Hnar thisen i nei fo em? A nih loh leh i vunah, a bik takin i hmai, i hmui leh i kut zungtang-ah te, sen hring te te i nei em? I nu emaw, i pa emaw, i chhungkuaa mi dang emaw pawhin heng harsatnate hi an lo nei tawh mai thei. Heng thilte hi kan ngaihven vak lo mai thei a, "Aw, chu chu kan thlah chauh a ni," kan ti mai thei. Mahse, a chang chuan he thil hi kan hriat tur damdawi lam thil a awm thei. Vawiin hian hetiang dinhmun hmuh tur awm lo mahse hriat tur pawimawh tak kan sawi dawn a ni. Chu chu HHT, a nih loh leh Hereditary Hemorrhagic Telangiectasia .
A awlsam zawngin, HHT chu eng nge ni?
HHT hi genetic condition a ni a, thlah khat atanga thlah dangah a kal zel a ni. Kan thisen kalna kawng, a nih loh leh thisen kalna kawng siam dan a nghawng ber a ni. Kan taksaa thisen kalna kawng (blood vessel system) hi kawngpui (network of road) angin han ngaihtuah teh. Highway lian ang maia artery a awm a, kawngpui lian ang maia vein a awm bawk. Heng kawngpui lian pahnih inzawmkhawmtu side road te tak te, te tak te te hi capillaries an ti a.
HHT vei pakhata thil thleng chu heng inzawmna nalh tak tak, capillaries an tih te hi an lo piang tha lo a ni. Chu ai chuan artery leh vein inkarah hian direct, irregular, weak connection a awm zawk. Damdawi lamah chuan heng inzawmna dik lo tak takte hi Arteriovenous Malformations (AVMs) kan ti thin.
Heng AVM te hi kan taksa peng hrang hrangah te, entirnan hnar, lung, intestine, thluak thlengin a lo awm thei a ni. AVM te tak te tak, vun chunglama lo piang chu Telangiectasia an ti a . Hengte hi i vun chhungril lama rawn lang sen te te, hmui ang chi te hi an ni. Heng thisen kalna kawng chak lote hi awlsam takin a puak thei a ni. An puak a, thisen an chhuah chuan a awmna hmun a zirin natna khirh tak pawh a thlen thei a ni.
A pawimawh ber chu HHT vei tam tak hi an hre lova kum tam tak an khawsa thin hi a ni. Damdawi awm lo mah se, tûn laiah hian natna lan chhuah dân thunun tûr leh natna khirh tak tak laka invenna tûr damdawi ṭha tam tak a awm a.
He natna hian khawvel puma mi 5,000 zinga pakhat vel a vei a ni. Mahse mi tam takin natna an hmuhchhuah loh avangin damlo an tam zawk mai thei niin an ngai. Kum eng pawha mi leh chi hrang hrangah a awm thei. Osler-Weber-Rendu syndrome an ti bawk.
HHT natna lan chhuah dan berte chu engte nge ni?
HHT natna lan chhuah dan hi mi hrang hrangah a inang lo thei. A hmaa kan sawi tawh thisen kalna kawng dik lo (AVM) te taksa chhunga khawiah nge a awm tih a innghat a ni. Mi thenkhat chuan natna lanchhuahna langsar tak tak an nei lo mai thei. Mahse mi dangte chuan symptom na tak tak an nei thei.
A hnuaia table-ah hian heng symptoms te hi categorize ila.
| Symptom chi hrang hrang | Hrilhfiahna leh entirnan |
|---|---|
| Mi tam tak tan chuan mizia tlangpui |
|
| Internal bleeding symptoms te | |
| AVMs avanga taksa peng lian tak tak, lung leh thluak-a natna lan chhuah dan | |
| Harsatna awm lo mahse na tak tak |
Engvangin nge HHT hi a lo awm?
Hei hi genetic vek a ni.Thil engemaw avanga lo awm a ni. Chu chu natna hrik kai theih a ni lo tihna a ni. Nu leh pa atanga fate rochun thil a ni. HHT hi natna chi khat, dominant gene (dominant disorder) avanga lo awm a ni. Chumi awmzia chu, i nu leh pa zinga pakhatin he natna hi a neih chuan nang pawhin he natna hi i vei theihna chance 50% i nei tihna a ni.
HHT hi gene pahnih (`ENG` gene leh `ACVRL1` gene)-a mutations avanga lo awm a ni a, scientist-te chuan he thil hi an la zirchiang mek a ni.
HHT i vei a nih chuan i fate’n an rochun theih dan tur chungchangah doctor nen inbiakna neih a pawimawh hle.
Engtin nge HHT hi hmuhchhuah a nih?
Doctor chuan i natna lan chhuah dan leh chhungkaw chanchin a hriat hnuah hei hi a ringhlel mai thei. Genetic testing hmanga natna hriat chian theih ni mah se, a natna lan chhuah dan a zirin hriat theih a ni fo.
I doctor chuan HHT i vei tih a ringhlel ang a, a hnuaia mi pathum tal i neih chuan:
- Hnar thisen chhuak nawn leh thin.
- Vun chhunga spot awm tamna hmun (hmai, hmui, kut zungtang)-ah telangiectasias tam tak awmna.
- Taksa chhunga taksa peng pakhat (lung, thluak, thin ang chi)-a AVM emaw Telangiectasia emaw neih.
- HHT nei chhungkaw hnai tak (nu, pa, unaupa) neih.
Natna finfiah nan test neih a ni
I doctor chuan test thenkhat tihsak turin a rawt thei che a, chungte chu:
- Ultrasound Scan: Hei hian liver-ah AVM a awm em tih hriat nan a pui a ni.
- MRI (Magnetic Resonance Imaging) Scan: A tangkai hle a, a bik takin thluaka AVM awm leh awm loh enfiah nan a tangkai hle.
- CT (Computed Tomography) Scan: Taksa chhunga taksa peng hrang hrangte thlalak chiang zawk a hmu thei.
- Bubble Test (Echocardiogram): Hei hi test bik a ni. Chu chu lungah AVM a awm leh awm loh hriat nan hman a ni.
HHT enkawl dan tur chu engte nge ni?
Kan hriat reng tur chu HHT damdawi a awm lo tih hi a ni. Mahse, enkawlna tha tak tak a awm a, chu chuan natna lan chhuah dan a thunun thei a, harsatna lian tham a thlen theihna a ti tlem a, nun pangngaia i nun theih nan a pui thei bawk.
Tuna i natna lan chhuah dan i enkawl lai hian i doctor chuan AVM thup, chhinchhiahna la nei lo mai thei te pawh a enfiah bawk ang.
Enkawl dan thenkhat chu hetiang hi a ni:
- Hnar thisen chhuak fo tan: Hnar a hnim reng theih nan ointment leh saline spray hmang thin ang che.
- Anemia tan: Iron thlakna pill emaw, a tul chuan thisen pek emaw pe thin ang che.
- Thisen chhuah tihtawp nan: Laser hmanga enkawlna (`Ablation`) hian thisen kalna kawng te tak te, thisen chhuak mekte chu a tichhia a ni.
- Embolization: Hei hian thisen kalna kawng khap a, chu chuan AVM a siam a, chu chu thisen chhuah emaw, thil bik hmanga puak emaw hlauhawm tak a ni.
- Surgery emaw radiation therapy: Hengte hi AVM thenkhat lakchhuah emaw tihtlem emaw nan an hmang thin.
- Damdawi thenkhat tihtawp: Thisen zam tihtlemtu aspirin ang chi damdawi hi damdawi lam thurawn angin tihtawp a ngai mai thei.
Pawimawh tak: HHT i neih chuan, a bik takin i lungah AVM i nei tih i hriat chuan ha zai hmain antibiotics ei a ngai mai thei, ha lakchhuah ang chi. Hei hi thluaka natna hrik (brain abscesses) ang chi natna laka inven nan a ni. Hemi chungchang hi i doctor nen inbia ngei ang che.
HHT nena kan khawsak huna ngaihtuah tur thil
HHT vei chuan dam chhung zawng damdawi lam enkawl leh enkawl a ngai mai thei a, mahse enkawl dan dik a nih chuan HHT vei te hian dam rei zawng pangngai an nei thei a ni.
Hnar thisen chhuak lo turin eng nge tih theih ang?
- I doctor nen inbia la, thisen chhuahna tipung thei damdawi (e.g. aspirin, NSAIDs) ei loh tur.
- Engtik lai pawhin i hnar chu a hnim reng tur a ni. In lama humidifier hman te, saline nasal spray hman te, doctor-in ointment a pek te hnawih te hi a pawimawh hle.
- Chaw emaw, thil tih emaw hian hnar thisen chhuah theihna a tipung em tih en turin diary ziak la.
HHT i vei tih hmuhchhuah a nih chuan i chhungkaw dangte pawh test turin thurawn pek a pawimawh hle a, a chhan chu natna hi i hriat thuai poh leh harsatna lian zawk a awm theih nan a veng thei a ni.
Take-Home Thuchah a ni
- HHT hi genetic condition a ni a, thisen kalna kawngah chak lohna a thlen thin.
- A lan chhuah dan ber chu hnar thisen chhuak fo leh vun, hmui leh kut zungtang-a hmun sen (telangiectasias) awm hi a ni.
- Nangmah emaw, i chhungte zinga tu emaw hian heng symptoms te hi an neih chuan doctor nena inbiakna neih a pawimawh hle.
- He natna hi tihdamna famkim awm lo mah se, a natna lan chhuah dan thunun thei leh harsatna lian tham (stroke leh thisen chhuah tam lutuk ang chi) venna tur damdawi tha tak tak a awm a ni.
- A hmaa hriat chhuah leh enkawl dan dik tak chuan nun pangngai, hrisel takin i nung thei ang.











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