I thisenah triglycerides level sang tak i nei em? He genetic condition hmuh tur awm lo, Familial Hyperchylomicronemia Syndrome (FCS) chungchang hi han sawi ila.

Tunlai mi tam takin harsatna an tawh chu thisenah thau tamna, cholesterol leh triglycerides te hi a ni. Mahse a chang chuan heng thau level, a bik takin triglyceride level hi a pangngai lo takin a sang a, a tlangpui aiin a let sang tam takin a sang zawk thin. Hei hi a chhan chu nu leh pa atanga lo chhuak, genetic condition hmuh tur awm lo tak a ni thei. Vawiin hian chutiang dinhmun pakhat, Familial Hyperchylomicronemia Syndrome, a tawi zawngin FCS kan sawi dawn a ni. Hei hi thupui buaithlak deuh ni mahse, awlsam takin i hrethiam ang u.

A awlsam zawngin FCS chu eng nge ni?

FCS hi i nu leh pate hnen atanga i rochun genetic condition hmuh tur awm lo tak a ni. Hetiang natna vei hian thau, a bik takin triglycerides -te chu a ti chhe thei lo va, a ei thei lo bawk.

Kan taksa hian thau tichhe thei factory tlemte a nei ang tih han ngaihtuah teh. Damdawi lam hawi chuan hei hi enzyme pakhat lipoprotein lipase (LpL) an tih a ni. FCS nei mi pakhatah chuan he ‘factory’ hi a thawk tha lo a, a thawk tha lo tak zet a ni.

Chumi avang chuan kan ei tur thau, triglycerides an tih chu taksain a hip lut thei lo va, chu ai chuan thisenah thau droplet chylomicrons tia koh angin a pung khawm ta zawk a ni. Hetiang chylomicrons pungkhawm hi chylomicronemia syndrome kan tih chu a ni. Hei hian thisenah triglyceride level a tisang lo hle.

Engvangin nge hetiang hi a lo awm?

Hei hi a chhan ber chu genetic mutation a ni. A chhan chu kan sawi tawh ang khan lipoprotein lipase (LpL) enzyme siamna atana inrawlh genes a chhiat vang a ni.

A pawimawh ber chu he natna vei tur hian he gene chhia hi i nu leh pa hnen atangin i rochun a ngai a ni. Nu leh pa pakhat chauh atanga i rochun chuan natna hi i nei lovang, mahse chu gene ‘carrier’ i ni ang. Chumi awmzia chu he gene hi i fate hnena pek chhuah theihna i nei tihna a ni.

LpL enzyme hi kan pancreas atanga siam a ni. A hnathawh ber chu thisena chylomicrons te tihchhiat a, taksain a chhunga thau awmte chu chakna atana a hman theih nan a pui a ni. Chuvangin LpL hian hna a thawk tha lo a nih chuan thau chu a chhe thei lo va, thisena triglyceride level pawh a sang chho zel a ni.

Hei hi eng symptoms nge ni?

FCS symptoms hi thisenah triglycerides a tam vang a ni. Mi hrisel chuan triglyceride level 150 mg/dL aia tlem a ni. Mahse, FCS vei tan chuan he level hi 1,000 mg/dL aia tam a ni thei. Heng symptoms te hi hriat chian a pawimawh hle.

Engtin nge hei hi hriatchhuah leh enkawl a nih?

Diagnosis tih a ni

Thil awm lo tak a nih avangin a chang chuan diagnosis dik tak hmuh theih nan hun rei tak a ngai thei bawk. I pum na reng reng, pancreatitis natna i neih nawn fo a, i thisenah triglyceride level sang tak i neih chuan i doctor chuan FCS a ringhlel mai thei.

Natna finfiah nan a hnuaia test te hi tih thin a ni:

• Fasting triglyceride blood test: He test hi darkar eng emaw zat chaw nghei hnua tih thin a ni. Triglyceride level 750 mg/dL aia tam a nih chuan FCS a ni thei. A châng chuan thisen sample chu bawnghnute ang maiin a lang thei.
• Lipase level testing: Taksain LpL enzyme a siam chhuah zat tehna atan special injection (heparin) pek a ni.
• Genetic test: Hei chauh hi 100% a chiang thei tur a ni. Gene chhia chu nu leh pa pahnih atanga rochun a nih leh nih loh a enfiah thei a ni.
• CT scan: Hei hian pancreas leh liver ang chi taksa peng hrang hrang a tichhia em tih hriat nan a pui a ni.

Enkawl dan tur

FCS enkawlna bulpui ber chu ei leh in a ni a, damdawi thar pawh tun hnaia pawm a ni bawk.

FCS tan ei leh in tur

Hei hi management lama thil pawimawh ber a ni. He chaw ei dan tur ruahmanna siam tur hian i doctor leh nutritionist te nen thawhhona tha tak neih a pawimawh hle.

• Thau tihtlem: Ni khata thau ei zat chu gram 20 aia tlem lo tur a ni.
• Zu in hi bansan rawh: Zu hian triglyceride level a tisang lehzual.
• Sugar leh carbohydrates awlsam tak tak ei tlem: Sugary drinks leh sweets ang chi thil hi pumpelh rawh.
• Ei tur: Thlai, thei, legumes, whole grains, egg whites, thau tel lo dairy products, leh sa thau lo.
• Vitamin supplement: I doctor chuan thau-a inthiar theih vitamin A, D, E, leh K te ei turin a rawt mai thei a, chu chu taksain thau tihtlem nan a mamawh a ni.

Damdawi thar

Olezarsen (Tryngolza) chuan a rawn ti a.Damdawi thar December 2024-a pawm a ni a, thla khatah vawi khat vun hnuaiah injection a ni. A awlsam zawngin sawi ila, taksain thisenah thau a khawlkhawmna tur protein apoC-III tih a tihtlem avangin hna a thawk a ni. Hemi chungchang hi i doctor hnen atangin i hre belh thei ang.

FCS nena khawsakna kawnga harsatna awmte

FCS hi dam chhung zawnga natna a ni a, taksa leh rilru hriselnaah pawh nghawng lian tak a nei thei a ni.

• Ei leh in lama harsatna: Pawn lama chaw ei leh party-a kal hi a harsa thei hle.
• Psychological effects: Natna awm fo, hmalam hun hlauhna, lungkhamna, leh "brain fog" te a awm thei.
• Khawtlang leh hnathawhna nunah nghawng a neih: Damdawi ina awm fo hian khawtlang inlaichinna leh hnathawhna vawn nun a ti harsa thei hle.

Chuvangin, i medical team te nen thawhhona tha tak neih a pawimawh hle a, a tul chuan mental health counselor te puihna dil a pawimawh hle.