Familial Hypercholesterolemia - A chungchanga hriat tur awmte

I chhungkuaah hian tar hma, chu chu kum 40-50 inkar vela heart attack nei tawh an awm em? A nih loh leh i chhungkuaa miten, "High cholesterol, high cholesterol" an tih fo hi thil awmze nei tak a ni em? A tam zawkah chuan kan ei leh in ang chi, leh exercise lak loh ang chi thil hi a chhan ber niin kan ngai thin. Chu chu a dik a, chu pawhin nghawng a nei bawk. Mahse, a chang chuan, he cholesterol sang chhan tak tak hi kan taksa chhunga harsatna, kan genes-a harsatna a ni thei. Chu chu damdawi lama `Familial Hypercholesterolemia` emaw `FH` emaw kan tih chu a ni. Vawiin hian he thu hi awlsam takin, i hriatthiam theih dan tur angin kan sawi dawn a ni.

A awlsam zawngin, Familial Hypercholesterolemia (FH) chu eng nge ni?

Family Hypercholesterolemia hi nu leh pa emaw, an pahnih emaw atanga rochun, genetic condition a ni. Hei hi kan taksaa cholesterol "tha lo", a nih loh leh LDL cholesterol, pian tirh atanga a san em em lai a ni.

Kan thisen kalna kawngte hi thisen kalna kawng (arteries) angin han ngaihtuah teh. He `LDL` cholesterol hi chu artery bang-a innghat, yellow, waxy layer ang mai a ni. A tlangpuiin hengte hi a lo pung zauh zauh zauh zauh thin. Mahse `FH` vei tan chuan `LDL` cholesterol level a sang em em a, chuvang chuan he waxy layer hi thisen kalna kawngah hian a lo piang rang hle a, a thuk hle bawk. Hei hi `atherosclerosis` kan ti a. Hun a kal zel a, he layer hian thisen kalna kawngte chu a lo thang lian a, a tizau a, a chang chuan a tikhawlo vek bawk. Chutah chuan nunna atana hlauhawm thil, heart attack leh stroke te a lo awm thin.

Mi hrisel pangngai tan `LDL` cholesterol level chu 100 mg/dL aia tlem a ni tur a ni. Mahse, `FH` vei tan chuan he value hi a sang zawk thei a, chu chu 160 mg/dL, 190 mg/dL te hi a ni . A nasat deuhah chuan 400 mg/dL aia tam pawh a ni thei. Thil hlauhawm ber chu naupan lai atanga he natna hi a awm tawh avangin enkawl loh chuan mi pangngai aiin kum naupang zawkah chuan thinlung natna vei theihna a sang hle a ni.

He thawnthu hi a hlauhawm deuhin a lang thei a, mahse thu lawmawm tak chu a hmaa i hriat chian a, damdawi dik tak i ei a, nunphung i thlak danglam chuan i thunun vek tawh mai thei a, nun hrisel tak i hmang thei a ni.

FH hi chi hnih a awm a.

He dinhmun hi kan rochun dan a zirin chi hnih lian takah then a ni. Hei hi hriatthiam a awlsam hle.

Kan taksa hian cholesterol control dan tur ‘instruction’ pahnih a nei ang tih han ngaihtuah teh. Pakhat chu kan nu hnen atangin kan hmu a, pakhat zawk chu kan pa hnen atangin kan hmu bawk.

1. Heterozygous FH: Hei hi a tam ber a ni. Heta thil thleng hi ‘instruction book’ (gene)-ah hian nu leh pa pakhat chauh, nu emaw, pa emaw atanga rochun tlin lohna a awm a ni. Chumi awmzia chu Lehkhabu pakhat chu a tha a, lehkhabu dangah chuan tihsual tlem a awm tihna a ni. Hei hian cholesterol control chu a out of whack deuh a ni.

2. Homozygous FH: Hei hi a tlem hle a, a na hle bawk.Lampang. Heta thil thleng chu mom leh dad pahnih hnen atanga ‘instruction’ dawn pahnih hi a dik lo vek a ni. Tichuan cholesterol control tu mechanism chu a chak lo hle a ni. Heng mite `LDL` cholesterol level hi a sang hle a ni.

`FH` hi `autosomal dominant` category-a awm, genetic disease a ni. Hei hian a awmzia chu naupangin natna a neih theih nan nu leh pa pahnih a\anga gene \ha lo chu a rochun a ngai lo tihna mai a ni a, nu leh pa pakhat chauh a\anga rochun chu a tawk tihna a ni

Hetiang dinhmun hi engtiang chiahin nge a awm? Eng nge a lan chhuah dan?

Khawvela mi 250 zinga pakhat hian natna tlangpui `Heterozygous FH` an nei nia chhut a ni. Mahse a lungchhiatthlak ber chu heng mi 10 zinga 9 hian he natna hi an nei tih an hre lo .

Vawi tam tak chu FH hian a tir lamah chuan symptom bik a nei lo. Heart attack, rilru natna ang chi i neih hma chuan FH i nei tih i hre lo mai thei. Mahse, chutih hunah chuan a tlai tawh mai thei. Hei hi a chhan chu FH vei hian an pian ni atang khan thisen kalna kawngah cholesterol a lo awm tan thin a ni.

Hun a kal zel a, cholesterol buildup hian a hnuaia symptoms te hi a thlen thei a ni.

Engtin nge FH hi hmuhchhuah a nih?

I doctor-in FH i vei nia a rinhlelh chuan thil engemaw zat an en ang a, i natna hmuhchhuah chu a nemnghet ang.

• Thisen test: Hei hi a pawimawh ber a ni. Thisen test `Lipid Profile` an tih hian i cholesterol level a enfiah a, chu chuan `LDL` cholesterol level sang lo tak a zawng thin. Puitlingah 190 mg/dL aia tam a nih chuan, naupang pakhatah 160 mg/dL aia tam a nih chuan `FH` rinhlelh a ni.
• Chhungkaw damdawi lam chanchin: Hei hi a pawimawh hle. I doctor chuan, "I pa, i nu, i pi leh pu emaw, i unaute emaw hian i naupan laiin heart attack an nei tawh em?" "I chhungkuaah hian cholesterol sang an nei em?"
• Taksa enfiah: Doctor chuan i taksaah xanthomas (vun hring) emaw, a hmaa kan sawi tawh ang khan corneal arcus (mit circles) emaw a awm leh awm loh a enfiah ang.
• Genetic testing (DNA test): A then phei chuan genetic testing hmangin natna hi chiang taka finfiah theih a ni. Hei hian FH thlentu gene lian ber ber (APOB, LDLR, emaw PCSK9 ang chi)-a chhiatna awm leh awm loh a enfiah thei a ni.

Enkawlnate chu engte nge ni?

`FH` hi kan tihdam theih natna a ni lo. A chhan chu kan genes nena lo kal thil a nih vang a ni. Mahse a control tha hle thei . Enkawlna tum ber chu `LDL` cholesterol level a theih ang anga tihhniam a ni a, chu chuan lung natna vei theihna a ti tlem a ni.

1. Damdawi (damdawi chi hrang hrang) .

`FH` vei tan chuan nunphung thlak danglam ringawt hian cholesterol control nan a tawk lo. Chuvangin an dam chhung zawng damdawi pakhat emaw a aia tam emaw ei a ngai a ni .

• Statins te hi:Hengte hi damdawi hman tlanglawn ber leh first-line damdawi an ni. Liver-in cholesterol a siam chhuah tihtlem nan hna an thawk a ni.
• PCSK9 inhibitors: Hengte hi injectable damdawi class thar tak a ni. Statin chauh hmanga cholesterol level control theih loh tan chuan an tangkai hle.
• Ezetimibe: He damdawi hian kan ei leh in atanga cholesterol lakluh a tihtlem avangin hna a thawk a ni. Statin nena inkawp hian pek a ni fo.
• Damdawi dang: Hei bakah hian damdawi dang `Bile acid sequestrants` leh `Fibrates` te pawh a awm bawk. I doctor chuan eng damdawi nge i tan a tha ber tih a rel ang.

2. Nunphung inthlak danglam

Damdawi ei rual hian nunphung hrisel zawm a pawimawh bawk. Hei hian damdawi hman tangkaina a tipung mai ni lovin, thinlung natna vei theihna a tiziaawm lehzual thei bawk.

3. Enkawlna bik dang

Homozygous FH na tak nei tan chuan damdawi chauh hian an cholesterol a control thei lo mai thei. Heng mite tan hian LDL apheresis tih damdawi an ti thin. Hei hi dialysis ang a ni. Thisen tlemte lak a ni a, machine hmangin an pass a, chu chuan LDL cholesterol tha lo chu a paih chhuak a, chu chu taksaah a inject leh a ni. Hei hi kar khatah vawi khat emaw vawi hnih emaw tih thin a ni.

FH i neih chuan eng nge i tih ang?

`FH` i vei tih hmuhchhuah i nih chuan nangmah chauh a ni lo. I chhungkaw pum tan thuchah pawimawh tak a ni.

Naupangin chhungkaw history-a `FH` a neih chuan kum 2 a nih atang pawhin cholesterol test theih a ni a, a chhan chu he natna hi enkawl tan a nih chuan nakin lawkah harsatna awm thei chu a tlem thei ang.

`FH` i neih chuan hlau suh. I doctor nen inzawmna nei reng rawh. I damdawi ei tur chu a hun takah ei thin ang che. Nunphung thlak danglam rawh. Tichuan nang pawh mi dang ang bawkin nun rei tak, hrisel takin i nung thei ang.