Tangier Disease hi kan taksaa cholesterol tha (HDL) level hniam tak tak nei te zingah a awm thin tih i zir chiang ang u.

Cholesterol chi khat good cholesterol (HDL / high-density lipoprotein) tih hi i hre tawh em ? Ni e, ni e... Vawiin hian natna mak tak mai, kan taksaa he cholesterol tha level a hniam lutuk huna lo awm thin chungchang kan sawi dawn a ni. Hei hi Tangier Disease an ti a . He hming hi i hre lo mai thei, mahse a chanchin hriat a pawimawh hle.

Tangier natna hi eng nge ni? A awlsam zawngin i hrethiam ang u!

Awle, a tir atang khan tan ila. Tangier Disease hi natna hlauhawm tak a ni a, kan nu leh pate hnen atanga kan rochun, chu chu genetically inherited tihna a ni . He natna vei te hian an taksaah cholesterol tha (HDL) a hniam hle. I hria em, he HDL cholesterol hi engmah lovah "good cholesterol" an ti lo. Kan thisen kalna kawng, chu chu kan thisen kalna kawnga awm cholesterol tha lo (LDL / low-density lipoprotein) paih chhuakin kan taksa thianghlimna tura puitu an ni

Tunah chuan he cholesterol tha (HDL) hi a tlahniam chuan eng nge thleng tih hi han ngaihtuah teh. Chu cholesterol tha lo (LDL) leh thau chi dang (lipids) te chu awlsam takin kan thisen kalna kawng chhungah a awm tan thin. Tui pipe-a bawlhhlawh a tlak ang bawkin. Hei hian kan thinlung tan harsatna a thlen thei a , heart attack a thlen thei a , stroke ang chi dinhmun pawh a thlen thei bawk . Chu mai a ni lo, he natna hian kan taksa peng dang tam tak a nghawng thei a ni.

Engvangin nge "Tangier" tih a nih?

Hei pawh hi thawnthu ngaihnawm tak a ni. He natna hi "Tangier" tia vuah a ni a, a chhan chu Tangier Island , US state Virginia atanga tuipui kam a awm a ni. He natna hi chu thliarkara chengte zingah hmuhchhuah hmasak ber a ni. Mahse, chu chu chu thliarkar chauha tihkhawtlai thil a ni tihna erawh a ni lo. Khawvel ram dangah pawh he natna vei an awm a.

Tangier Disease hi engtiang chiahin nge a hluar?

Dik tak chuan Tangier Disease hi a awm lo hle . Khawvel pumah mi tlemte, mi 100 vel chauh hriatchhuah an ni. Chuvang chuan sawi tam a ni lo. Mahse, a tlem hle nachungin, he dinhmun hi hriat chian a pawimawh a ni lawm ni?

Tangier Disease hian engtin nge kan taksa a nghawng?

Hetah hian thil thleng chu a awm. Zunthlum kan neih hian kan taksaa cholesterol tha (HDL) level a hniam a, chuvangin thau (lipids) te hi kan thin (liver) ah a phurh mumal thei lo. He thau duh loh tak hi taksa atanga paih chhuaktu chu thin (liver) a ni. Chuvangin, he thau paih theih loh hi zawi zawiin kan taksa hmun hrang hrangah a awm tan ta a ni. He thau hi a lo punkhawm chuan chu taksa peng chu a lian deuh thei bawk.

Thau hi hetiang hian a awm fo thin:

• Liver: Liver a lian thei.
• Spleen: 1.1.Spleen pawh a hring thei a, a lian thei bawk.
• Tonsils: Kan kawr chhunga tonsils awm. Thau an khat chuan an rawng chu yellowish-orange an ni thei.
• Lymph nodes: Hengte hi kan taksa hriselna (immune system)-a tel a ni. An lo hring thei bawk.
• Thinlung: Hei hian thinlung hnathawh dan a nghawng thei bawk.
• Thluak: A chang chuan thluak pawh a nghawng thei bawk.

Hei bakah hian harsatna dang engemaw zat a awm thei bawk:

• Nerve chungchanga harsatna: Nerve lama harsatna, ke ruh natna ang chi hi a awm thei.
• Triglyceride level sang: Hei hi thisena thau chi khat a ni a, cholesterol ang bawkin.
• Mit lama harsatna: Mit-ah hian chhum danglam tak a awm thei a, a chang chuan mit hmuhna a tlahniam thei bawk.

Tangier Disease natna lan chhuah dan chu engte nge ni?

Hei hi a pawimawh hle. Tangier Disease symptoms hi mi hrang hrangah a inang lo thei. Taksa chhunga thau awmna hmun ah a innghat a, a hmaa ka sawi tawh ang khan. Mi thenkhat chuan an pian tirh atangin symptoms an nei thei a, thenkhat erawh chuan kum 60-65 an nih hma chuan symptom lian tham an nei lo thei bawk.

Heng a lan chhuah dan tlangpui thenkhat chu hetiang hi a ni:

• Pum natna emaw, rilru hahna emaw.
• Arteries-a thau awm (atherosclerosis): Hei hian thinlung natna vei theihna a tipung a ni.
• Vun a vawt.
• Mitmeng chhunga inher (Ectropion): Hei hi a lan dan danglam deuh a ni.
• Anemia: Hei hian thisen tlakchhamna a kawk a ni.
• Kut, ke, kut leh ke ruh chak lohna.
• Infection awm lovin hun rei tak chhung lymph node a hring.

Hriat reng tur chu, a chang chuan heng symptoms te hi natna dang nen pawha ngaihsual theih a ni a, chuvang chuan diagnosis dik tak neih a pawimawh hle.

Eng thilin nge Tangier Disease hi a thlen? Genes chungchang hi han sawi ila!

Tangier Disease hi kan nu leh pate hnen atanga kan rochun ABCA1 gene an tih gene a defect vang a ni. A awlsam zawngin sawi ila, ABCA1 gene hian kan cell atanga cholesterol paih chhuah a, cholesterol tha (HDL)-a dah tur thupek a keng tel a ni. HDL hian he cholesterol hi liver-ah a phur a, chuta tang chuan a chhuak chhuak thin.

Tunah chuan he ABCA1 gene-ah hian chhiatna emaw, mutation emaw a awm a nih chuan, chu thupekte chu a thawk tha lo hle. Tichuan cell atanga cholesterol lakchhuah leh HDL siam chu a thleng dik lo. Chumi rah chhuah chu HDL level nasa takin a tlahniam a ni.

Hei hi a rochun dan chu a ni:

• Nu leh pa pahnihin ABCA1 gene chhia an rochun chuan naupang chuan Tangier Disease a nei ang.
• Nu leh pa pakhat chauhvin gene chhia chu a rochun chuan naupang chu carrier a ni ang. Symptom an nei lo mai thei a, mahse an HDL level chu a pangngai aia hniam a ni thei.

Tunah chuan nu leh pa pahnih hi he gene chhia hi kengtu an ni ang tih han ngaihtuah teh, chu chu symptoms an nei lo mai thei tihna a ni a, mahse an pahnih hian gene hi an nei ve ve tihna a ni. Chutiang nu leh pate chuan fa an neih hunah:

• Naupangin Tangier Disease a vei theihna chance 25% (1/4) a awm.
• Nausen chu natna kengtu a nih theihna chance 50% (1/2) a awm.
• Naupang hian genes chhia a rochun loh bakah a hrisel theihna chance 25% (1/4) a awm.

Hei hi pawisa fai flipping a, lu emaw, tail emaw hmuh ang mai a ni. Naupai apiangin probabilities a inang vek.

Doctor-te hian engtin nge Tangier Disease hi an hriat theih?

Tangier Disease i vei em tih chiang taka hriat duh chuan doctor pan a ngai a ni. A taksa enfiah hmasain i natna lan chhuah dan a zawt hmasa ang. Tichuan, thisen test eng emaw zat an order ang . Heng thisen test te hian i HDL cholesterol level leh apolipoprotein A1 (ApoA1) tih protein i level hniam dan te an en ber a ni. Heng pahnih hi Tangier Disease vei tan chuan a hniam hle.

Genetic testing pawh tih theih a ni a, a natna hmuhchhuah chu a dik tih finfiah theih a ni bawk. ABCA1 gene-a mutation bik a awm leh awm loh a enfiah thei a ni. Mahse, genetic testing hi hmun tinah a awm lo mai thei. Chutiang hunah chuan i doctor chuan i taksa peng pakhat, i tonsils, vun emaw, i thin emaw atanga tissue te tak te (biopsy) a la thei a ni. Heng tissue-a thau chi hrang hrang hmang hian natna hi hriat theih a ni.

Tangier Disease-ah hian eng protein nge tlakchham?

Tangier natnaah chuan protein pakhat apolipoprotein A1 (ApoA1) tih chu nasa takin a tlahniam a. He ApoA1 hi HDL cholesterol molecule siamtu lian tak a ni. Chuvangin ApoA1 a tlahniam chuan HDL level pawh a tlahniam bawk.

Diagnosis leh follow-up atan eng test nge hman thin?

Doctor-te pawhin test dang eng emaw zat an ti thei bawk a, natna hriat theihna tur leh natnain taksa a nghawng dan enfiahna tur a ni.

• Nerve leh muscle function test (Electromyograms / EMGs): Nerve chhia leh thau chak lohna te enfiah thin ang che.
• Mit enfiah: Mit a fiah lo emaw, mit a buai em tih enfiah thin ang che.
• I pum / i pum a ultrasound: I taksa peng hrang hrang, thin leh ril (spleen) te dinhmun enfiah thin ang che.
• I carotid arteries-a ultrasound: I kawr chhunga main arteries-a thau awm leh awm loh enfiah thin ang che.
• I thinlung CT angiogram hmangin:Thinlung thisen petu thisen kalna kawng (arteries)-ah blockage a awm leh awm loh enfiah thin ang che.
• Echocardiogram: Thinlung hnathawh dan leh a insiam dan enfiahna scan.
• Exercise stress test: Exercise a tih hian thinlungin a chhan dan en rawh.

Tangier Disease hi engtin nge enkawl a nih?

Dik tak chuan Tangier Disease tidam vek thei tur damdawi bik a awm lo. Genetic condition a nih avangin a symptoms control nan leh complication tihziaawmna tur thil tam tak i tih theih a awm.

A châng chuan, taksa peng thau awmna avanga tihchhiat, entirnan, tonsils emaw, spleen emaw chu operation hmanga lakchhuah a ngai thei bawk.

Ei leh in hian a pui thei ang em?

Ni e, a theih tak zet a ni. Kan cholesterol tha (HDL) level tlem a tisang thei tur ei tur thenkhat a awm a, chubakah kan cholesterol tha lo (LDL) level tihhniam thei bawk.

• Avocado a ni
• Olive hriak a ni
• Legumes te chu bean leh chickpeas (Beans) te ang chi te hi a ni.
• Thlai chi hrang hrang (e.g., buhfai pum, buhfai chi hrang hrang) .
• Nga thau ( e.g. salmon, mackerel, herring) te chu a thau hle.
• Nuts ( e.g. buhfai, almond, cashew - mahse chi tel lo) .
• Thlai fiber tam tak nei (e.g. guava, mango, banana) .
• Chia chi leh flax chi

Heng ei turte hi i ei leh inah telh fo tum hian cholesterol level tha tak a vawng reng thei ang.

Damdawi leh nunphung inthlak danglamna te chu engte nge ni?

I doctor chuan cholesterol tihhniamna damdawi , statin ang chi a pe thei ang.

HDL level tihsan thei tur nunphung tihdanglamna engemaw zat siam a rawt bawk:

• Exercise tam: Hei hian ni khatah minute 30 tal kea kal emaw, tlan emaw a huam a ni.
• Zuk leh hmuam hman loh: Cigarette in hi i bansan vek tur a ni.
• Weight hrisel takah awm reng.
• Saturated fats ai chuan monounsaturated fats ei rawh: Saturated fats hi coconut oil, palm oil, leh animal oil ang chi thilah te a awm a. Monounsaturated fats hi olive oil leh avocado-ah te a awm a.

Enkawlna hian eng side effect nge a neih?

Cholesterol tihhniamna damdawi hian a chang chuan side effect tenau te a thlen thei bawk. Mi zawng zawngin kan hmu vek lo a, mahse fimkhur a tha.

• Lu na
• Muscle na a awm
• Ek khal
• Pum natna (upset stomach) a awm

Hetiang thil a lo thlen chuan doctor hnenah i hrilh tur a ni. Tichuan damdawi chu a thlak thei ang a, thurawn dang a pe thei bawk ang che.

Tangier Disease hi ven theih a ni em?

Hei hi genetic condition a nih avangin a lo thang zel tur chu kan dang thei lo. Chumi awmzia chu i gene-ah he chhiatna hi i neih chuan tihdanglam theih a ni lo tihna a ni. Mahse, he natna hi i vei a nih chuan, a nih loh leh carrier i nih chuan, i fate’n an rochun theih dan tur hriat tumin genetic counselor i zawt thei ang. Tichuan, nu leh pa dang genetic status ngaihtuah chuan i fate’n an neih theihna chance chu chiang takin i hre thei ang.

Tangier Disease ka neih chuan nakin lawkah eng nge thleng ang?

Tangier Disease vei tan chuan prognosis hi a tlangpuiin a tha a, mahse i symptoms a hmasawn danah a innghat thung. Natna dang ang bawkin, a hmaa hriatchhuah a tha ber a, a lan chhuah dan a nasat hmain.

Hei hi damdawi chiang tak la awm lo mah se, nakin lawkah gene therapy ang chi thilte hian a puih ngei beiseina a awm. Research a la kal zel a.

Tangier Disease hi eng chen nge a awm?

Hei hi genetic disease a nih avangin dam chhung zawng a ni. Amaherawhchu, a hmaa hmuhchhuah a nih a, enfiah fo a nih chuan he natna hi uluk taka enkawl theih a ni a, nunpui theih a ni bawk.

Engtin nge ka in enkawl ang?

Tangier Disease avanga spleen lian zawk i neih chuan contact sports hi i pumpelh ngei tur a ni, chutiang contact chuan spleen a ti chhe thei a, chu chu a hlauhawm hle.

Tin, thinlung natna thlen thei thil dangte pawh hi control a ngai a ni:

• Thisen sang control dan .
• Zunthlum i neih chuan , control tha rawh.
• Zuk leh hmuam hman loh.

Engtikah nge Doctor ka hmuh ang?

Tangier Disease i neih chuan i healthcare provider hnenah i hmu fo tur a ni . Chutiang chuan i natna lan chhuah dan a na zual a nih chuan hma i la thuai thei a ni.

Doctor chuan heng thilte hi a enfiah fo ang:

• Nervous system a ni.
• Thinlung.
• Mit.

Doctor hnenah eng zawhna nge ka zawh ang?

Doctor i hmuh hunah hetiang zawhna hi i zawt thei ang:

• Ka cholesterol level tihsan nan damdawi ka ei a ngai em?
• Tangier Disease vei te pawhin an zawm theihna tur support group a awm em?
• Eng chen nge ka rawn hmu ang che?

Tangier Disease hian taksa peng hrang hrang a nghawng thei a, chuvangin taksa peng hrang hrangah checkup neih fo a tha ber. Specialist hrang hrang engemaw zat i hmuh a ngai mai thei. Damdawi hi i doctor-in a tih ang chiah chiah ei la, ei leh in hrisel leh exercise tih dan tur vawng reng turin theihtawp chhuah rawh.

Kan hriat reng tur pawimawh ber berte (Take-Home Message) .

Okay, kan sawi tawh atang khan i hriat reng tur thenkhat ka rawn tarlang ang.

• Tangier Disease hi natna awm lo, genetic disease a ni a, good cholesterol (HDL) a tihhniam nasa hle.
• Hei hian taksaah cholesterol tha lo (LDL) leh thau a ti khawm thei a, taksa peng hrang hrang liver, spleen, tonsils, leh heart te a nghawng thei a ni.
• A lan chhuah dan a inang lo va, mi thenkhat chuan thluak lam harsatna, vun lama harsatna leh mit lama harsatna te an nei thei bawk.
• Hei hi gene ABCA1 a chhiatna vang a ni.
• He natna hi thisen test, genetic test, leh a chang chuan tissue test (biopsies) hmanga hriat theih a ni .
• Damdawi bik awm lo mah se, ei leh in, nunphung thlak danglam, damdawi leh surgery thenkhat hmangin a natna lan chhuah dan chu control theih a ni.
• Hriat hmasak leh damdawi lam pan fo hi a pawimawh hle.

Chuvangin, he thu hi in tan a tangkai ngei ka beisei. Hemi chungchanga zawhna dang i neih chuan doctor biak hreh suh. Hrisel takin awm rawh!

` Tangier natna, HDL cholesterol, LDL cholesterol, genetic natna, cholesterol enkawl dan, thinlung natna hlauhawmna, ApoA1