Ṭhian sang tak, kut leh ke sei tak, vun sei tak i nei em? Chutiang mi kan hmuh chuan basketball ang infiamna atan chuan a ropui hle ang tih kan ring. Mahse vawiinah hian heng taksa pianphung nena lo kal hriselna dinhmun kan sawi dawn a, chu chu kan sawi tam lo hle a, mahse a pawimawh hle thei a ni. Chu chu Marfan Syndrome a ni. A bik takin thinlung a nghawng thei a, chuvangin hei hi hriat chian a pawimawh hle.
A awlsam zawngin Marfan Syndrome hi eng nge ni?
Marfan syndrome hi kan genes-a awm thin genetic condition a ni. Hei hi kan taksaa ‘connective tissue’ a lo thang tha lo vang a ni. Kan taksa hi building ang maiin han ngaihtuah teh. He building chhunga thil awm zawng zawng, brick, bang leh inchung ang chi hi cement hmanga vawn tlat leh nghet taka vawn reng a ni. Chutiang bawkin connective tissue hi kan taksa chhunga thil awm zawng zawng, taksa peng hrang hrang, ruh, thisen kalna kawngte ang chite chu inzawmkhawma nghet taka vawn tlattu ‘glue’ a ni.
Marfan syndrome veiah chuan taksaa ‘gum’, a nih loh leh connective tissue chu a chak lo hle. Cement man tlawm tak hmanga in sak ang mai a ni. Hei hian hmun tam tak a nghawng thei a, mit, lung leh ruh lam system te pawh a nghawng thei a ni. Mahse a nghawng na ber chu kan thinlung leh thisen kalna kawng lian ber berte chungah a ni thei.
Engvangin nge hei hian thinlung a nghawng nasa em em? Harsatna lian ber pahnih!
Connective tissue chak lo hian thinlung hmunpui pahnih a tichhia thei a ni.
1. Aorta: Hei hi kan thinlung atanga taksa pum puia thisen phurtu thisen kalna kawng lian ber, lian ber a ni. Kan in chhunga tuikhuah atanga hmun tinah tui phurtu main pipe system ang bawkin.
2. Heart Valve: Hengte hi thinlung chhunga kawngkhar te te ang mai a ni. Heng valve te hian thisen chu kawng khatah chauh a kal theih nan a enkawl a ni.
Tunah chuan heng hmun hnih ah hian eng nghawng nge a neih tih i lo en ang u.
1. Aorta hi eng nge thleng?
Marfan syndrome vei chuan an aorta wall-ah connective tissue chak lo tak a nei a. Hei hian harsatna lian tak pahnih a thlen thei a ni:
- Aortic Dilation: A tlangpuiin thisen kalna kawng hi a flexible hle. Mahse, he chak lohna avang hian aorta chu zawi zawiin a zau leh lian tan a, thinlung thawk apiangin pressure a siam chu a tuar thei lo.
- Aortic Aneurysm: A chang chuan he widening hi a tawp mai lo. Artery chak lo ber chu balloon ang maiin a bulh tan thin. Hei hi ``Aortic Aneurysm'' kan tih chu a ni. Hei hi Marfan syndrome natna hlauhawm ber a ni. A chhan chu he balloon ang maia bulge hi engtik lai pawhin a puak (rupture) emaw a tear (dissection) thei a ni. Nunna atana hlauhawm emergency a ni.
2. Heart valve te hi engtin nge an awm?
Connective tissue chak lo hian heart valve te pawh a thawk tha lo thei bawk. An chak lo thei a, an inhnaih tawk lo thei bawk. Hei vang hian a ni:
- Valve Regurgitation: Valve a khar tha lo a nih chuan thisen chu thinlungah a leak leh thei a ni. Hei hian thinlung chu taksaah thisen pump turin a thawkrim zawk thin. Hun a kal zel a, hei hian heart failure a thlen thei a ni. Valve a nghawng tam ber chu Aortic Valve leh Mitral Valve te an ni.
- Mitral Valve Prolapse: Hetiang dinhmunah hian thinlung veilam a mitral valve chu a chak lo va, a khar tha lo va, chu ai chuan a fold leh ta zawk a ni.
A pawimawh ber chu Marfan syndrome vei mi sawm zinga 9 velin thinlung emaw aortic emaw harsatna an nei dawn a, chuvangin hei hi hriat a pawimawh hle.
Heng thinlung natna lanchhuahnate hi engte nge ni? Engtin nge kan hriat chian?
A tam zawkah chuan a tir lamah chuan symptom a awm lo thei. Mahse, heng symptoms te hi aorta a zau deuh emaw, valve lama harsatna a zual deuh emaw chuan a lang thei a ni. Heng zinga pakhat emaw a aia tam emaw i neih chuan Doctor pan a pawimawh hle.
| Symptom a ni | A sawifiahna awlsam tak |
|---|---|
| A rilru emaw, a hnungzang emaw a na | A bik takin a natna chu a lo thleng thut a, a nasat chuan a hlauhawm hle. |
| Thâwk harsa emaw, thawk harsa emaw | Tlemte kal emaw hna thawh emaw avanga hahna lo awm thin. |
| Palpitations (i thinlung a thawk chak ang maia hriat) . | I heart rate a danglam emaw, i rilru a na emaw ang maia hriat. |
| Luak chhuak emaw, lu hah emaw tih hriat | Ding emaw, tho nghal emawa luak chhuak tih hriat. |
| Chauhna leh chak lohna danglam tak | Chhan awm lovin chau tih hriat reng reng. |
| Ke leh ke a hring chhuak | Lung hnathawh tlahniam chhinchhiahna a ni thei. |
Engtin nge doctor hian he natna hi a hriat chian tak tak?
Marfan syndrome hmuhchhuah hi a harsa deuh thei a, a chhan chu mi zawng zawng hian symptoms inang an nei vek lo a, chuvangin doctor chuan thil engemaw zat a en ang.
- Taksa enfiahna: Doctor chuan i san zawng, i kut leh ke sei zawng, i kut zungtang sei zawng, i rilru pianzia, i mit leh i ruhro te a enfiah ang.
- Chhungkaw damdawi lam chanchin: Hei hi thlahtute atanga lo piang natna a nih avangin i chhungkuaah hian heng symptoms hi an nei tawh em tih emaw, cardiac arrest nghal vanga thi emaw an awm em tih zawh che a ni ang.
- Special test: Thinlung dinhmun dik tak hriat theih nan test eng emaw zat neih a ni.
- Echocardiogram: Hei hi thinlung scan ang a ni. Aorta lian tham leh valve hnathawh dan te chiang takin a hmu thei a ni.
- Electrocardiogram (EKG): Hei hian thinlung electrical activity enfiah nan a pui a, chu chu heartbeat rhythm-ah harsatna a awm leh awm loh enfiah nan a pui a ni.
- Genetic Testing: Marfan syndrome thlentu genetic mutation i neih leh neih loh hriat nan thisen test a ni.
- Thinlung tana harsatna thlentu thiltih pumpelh. Entirnan, infiamna nasa tak, weightlifting, rugby, football te hi a tha lo hle.
- Aorta chu hlauhawm takin a zau a nih chuan a tear loh nan operation (dissection) tih a ni. Hei hi emergency a nih hmaa ruahmanna siam a tha ber.
- Doctor chuan damdawi ``Beta-blockers'' emaw ``ARBs'' emaw ang chi a pe thei a ni. Hengte hian thisen sang control-na leh aorta-a pressure tihhniamna hmangin hna an thawk a ni. Hei hian artery a zau chakna rate a ti hniam a ni.
- Aorta chhia chu la chhuakin artificial tube (graft) hmanga siamthat a ni.
- Heart valve tha lo siamthat emaw, artificial valve hmanga thlak emaw.
- Aorta lian leh te enfiah nan kum khatah vawi khat tal echocardiogram ang chi scan neih a pawimawh hle. Hei hian harsatna eng pawh a lo awm a nih chuan hriat hmasakna kawngah a pui thei a ni.
- Aorta diameter centimetre 5 aia tam a nih chuan.
- Kum khat chhunga artery chu a zau chak hle a nih chuan.
- I chhungkuaa mi pakhatin aortic dissection history a neih chuan.
I chhungkuaa mi tu emaw hian he natna hi a nei emaw, a lan chhuah dan ang chiah a nei emaw a nih chuan, i doctor hnena hrilh hi natna hriatchhuahna kawngah a pui hle.
Okay, tunah chuan hei hi eng enkawlna nge ni?
Marfan syndrome hi a dam kim thei lo. A chhan chu kan gene-a awm thil a nih vang a ni. Amaherawhchu, thinlunga a tihchhiatna thunun nan te, natna khirh tak tak venna tur te, miten nun pangngaia an nun theihna tur te, enkawlna tha tak tak a awm a ni. Enkawlna hi hmunpui pahnih ah then theih a ni.
| Surgery hmanga enkawlna ni lo | Surgery hmanga enkawl a ni |
|---|---|
Nunphung inthlak danglamna: | Engvangin nge operation hi a tul?: |
Damdawi: 1.1. | Surgery chi hrang hrang: 1.1. |
Damdawi lam enfiah fo thin: | Surgery neih a tha a ni: |
Damdawi ina admission nghal ngai emergency thil thleng! (Hriattirna chhinchhiahna) .
Heng symptoms te hi aortic rupture emaw dissection emaw chhinchhiahna a ni thei. Heng zinga pakhat tal a lo awm a nih chuan Emergency Department (ETU) hnai ber pan tur a ni.
| Heng symptoms te hi i neih chuan ETU ah kal nghal rawh! | |
|---|---|
| - A rilru, hnungzang, emaw pum chhunga natna rang tak, tuar theih loh . | - Thâwk harsa na tak. |
| - Thil hriatna hloh. | - Taksa peng pakhata rilru hahna emaw, tingling emaw. |
| - Thaw tam lutuk, vun a vawt. | - Nausea leh luak chhuak. |
Marfan syndrome nen hian nun tha tak neih theih a ni em?
A ni tak zet! Kum tam tak kalta khan he natna vei te dam rei zawng hi a rei lo hle. Mahse tûn laiah chuan damdawi lam enkawlna hmasawn tak tak, screening neih fo leh surgery neih avângin Marfan syndrome vei chu mi pangngai ang bawkin kum 70 emaw 80 emaw thlengin nun hrisel tak a hmang thei a ni.
A pawimawh ber chu doctor nena inbiak reng a, a thupek dik taka zawm a, a hun taka test neih a, i damdawi hman dik hi a ni.
A bik takin Marfan syndrome vei hmeichhiain naupai a tum a nih chuan naupai hmain cardiologist leh gynecologist te a pan ngei tur a ni. Naupai lai hian thinlunga harsatna a tam avangin fimkhur bik a ngai a ni.
He dinhmun hi hriatthiam a, a tul anga hma la a, rilru pu chunga nun hian i thinlung i humhim thei a, hrisel, dam rei tak i nung thei bawk.
Take-Home Thuchah a ni
- Marfan syndrome hi thlahtu natna a ni a, taksa inzawmkhawmna (connective tissue) a tichak lo hle.
- Hei hian thinlung leh thisen kalna bulpui, aorta-ah nghawng na tak a nei thei a ni.
- Aortic dilation leh aneurysm te hi a hlauhawm ber a ni.
- A hun ruatah doctor hmu a, echocardiogram ang chi scan neih a pawimawh hle.
- Thinlung tichhe thei thil, thil rit phurh ang chi thilte chu pumpelh rawh.
- Vaukhânna chhinchhiahna, rilru natna rang tak, na tak ang chite chu fimkhur rawh.
- Enkawlna leh enkawlna dik tak nen chuan nun pangngai tak, dam rei tak i nung thei ang.
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