HATTR Amyloidosis (Hereditary Transthyretin Amyloidosis) chungchang hi kan sawi dawn em ni?

I kut leh ke chu a ti ting mai mai em? A nih loh leh chaw tlem i ei hnuah pawh i chawl em, a nih loh leh pum na mak tak mai i nei em? A châng chuan hengte hi thil pangngaiah kan ngai a, mahse hengte hnuaiah hian natna khirh zawk, mahse hmuh tur awm lo, tumahin an sawi loh a awm thei. Vawiin hian chutiang natna, thlah khat atanga thlah danga kai darh , zawi zawiin a zual zel chungchang kan sawi dawn a ni. Chu chu hATTR Amyloidosis an tih natna a ni.

A awlsam zawngin hATTR Amyloidosis chu eng nge ni?

Hei hi a hming kimchang chu Hereditary Transthyretin Amyloidosis a ni. A tawi zawngin hATTR kan ti ang. Hei hi natna hmuh tur awm lo tak a ni a, rochun a ni a, genes hmanga inthlunzawm tihna a ni.

Ngaihtuah teh, kan taksa thin hian protein bik transthyretin (TTR) tih a siam a. Mi hrisel takah chuan he protein hian a hna a thawk a, chutah chuan a bo ta a ni. Mahse, hATTR natna vei mi pakhatah chuan gene pakhata mutation a awm avangin he TTR protein hi a pianzia dik lo takin a lo piang ta a ni. Infiamna mawi taka siam a tlakchhiat ang mai a ni.

Heng proteins fold dik lo te hi an inzawm khawm a, clump te te an siam thin. Heng protein clump te hi amyloid kan ti a . Heng amyloids te hi kan taksa hmun hrang hrangah a awm tan a, a bik takin nerve, heart, leh kidney ang chi organs ah te a awm tan thin. Tui pipe-a bawlhhlawh a tlakbuak ang bawkin, heng protein awmte hian chu taksa pengte chu a tichhe tan a ni. Hei hian harsatna lian tak a thlen thei a, nunna atana hlauhawm pawh a ni thei. Mahse chanchin lawmawm tak chu tunah hian heng natna lan chhuah dan thunun theihna tur damdawi tha tak tak a awm ta a ni.

I doctor chuan "Amyloidosis" a ti mai thei a, a chhan chu hATTR hi chutiang chi natna chi khat a nih vang a ni.

hATTR natna lan chhuahna awm thei te chu engte nge ni?

He natna hian taksa peng tam tak a nghawng avangin a natna lan chhuah dan hi a inang lo hle. A châng chuan heng chhinchhiahnate hi natna tlanglawn dangte nen a inang a, chuvangin natna hriat chian a harsa thei hle.

Nervous system a nghawng ber a ni. Heng protein te hi thluak atanga taksa peng dang thlenga kal nerve-a an lo punkhawm hian he natna hi Polyneuropathy kan ti thin. Chuvangin hetiang thil hi i tawng mai thei.

He natna hi engtin nge a lo awm? Tute nge hlauhawma awm?

A hming ang bawkin hei hi thlahtu natna a ni . He natna hi i vei a nih chuan, i nu emaw, i pa emaw (a nih loh leh an pahnih) hnen atanga gene chhia i rochun vang a ni.

Hei hi khawvela natna hmuh tur awm lo tak anga ngaih a ni. Mahse, ram thenkhat, entirnan Portugal, Japan, Brazil-ah te chuan he natna hi hmuh tur a awm fo. Sri Lanka-ah hian he thil chungchangah hian data chiang tak awm lo mah se, natna dang nena a lan chhuah dan a inang avangin damlo hmuhchhuah dik loh hi an tam thei hle niin an ngai.

Kum eng pawhah a lan chhuah theih a, a tlangpuiin kum 30 leh 70 inkarah a lang thei a, mipa leh hmeichhia pawh inang tlangin a vei a ni.

He natna hi Doctor hian engtin nge a hriat chian?

He natna hi hriatchhuah a harsa deuh thei a, a chhan chu symptom tam tak a neih vang a ni.

Chhungkaw chanchin leh symptoms chungchang zawh

A hmasain doctor chuan nang leh i chhungte hnenah i hriselna chanchin a zawt ang.

• I chhungkuaah hian thinlung natna emaw, thinlung thim emaw nei an awm em?
• I taksa peng hrang hrangah hian rilru hah emaw, kang emaw i nei em?
• I pum a na, ril\am, emaw, chaw ei kham emaw i nei em?
• I ding lai hian i lu a buai em?
• Mit lama harsatna i nei em?

Test bik neih a ni

I natna lan chhuah dan azirin i doctor chuan test dang engemaw zat tih a rawt thei bawk.

• Thisen leh zun test: Hengte hian taksaa protein level dik lo awm leh awm loh enfiah nan a pui thin.
• Tissue Biopsy: Hei hi natna finfiah dan ber a ni. A tlangpuiin i pum vun hnuaia fat pad atang hian tissue te tak te chu anesthesia hmangin lakchhuah a ni a, laboratory-ah thawn a ni. Kan sawi tawh amyloid protein kha a deposit em tih chiang taka hriat theih a ni.
• Genetic Testing: Biopsy atanga amyloid awm leh awm loh a hriat chian hnuah DNA testing hmangin hereditary hATTR nge amyloidosis chi dang a ni em tih hriat a ni. Hei hi enkawl dan tur ruahmanna siam nan a pawimawh hle.
• Test dang: Lung hnathawh enfiah nan ECG leh Echo test te, nerve hnathawh dan enfiah nan nerve conduction study te pawh tih theih a ni bawk.

He natna hi eng enkawl dan nge ni?

hATTR enkawlna chu i natna lan chhuah dan leh natna stage-ah a innghat a ni. Enkawlna kawngah hian tum lian tak pahnih a awm a. Pakhat chu a symptoms control hi a ni. A dang chu natna thlentu bulpui ber TTR protein pangngai lo siam chhuah control a ni.

1. Natna lan chhuah dan enkawl dan

• Lung emaw, kal emaw avanga taksaah tui a pungkhawm a nih chuan, chu tui tam zawk chu paih chhuah nan diuretics hman a ni.
• Digestive system lama harsatna nei, ril\am leh pum na damdawi a awm bawk.
• Nerve natna control nan damdawi bik a awm bawk.

2. Natna thlentu lam hawi enkawlna

Hengte hi natna a zual zel tur venna atana hmanraw lian ber berte an ni.

I doctor chuan eng enkawlna nge i tan a tha ber tih a rel ang. Chûng duhthlanna zawng zawng chungchâng chu tlang takin sawipui rawh.

I tanpui tu medical team te

He natna hian taksa peng hrang hrang a nghawng avangin specialty hrang hrang nei doctor team te puihna i mamawh dawn a ni.

• Neurologist: Nerve nena inzawm harsatna nei tan.
• Cardiologist: Lung hnathawh enfiah leh enkawl.
• Nephrologist: Kal a nghawng a nih chuan.
• Gastroenterologist: Pum lam harsatna nei tan.
• Mit lam doctor: Mit lama harsatna nei tan.
• Genetic Counselor: Natna chungchang hriatthiamna pe turin leh a nghawng dan hi thlahtute atanga lo piang chhuak a ni.
• Physical Therapist: Kea kal harsatnaah tanpui tur leh taksa chak taka awm tir turin.