Hmun hla vak lo i kal lai pawhin a chang chuan i lu a buai thin em? I mut dawnin thawk hah i nei em? I ke leh kekawrte a hring em? I thinlung chu mak tak maiin a na em ni? Heng thilte hi kan tar chhoh zel a thleng thin thil pangngaiah kan ngai fo thin a, mahse a chang chuan heng symptoms hnung lamah hian kan hriat tam loh leh a dik taka kan hriat loh chuan a pawi thei hle. Chutiang natna pakhat chu Transthyretin Amyloidosis, a tawi zawngin ATTR-CM a ni.
A awlsam zawngin, Transthyretin Amyloidosis (ATTR-CM) hi eng nge ni?
Hei hi hming buaithlak deuh a nih avangin thumal khat hnu thumal khatin i then darh ang u. Tichuan awlsam takin i hrethiam mai ang.
- Transthyretin (TTR): Hei hi kan taksaa protein chi khat a ni . Kan thin (liver) siam a ni. A hnathawh ber chu vitamin A leh thyroid hormone thyroxine te chu kan thisen kaltlangin taksaa hmun tul hrang hrangah a phurh chhuah hi a ni. Delivery miin parcel a phur ang mai a ni.
- Amyloidosis: Hei hi protein chi khat, a chunga kan sawi tawh ang khan , a inzawm dik loh a, a inzawm khawm a, fibril a siam a, chu chu thread ball te tak te ang mai a ni a, kan taksa peng hrang hrangah a awm a ni. Chutianga dah a nih chuan chu taksa peng hrang hrangte hnathawh dan pangngai chu a tibuai a ni.
- Cardiomyopathy (CM): Hei hian thinlung ruh natna a kawk a ni .
Tunah chuan heng zawng zawng hi han dahkhawm ila, Transthyretin Amyloidosis (ATTR-CM) hi kan thin atanga siam protein TTR chu a inzawm sual a, a inzawm khawm a, a bik takin kan thinlung thauah a inzawm khawm a, chu chuan thinlung ruh a ti thim a, a ti nghet a, thisen a pump tha thei lo.
I thinlung thisen pump chhuahna hmunpui ber (left ventricle) chu rubber ball flexible ang mai a ni ang tih han ngaihtuah teh. awlsam takin a inzawm khawm a, a zau a, taksa pum puiah thisen a pump chhuak thin. Tunah chuan, chu protein clump-te chu chu thinlung ruh-ah chuan a awm chuan, chu rubber ang chi chu zawi zawiin a hrual a, a dense ta a ni. Rubber ball ni lovin coconut shell ball ang mai a lo ni ta a ni. Tichuan awlsam takin a contract leh expand thei lo a ni lawm ni? Chu chu he natnaah hian thinlungah a thleng thin. Hei vang hian heart failure ang chi condition a awm thin.
He natna hi chi hnih a awm a.
ATTR-CM natna hi chi hnihah then theih a ni.
1. Chhungkaw emaw, Hereditary emaw ATTR-CM:
Hei hi kan genes-a mutation awm vang a ni. Chumi awmzia chu he natna thlentu gene hi kan nu emaw, kan pa emaw, kan chhungkaw zinga tu emaw hnen atangin kan rochun a ni thei tihna a ni. Hetiang chiah hian protein clumps hi thinlungah chauh ni lovin, nervous system leh kidney ang hmunah te pawh a awm thei a ni.
2. 2. A rilru a hah lutuk chuan a rilru a buai em em a.Ramsa chi hrang hrang ATTR-CM:
Hetiang chi hi a chhan dik tak hi hriat a la ni lo. Hrilhfiahna a ni lo. Hetiang chi hi mipa kum 65 chunglam ah hmuh tur a tam ber a, thinlung leh nervous system a nghawng ber a ni.
Eng nge a lan chhuah dan? Engtin nge hei hi kan hriat chian?
He natna lan chhuah dan hi mi hrang hrangah a inang lo thei. A chang chuan ‘wild-type’ type nei te hian a tir lamah chuan symptom engmah an nei lo thei. Symptoms a lo lang chhuak a nih pawhin heart failure condition tlanglawn tak nen a inang hle a, chuvangin he natna hi hriat chian a harsa thei hle.
Hengte hi a lan chhuah dan tlangpui chu a ni.
| Symptom a ni | A sawifiahna awlsam tak |
|---|---|
| Thâwk a tawi | Thâwk harsa, a bîk takin hna rit tak thawh lai te, kea kal lai te, khumah mut lai te. |
| Ke, ke ruh, ke ruh (Edema) . | Taksa chhunga tui awm tam lutuk vangin ke a hring chhuak thin. Kekawr i bun hian a tight tih a hriat theih. |
| Thinlung thawk mumal lo (Arrhythmia) . | Lung na rang tak, rilru na (palpitations), emaw thinlung thawk mumal lo, a bik takin Atrial Fibrillation (Afib) an tih natna hi a awm fo thin. |
| Chau | Chhan awm lovin a chau hle tih hriat reng reng. |
| Luak chhuak emaw, rilru hah emaw | Ding emaw, tho nghal emawa luak chhuak emaw, muhil emaw tih hriat. |
| Pum a puak chhuak | Pum chhunga tui awm zat avanga puar chhuak anga inhria. |
He natna avanga harsatna dang lo awm thei te
He protein hi thinlungah chauh a awm lo. Tin, nervous system leh tissue dang a deposit avangin harsatna dang a thlen thei bawk.
- Carpal Tunnel Syndrome: Hei hi kutphah kal tlanga nerve kal chu a compress a ni. Hetiang natna nei te hian kut pahnih ah hian he natna hi an tawk thei a ni . A lan chhuah dan chu kut zungtang hrual leh natna te a ni.
- Peripheral Neuropathy: Hei hian ke ruh chhunga nerve te a tichhe thin. Chumi avang chuan i ke ruhte chu i bengchheng thei a, i tingling thei a, i na thei bawk.
- Spinal Stenosis: Ruh kawng (spinal canal) a tawi. Hei hian hnungzang na leh ke na a thlen thei a ni.
- Tendon Ruptures: Kut emaw ke emaw a tendon a lo chhuak a, a lo chhuak a.
Pawimawh tak chu thisen sang avanga enkawl i nih a, i kut pahniha carpal tunnel syndrome i neih chuan i doctor chuan ATTR-CM a ringhlel mai thei. Chuvang chuan i natna lan chhuah dan kimchang doctor hnenah hrilh a pawimawh.
Engtin nge doctor hian he natna hi a hriat chian tak tak?
He natna lan chhuah dan hi thinlung natna tlanglawn tak nen a inang avangin, dik taka hriat theih nan test bik eng emaw zat neih a ngai a ni.
- Electrocardiogram (ECG): Thinlung electric hnathawh dan tehna a ni.
- Echocardiogram: Hei hi thinlung scan a ni. Heart muscle thickness leh function ang chi thil te ngun takin a enfiah thei a ni.
- Cardiac MRI: Hei hi thinlung in-depth scan dang a ni.
- Bone Scintigraphy (Bone Scan): Hei hi test bik tak a ni. He scan hi ruh enfiahna atana tih a ni tlangpui a, amyloid protein chu thinlungah a awm leh awm loh a hriat theih a ni.
- Thisen test: TTR gene-ah hian danglamna a awm em tih hriat nan genetic test tih theih a ni.
- Heart Biopsy: A chang chuan, heart muscle te tak te tak te chu lak a ngai a, natna hi finfiah nan enfiah a ngai thin.
Enkawlna a awm em? Hei hi a dam kim thei ang em?
Tunah hian ATTR-CM hi damdawi a la awm lo a, thinlunga protein clump lo awm tawhte pawh paih chhuah dan tur a awm lo.
Mahse, lungngai suh. Tunah chuan he natna hi thunun thei damdawi tha tak tak a awm tawh a, chu chu protein clump thar siam leh awm dan tihtawp emaw tikhawtlai emaw a ni. Heng damdawi te hian natna a zual zel loh nan a veng thei a ni.
- TTR Stabilizers: Tafamidis (Vyndaqel®, Vyndamax®) ang damdawi hian TTR protein hi a binding a, a fold dik loh nan a veng a ni.
- TTR Silencers: Damdawi Patisiran (Onpattro®) leh Inotersen (Tegsedi®) te hian he protein dik lo hi liver-in a siam chhuah a ti tlem a ni.
Heng damdawi bakah hian i natna lan chhuah dan control nan enkawlna pawh pek i ni thei bawk. Entir nan:
- Lung natna control nana damdawi.
- Heart rhythm disorder natna damdawi.
- Fluid retention atana damdawi.
A tlem hle a, natna na tak takah chuan liver transplant, kidney transplant, heart transplant ang chi thil hi hman a ngai mai thei.
He natna nena nunpui tur hian a pawimawh ber chu i cardiologist nen inzawmna mumal tak neih a, i damdawi pek te chu a hun taka ei hi a ni. Damdawi thar leh enkawlna thar zirchianna kalpui mek a nih avangin hmalam hun atan beiseina a awm.
Take-Home Thuchah a ni
- ATTR-CM hi natna a ni a, protein chi khat chu thinlungah a awm a, chu chuan heart muscle a ti thim a, a ti nghet a, a hnathawh a tichak lo bawk.
- Thâwk hah, ke ruh hring, thinlung thawk mumal lo, leh hah lutuk te hi a lan chhuahna ber a ni. Hengte hi tar chhinchhiahna pangngai angin ngaihthah suh.
- He natna hi rochun (familial) a ni thei a, a chhan awm lo (wild-type) a awm thei bawk.
- Kut pahnih ah Carpal Tunnel Syndrome i nei a, thinlung natna i neih chuan fimkhur hle ang che.
- He natna hi tihdam kim theih ni lo mah se, tunah chuan natna a zual zel tur titawp thei damdawi tha tak tak a awm ta.
- Heng symptoms te hi i neih chuan a rang thei ang bera doctor pan a, a tul anga test neih tir a pawimawh hle.
💬 Comments (0)
Comment a la awm lo. Hetah hian i comment hi a vawikhatna atan add rawh.
I comment kha add rawh