te hian direct-in inzawmna pangngai lo an nei thei a, capillaries kal tlang lovin. Hengte hi arteriovenous malformations, a tawi zawngin AVM kan ti thin.

Heng thisen kalna kawng pangngai lote hi a chak lo hle a, a chhe thei hle bawk. awlsam takin an puak emaw, an puak emaw thei a, thisen chhuah (hemorrhaging) a thlen thei a ni. Symptoms leh complications chu thisen chhuahna hmun a zirin a ni.

Tute nge HHT vei thei?

He HHT tia koh hian hmeichhia, mipa, leh naupang tê tê pawh a nghawng thei a ni . Hnam, sakhua leh vun rawng te hi he thil nen hian a inzawm lo. A chhan ber chu genes hmanga thlah khat atanga thlah dangah a kal zel a, chhungkaw zinga mi tu emaw chuan a neih chuan midang pawhin an neih theihna chance an nei ve tho.

Hei hi thil awm lo tak anga ngaih a ni . Mahse, a underdiagnose fo thin. Chumi awmzia chu, khawvel puma mi 5,000 zinga pakhat velin he natna hi an vei nia chhut ni mah se, a tam zawk chuan an vei tih an hre lo tihna a ni.

Eng thilin nge HHT hi a thlen?

Ka sawi tawh ang khan HHT hi genetic condition a ni vek a . Chumi awmzia chu nu leh pa atanga fate rochun a ni tihna a ni. ``Dominant disorder`` anga ngaih a ni. A awlsam zawngin, naupang chuan nu leh pa pakhat, nu emaw, pa emaw chauhvin gene pangngai lo chu rochun mah se, chu natna chu a vei thei a ni .

Scientist-te chuan HHT nena inzawm gene parukah mutation hrang hrang za tam tak an hmuchhuak a. Mahse, tun dinhmunah chuan mutations awm tam ber chu gene pahnih ENG leh ACVRL1 an tihah te a ni. Scientist ten he thil hi an la zirchiang mek a ni.

HHT natna lan chhuah dan chu engte nge ni?

HHT natna lan chhuah dan hi mi hrang hrangah a inang lo thei hle. Taksa chhunga ka sawi tawh thisen kalna kawng pangngai lo awmnaah a innghat. Mi thenkhat chuan symptom lian tham an nei lo mai thei. Mahse mi dangte chuan symptom na tak tak, na tak tak an nei thei.

Mahse, HHT vei zingah chuan a lan chhuah tam ber chu hnar thisen chhuak fo (epistaxis) a ni . Mi thenkhat chuan ni khatah vawi tam tak hnar thisen an nei thei. He harsatna hi naupan lai atang tawhin a awm tawh mai thei.

Tin, HHT vei thenkhat chuan an taksaah hian hmun sen te te (telangiectasias) an hmu thei bawk. Chungte chu i tan chuan a rawng a danglam deuhin a lang thei. Hengte hi a tam ber chu:

• Hmaiah chuan
• Kut zungtang emaw kut zungtang emaw ah
• Kut ah chuan
• Kawng chhungah (mucous membrane-ah) .
• A hmuiah chuan
• Hnar vel chu

Heng bakah hian HHT vei thenkhat chuan:

• Anemia : Hei hian taksaa thisen sen (red blood cells) a tlakchham tihna a ni. Hei hi thisen chhuah fo vang a ni thei.
• Pum emaw, intestines atanga thisen chhuak : Hei hi hmuh theih loh a ni thei a, mahse ek chhungah thisen a awm emaw, ek a dum emaw a ni thei.

Arteriovenous Malformations (AVMs) leh a nghawng dan te

HHT vei te hian a chang chuan thisen kalna kawng lian takah aneurysm (AVM) an nei thei a. Heng AVM te hi taksa peng hrang hrang, lung, thluak, ruhro, leh thin (liver) ah te a lo awm thei ber a ni. Chumi hnuah chuan taksa peng hrang hrang nena inzawm symptom hrang hrang an siam thei a ni.

• I lungah AVM i neih chuan:
• Vun (a bik takin hmui, kut zungtang) rawng dum (bluish discoloration) .
• Thisen khuh (hemoptysis) .
• Chau lutuk tih hriatna
• Thâwk harsa (dyspnea) .
• Thluak-ah `(AVMs)` i neih chuan: Hengte hi a hlauhawm deuh deuh a ni. A chhan chu heng `(AVM)` zinga pakhat hi thluak chhungah a puak chhuak a nih chuan a pawi hle mai.
• Luak chhuak thei
• Double vision hmuh theihna
• Seizures a awm thin
• Stroke ang chi hi a awm thei.
• Liver AVM i neih chuan:
• Thinlung hi ``Heart failure'' dinhmunah a lut thei a, a chhan chu thisen chu liver-a ``(AVM)'' kal tlanga a tlan chhuah hian taksa pum puia thisen pe turin thinlung hian nasa zawkin hna a thawk a ngai a ni.
• Spinal AVMs i neih chuan:
• Hnung lam natna
• Kut emaw ke emaw-ah hian rilru hah emaw, hriatna hloh emaw a awm thei.

Pawimawh: Heng ``(AVM)`` te hi an rupture a, thisen an chhuah chuan emergency pawi tak a ni. Chuvangin, a chunga kan sawi tak symptoms te hi i nei a nih chuan damdawi lam pan vat a pawimawh hle.

Ka fate pawhin HHT an vei thei em?

Ni e, HHT i neih chuan i fate hian natna hi an rochun theihna chance 50% an nei vek a ni.Awle. Chumi awmzia chu naupang a pian apiangin naupangin HHT a vei theihna chance 50% a awm a, a vei loh theihna chance 50% a awm tihna a ni. Coin flipping a, lu emaw tail emaw hmuh ang mai a ni.

Engtin nge HHT hi hmuhchhuah a nih?

HHT finfiah nan genetic testing tih theih a ni. Mahse a tam zawkah chuan doctor-te chuan clinical information, symptoms leh family history te hmangin an hmuchhuak thin. Doctor i hmuh hunah a hnuaia mi ang hian an ti fo ang:

• I mimal damdawi lam chanchin (e.g. eng chen nge hnar thisen i neih tawh, eng chen nge i neih tawh, leh eng harsatna dang nge i neih tawh) chungchang chipchiar takin an zawt ang che.
• I chhungte hnai ber (nu leh pa, unau, fate) medical history zawt la, a chhan chu hei hi chhungkuaah a kal zel a ni.
• I taksa chu enfiah a ni ang (a sen emaw chutiang chi emaw a awm leh awm loh en turin).
• A tul chuan test (e.g. `(scan)` types, `(Endoscopy)`) en la, internal organs dinhmun enfiah rawh .

Doctor chuan HHT i vei tih a ringhlel thei a, a hre thei bawk a, a hnuaia symptom pathum tal i neih chuan:

• Hnar thisen chhuak fo thin.
• Telangiectasias engemaw zat vunah hmuh tamna hmun (hmai, hmui, kut zungtang ang chi) a awm.
• Internal organs (e.g. lung, thluak, pum, intestines)-a ``telangiectasias'' emaw ``AVMs'' emaw awm (hengte hi test hmang chauha hmuh theih a ni).
• HHT nei chhungkaw hnai tak neih.

HHT enkawl dan tur chu engte nge ni?

Vanduaithlak takin HHT hi damdawi a la awm lo. Mahse, natna lan chhuah dan control thei, symptom tihtlem thei, leh harsatna lian tham thlen theihna tur tihziaawm thei enkawlna a awm . Scientist-te chuan HHT enkawlna tur zawngin an la thawk mek a, a bik takin anti-angiogenic medical therapies siam chhuah a nih avangin.

I doctor hian symptom awmsa a enkawl lai hian HHT nena inzawm harsatna, symptom la nei lo mai thei a awm leh awm loh a enfiah bawk ang.

Enkawlnaah hian a hnuaia mite hi a tel thei ang:

• Laser treatment (`(Ablation)`): Laser beam hmanga thisen chhuah tamna hmun (telangiectasias) tihtawp nan surgery tenau tak a ni.
• Embolization: Thisen kalna kawngkhar tikhawlo, thisen chhuahna hmun emaw AVM emaw, thisen chhuah hlauhawmna hmuna kalna tur surgery tenau tak.
• Anemia enkawl dan: Iron tablet pek, leh a tul chuan thisen (thisen pek) pek.
• Hnar thisen chhuak control nan: Hnar chhung lam a hnim theih nan ointment leh saline spray ang chi thil hmang thin ang che.
• AVM lakchhuah dan: AVM thenkhat chu radiation therapy emaw surgery emaw hmangin lakchhuah theih a ni.
• Anti-angiogenic medical therapies: Hengte hi infusion angin emaw, pill angin emaw pek theih a ni.

Taksa system bik, liver, lung, gastrointestinal system, leh thluak enkawlna tur specialist te pawh i hmu thei bawk.

HHT hi ven theih a ni em?

Genetic disease a nih avangin HHT venna tur emaw, i vei theihna tihziaawmna tur emaw a awm lo. Amaherawhchu, i nu leh pa, i unau, i fate hian HHT an vei a nih chuan i doctor hnenah i hrilh a pawimawh. Hei hian he natna hi i neih hmasak leh neih loh hriat theih nan a pui thei che a, enkawlna i tan hma thei a, harsatna i neih loh nan a pui thei bawk.

HHT vei te tan engtin nge an hmalam hun?

HHT vei te hian dam chhung pangngai hnaih tak an hmang thei a ni. Mahse, AVM leh lung leh thluaka thisen chhuak reng reng hi a nasat avangin enkawl a ngai a ni. Enkawlna mumal a awm chuan mi tam zawk chuan nun pangngaiin an hmang thei a ni.

HHT vanga hnar thisen chhuak tan eng nge tih tur?

HHT-ah hian hnar thisen chhuak hi a tam hle a, chuvangin a venna atana pui thei tur thil tlemte a awm a:

• Damdawi thenkhat, a bik takin natna tidamtu aspirin leh NSAID (e.g. ibuprofen, diclofenac) te hman loh tur . Chungte chuan thisen zam a tihziaawm bakah thisen chhuah a tipung thei bawk. Damdawi eng pawh i ei hmain doctor zawt hmasa phawt ang che.
• Diary ziak rawh. Eng ei tur nge i ei a, i hnar thisen chhuahna thlentu thiltih eng nge i tih tih ziak rawh. Tichuan chutiang thilte chu i pumpelh thei tawh ang.
• I hnar chhung lam chu hnim leh lubricated takin dah la. Hei hi humidifier hmanga tih theih a ni a, doctor-in a rawt ointment hmanga hnawih emaw, saline nasal spray hmanga tih theih a ni. Hnar vawt chuan thisen a chhuah tam zawk.

HHT chungchang hi ka doctor hnenah eng nge zawh belh theih ang?

HHT i vei tih hmuhchhuah a nih chuan doctor hnenah hetiang zawhna hi zawh a tha ang:

• Hemi chungchangah hian ka chhungte dang zawng zawng hi test an ni tur em ni?
• Sports ka khel thei ang em? Eng ang infiamna nge tha leh tha lo?
• Thil tih tur bik ka pumpelh tur a awm em?
• Zu, chaw bik, damdawi dang ka ei loh tur a awm em?
• Hnar thisen chhuah loh nan emaw, rang taka tihtawp nan eng nge ka tih theih ang?
• Naupai hi ka tan a him em? Thil bik ka hriat chian ngai a awm em?
• Thisen chhuak ka neih chuan engtikah nge damdawi lam pan vat tur?

A tawp berah chuan eng nge hriat reng tur

HHT hi genetic condition a ni a, hriat lohvin a awm fo thin. A lan chhuah dan chu hnar thisen chhuak fo atanga taksa system hrang hrang tichhe thei harsatna lian tak thlengin a awm thei. Nangmah emaw, i chhungte zinga tu emaw HHT vei nia i rinhlelh chuan doctor nen inbia ang che.Enkawl hmasak hian harsatna a awm loh nan a pui thei a ni. Genetic testing hian chhungkaw member dangte pawhin he natna hian a nghawng em tih hriat theih nan a pui thei bawk. Hetiang dinhmunah hian hriat reng hi a pawimawh ber a ni.

` HHT, Hereditary Hemorrhagic Telangiectasia, Osler-Weber-Rendu syndrome, hnar thisen chhuak, thisen kalna kawng, thlah natna, AVM, telangiectasia