I veins te hi a hliam awlsam em? I vun chu a hring hle em? Vascular Ehlers-Danlos Syndrome (vEDS) hlauhawm tak hi i hrechiang ang u!

I taksaah hian a chang chuan bump te tak te hnuah pawh blue spot lian tak i nei thin em? A nih loh leh i vun chu a thim lutuk a, i thisen zamte chu a lang thei em? Chûngte chu a châng chuan natna tlêm tak, mahse na tak tak chhinchhiahna a ni thei a, mahse a châng chuan kan ngaihven vak lo. Vawiin hian chutiang natna chiah kan sawi dawn a, chu natna, taksa inzawmkhawmna, a bik takin thisen kalna kawngte tichak lotu natna a ni. Hei hi Vascular Ehlers-Danlos Syndrome , a tawi zawngin (vEDS) an ti a. Hei hi a buaithlak deuhin lang mahse, awlsam takin i hrethiam ang u.

Ehlers-Danlos Syndrome (EDS) hi eng nge ni? Vascular type hi a hlauhawm em?

A awlsam zawngin sawi ila, Ehlers-Danlos Syndrome (EDS) hi kan taksaa inzawmna (connective tissue) tichhe thei genetic diseases pawl a ni. Tunah chuan connective tissue hi eng nge ni tih i ngaihtuah mai thei. Kan taksa thlunzawmtu leh chakna petu an ni. Entirnan, thil chi hrang hrang, ligament, tendon, leh cartilage te. Chungte chu kan taksa pianzia, chakna leh inthlak danglam theihna petu a ni.

EDS hi chi 13 vel a awm a. Tuna kan sawi vascular EDS (vEDS) hi chi li (`(Type IV)`) a ni. Hei hi heng EDS chi hrang hrang zingah hian a tlem ber leh a pawi ber a ni. Hetiang natna vei te hian thisen kalna kawng chak lo tak tak an nei a, chu chu thisen phurtu thisen kalna kawng (arteriries) te, leh an chhunga taksa peng hrang hrang (`(internal organs)`) chak lo tak tak leh awlsam taka tihchhiat theih te an nei a ni. Glass te tak te ang a ni a, thil tenau ber pawhin a chhe thei a ni.

He natna hi tuin nge nei thei ang? Engtiang chiahin nge a hluar?

Because this is a genetic disease , nu leh pa pakhat emaw, nu leh pa pahnih emaw atanga rochun a ni fo thin. Chu chu rochun a ni tihna a ni. Mahse, a chang chuan chhungkaw zingah he natna hi tu mahin an la nei lo a nih pawhin, mi tu emaw chuan he gene hi a takin a inthlak danglam (spontaneous mutation) hmangin natna hi a nei thei a ni.

EDS hi a tlangpuiin natna hmuh tur awm lo tak a ni. Mi 5,000 zinga pakhat velin a nghawng a ni. Hetiang EDS (vEDS) hi a tlem zawk zia han ngaihtuah teh. Mi 200,000 emaw 250,000 emaw zinga pakhat vel a vei a ni . Chuvangin mi tam takin an hriat loh hi thil mak a ni lo.

Hei hian engtin nge taksa a nghawng?

Vascular Ehlers-Danlos Syndrome (vEDS) hi taksa peng hrang hrang, a bik takin thisen kalna kawng (arteries) leh chhungril lam taksa peng hrang hrangte chakna leh elasticity hloh tir thin a ni Hei hian mi a ti na awlsam thei a, hliam avanga chhungril thisen chhuah theihna a sang zawk bawk.

Ngaihtuah teh, lu chunga bump te tak te hi mi pangngai tan chuan thil lian tak a ni lo. Mahse hetiang natna vei tan chuan a chhunga thisen kalna kawng a puak thei a, a nih loh leh a bang chu a inthen thei (arterial dissection). Rubber tube hlui ang mai a ni a, pressure tlemte pawha puak thei tura inpeih a ni.

Vascular EDS natna lan chhuah dan chu engte nge ni?

He natna lan chhuah dan hi mi hrang hrangah inang lo thei mah se, a lan chhuah dan tlanglawn thenkhat hmuh theih a awm. Eng nge an nih hi han thlir ila:

• Vun inthlak danglamna:
• The skin becomes very thin, translucent, and delicate , chu chuan taksaa veins te chu chiang takin a lang thei a ni.
• Hmun thenkhatah, a bik takin kut leh keah vun hi hmun dang aiin a upa chak zawk angin a lang.

• Hmai lam thil danglam tak tak:
• He natna vei te hmui leh hnar hi a danglam bik hle thei .
• Chin te tak te .
• Mit chu a lian a , a inthlau deuh bawk .
• Mi ṭhenkhat chuan mitmeng an nei tlem hle a, a nih loh leh an nei lo .
• Earlobes hi a te hle a, a awm lo tluk a ni . Beng pahnih hi lu atanga hla deuhah dah a ni a, hmalam pan angin a lang thei bawk.

• Circulatory system lama harsatna awmte:
• Taksa chu a hliam awlsam hle . Thil tenau takin a rawn nuai pawhin blue spot lian tak tak i hmu thin.
• Varicose veins hi a tlangpui aia kum naupang zawkah a awm thei .
• Thisen sang (hypertension) leh heart valve lama harsatna (e.g. mitral valve prolapse ) te hi a awm fo thin.
• Arterial injury a awm theihna a sang hle. Hei hian arterial dissection ang chi thilte pawh a huam tel a ni . Chungte chuan aneurysm hlauhawm tak (thisen kalna bang chak lo leh ballooning) emaw, rupture emaw a thlen thei a ni.

• Internal organs chak lohna te: 1.1.
• Harsatna hlauhawm tak tak a awm thei a, chu chu lung chim (pneumothorax) leh chhungril taksa peng hrang hrang a chhe thei a, chu chuan thisen chhuah tam lutuk a thlen thei a ni.

• Skeletal inthlak danglamna:
• A rilru (pectus excavatum) pil tawh (pectus excavatum) hmuh tur a awm thei.
• A tlangpui aia tawi zawk pawh a ni thei.

Engvangin nge hetiang dinhmun hi a lo awm? Eng nge a chhan?

Hei hi genetic disease a ni tih kan sawi tawh a. Chu chu kan DNA-a gene pakhata mutation (`(genetic mutation)`) avanga thleng tihna a ni . Ngaihtuah teh, kan DNA hi taksa siamtu leh thununtu zirtirna bu ang a ni. Kan cell leh taksa peng hrang hrangte hian he thupek bu ang hian hna an thawk a ni. Genetic mutation hi he instruction book-a tihsual ang mai a ni. Mahse taksa chuan engtin pawhin chu thupek dik lo chu a zawm a ni.

Vascular Ehlers-Danlos Syndrome (vEDS) hi collagen III tih protein siamtu gene pakhata mutation avanga lo awm a ni. A tlangpuiin kan taksa hian he collagen III hi tissue leh structure thenkhat tichak turin a hmang thin. Mahse chu gene mutation avang chuan taksain collagen III a siam tling lo emaw, a siam chhuah collagen III hian chhiatna a nei emaw a ni. Chuvangin tissue te chu an mamawh chakna a pe lo.

Genetic a nih avangin he natna vei hian an fate hnenah a kai thei a ni.Nu leh pa pakhatin he natna hi a neih chuan naupai apiangin naupangin he natna hi a rochun theihna chance 50% a awm. Nu leh pa pahnihin he natna hi an neih chuan naupang hian 100% in a rochun theihna chance a nei a ni.

Hei hi hri kai theih a ni em?

Ni lo, hei hi mi pakhat atanga mi dang hnena kai theih natna a ni lo. Hei hi genes hmanga rochun vek thil a ni.

He natna hi engtin nge an hriat chhuah?

Doctor chuan Vascular Ehlers-Danlos Syndrome (vEDS) hi i medical history, taksa enfiahna, symptoms leh i chhungte zingah he natna hi an vei em tih te a zirin a ringhlel thei a ni. Chu rinhlelhna chu a lo chhuah hnu chuan, a awm theihna tur chu finfiah emaw, paih chhuah emaw turin genetic testing an nei thin. He natna thlentu hi genetic mutations (tunah hian hriat chhuah tawh) lian pahnih a awm avangin, genetic testing chauh hi definitive diagnosis siam theihna kawng awmchhun a ni.

Eng ang test nge tih thin?

Thil awm lo tak a nih avangin doctor-te chuan genetic test hmain test dang eng emaw zat an nei thei bawk. Entîr nân, echocardiogram (thinlung scan) emaw, CT scan emaw an ti thei a ni . Heng test te hi thisen lama harsatna dang, thisen chhuah tam lutuk emaw, hliam awlsam tak emaw awm thei te hriatchhuah nan tih hmasak a ni. Mahse, genetic testing chauh hian Vascular Ehlers-Danlos Syndrome (vEDS) i neih leh neih loh chu chiang takin a hriat theih.

Enkawlnate chu engte nge ni? Tihdam theih a ni em?

Vascular EDS hi tihdam theih a ni lo. Genetic condition a ni a, dam chhung zawng a ni tihna a ni. Dam theih a nih loh avangin doctor-te chuan a natna lan chhuah dan enkawl leh a nghawng tih tlem hi an ngaih pawimawh ber a ni.

Eng ang damdawi/enkawlna nge hman?

Vascular Ehlers-Danlos syndrome vei te hi thisen kalna kawng ruptured avanga aneurysm (bulging blood vessels) leh internal bleeding hlauhawm tak an neih theihna a sang hle. Chuvangin, he natna hi i neih chuan thisen zam (aneurysm) awm leh awm loh enfiah nan medical screening neih fo a ngai dawn a ni. Thil engemaw takah chuan chhungril hliam emaw, thisen kalna (aneurysm) siamthat nan operation neih a ngai thei bawk.

Hetiang dinhmun hian naupai laiin harsatna lian tak tak, nunna atana hlauhawm tak tak, nausen pum a tihchhiat emaw, thisen chhuak nasa tak emaw a thlen thei bawk.

I doctor chu i mamawh tur damdawi, enkawlna leh surgery chungchang sawipui tur tha ber a ni. I dinhmun te, i mimal chanchin te, leh i mamawh enkawlna chipchiar tak takte a zirin engkim a hrilhfiah thei a ni.

Enkawlnaah hian harsatna a awm em?

He natna avang hian i tissue te chu a chak lo hle a, thisen chhuah theihna a sang zawk . Tin, operation hnuah pawh dam leh harsa tak a ni thei a, mahse hei hi chu tissue te chak lohna vang a ni. I doctor chuan i tawn theih tur side effect leh risk te chu a sawifiah tha ber thei ang.

Engtin nge ka in enkawl/symptoms ka enkawl?

Vascular EDS hi natna khirh tak a ni a , damdawi lam pawhin uluk taka enkawl a ngai a , doctor pan fo a ngai bawk . A buaithlak em avangin doctor puihna tel lo chuan nangmah chauhin i enkawl thei lo va, i enkawl thei lo bawk. Chuvang chuan doctor thupek zawm a pawimawh hle.

Hei hi ven dan tur a awm em?

Vascular EDS hi genetic condition a nih avangin a vei theihna tur leh a vei theihna tihziaawmna tur a awm lo. Hetiang natna vei te hian naupai emaw, fa neih emaw an tum a nih chuan an doctor nen genetic counseling chungchang an sawipui tur a ni. Chu chuan an fain he natna hi a rochun chuan eng nge an beisei tih an hrethiam ang.

Hetiang dinhmun hi ka neih chuan eng nge ka beisei theih ang? Eng ang thlirna nge ni ang?

Vascular EDS vei te hi thisen chhuah emaw, chhungril tissue leh organ chak lo emaw avanga harsatna leh harsatna an neih theihna a sang hle.

Hetiang natna vei tam zawk chuan kum 20 an tlin chuan harsatna lian tak pakhat tal an tawk ang a , kum 40 an tlin chuan nunna atana hlauhawm harsatna an neih theihna chu 80% vel a ni . Statistics atanga a lan dan chuan he natna vei zinga a chanve vel chu kum 48 thleng an dam .

Mahse he thu hi hre hlau suh. A chhan chu medical science a lo thang chho zel a, heng dinhmunte hi enkawl dan thar a lo chhuak a, miten a hma aia nun tha zawk an nei thei ta a ni.

Hetiang dinhmun hi eng chen nge a daih ang?

Vascular EDS hi pian tirh atanga awm, dam chhung zawnga awm thin a ni.

Engtin nge ka in enkawl ang?

Vascular EDS vei te hian doctor pan fo tur a ni a, an dinhmun enfiah a, harsatna thar an neih leh neih loh enfiah thin tur a ni.

Tin, chubakah, .

• Inkhelh emaw thil tih hlauhawm tak tak te chu i pumpelh tur a ni .
• Thil rit phurh emaw, hliam hlauhawmna sang tak nei thil dang emaw i tih loh tur a ni.
• Tin, thisen chhuah tam lutuk leh chhungril lama hliam a awm theihna a san avangin elective surgery tih loh hi a finthlak hle bawk.

Engtikah nge Doctor ka hmuh ang? / Engtikah nge emergency treatment ka zawn ang?

A rawtna angin doctor hmu fo ang che. Doctor i koh tur emaw, i hmuh hun tur emaw, symptom i neih leh neih loh te pawh a hrilh ang che.

Emergency-ah chuan chu chu:

• Hetiang natna i neih chuan chhan hriat loha natna nasa tak i neih chuan emergency medical attention i pan nghal tur a ni a, a bik takin i rilru emaw, i pum emaw ah
• Tin, pawn lam hliam thisen chhuak nasa tak emaw, tui chhuak emaw i neih chuan emergency medical attention zawng nghal rawh.

Take-Home Thuchah hnuhnung ber

Vascular Ehlers-Danlos Syndrome (vEDS) hi natna khirh tak, genetic disease a ni tih hi a dik a ni. Damdawi lam uluk taka enkawl leh enkawl a ngai a ni. Hei hi i tan chuan hlauhawm tak angin a lang thei a, mahse damdawi lama hmasawnna leh natna hriatthiamna avang hian tunah chuan he natna vei te hian kum kalta aiin an dam rei thei tawh a, an dam rei thei tawh lo tih hre reng ang che. Chuvang chuan doctor thurawn zawm a, hriselna enkawl a pawimawh. Nangmah emaw, i hriat emaw hian he thil chungchangah hian zawhna i neih chuan doctor biak hreh suh.

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