I naute pian tirh khan a taksaah birthmark lian tak, dum dum, wine rawng, i hmu em? A nih loh leh, i naute a lo seilian chhoh zêl chuan, ke pakhat chu a ke dang aiin a sei zâwkin a thuk zâwk niin a lang em? Vein inzawm khawm a awm tih pawh i hmu mai thei. I naute hian heng symptoms zinga pakhat emaw a aia tam emaw a neih chuan tunlaia kan sawi tak natna hmuh tur awm lo tak, Klippel-Trenaunay Syndrome (KTS) tih hi i hriat a pawimawh hle. Thil buaithlak tak angin lang mah se, a awlsam zawngin i dah ang u.
A awlsam zawngin, Klippel-Trenaunay Syndrome (KTS) hi eng nge ni?
KTS hi pianpui natna a ni a, pian tirh atanga lo awm thin a ni. Khawvel puma mi maktaduai khat zinga pakhat vel chauh a nghawng a ni. Chuvangin a tam vak lo. Hetiang natna nei mi pakhatah hian harsatna lian tham pathum hmuh theih a awm.
1. Vunah birthmark sen: Hei hi ``Port-wine stain'' an ti bawk a, a chhan chu wine tlemte rawng nen a inang a ni.
2. Varicose veins: Hei hi ``Varicose veins'' kan tih hi a ni.
3. Kut emaw ke pakhat emaw chu a dang aiin a lian zawk: A bik takin ke pakhat chu a dang aiin a sei emaw, a sei emaw pawh a ni thei.
Heng symptom lian pathum bakah hian mi thenkhat chuan lymphatic system-ah pawh harsatna an nei thei a, hei hian kan taksaa fluid balance awm reng turin a pui a ni.
Doctor-te chuan hei hi ``CLVM'' an ti \hin.Hei hian kan taksa peng engemaw zat a nghawng tihna a ni.
- C (Capillaries): Hengte hi kan thisen kalna kawng leh thisen kalna kawng hrang hrang inzawmkhawmtu thisen kalna kawng te tak te te an ni. Chu birthmark sen chu hengte hi a inthlak danglam vang a ni.
- L (Lymphatic system): Chu chu lymphatic system tihna a ni. Kan taksa hriselna (immune system) thenkhat.
- V (Veins): Chu chu veins tihna a ni. Thinlung thlenga thisen phurtu thisen kalna kawngte.
- M (Malformation): Chumi awmzia chu part pakhat chu a pangngaiin a lo thanglian lo tihna a ni.
He natna hi French doctor pahnih chuan kum 1900 khan an hmuchhuak hmasa ber a, chuvang chuan he syndrome hi an hming chawia an vuah a ni.
KTS vei mi pakhatin eng symptoms nge a neih?
He natna hian KTS vei thisen kalna kawng, soft tissue, ruh leh lymphatic vessel te a nghawng dan hi ngun zawkin han thlir ila. Heng symptoms te hi chiang taka hrethiam tur chuan a hnuaia table hi en la.
| Symptom a ni | A sawifiahna awlsam tak |
|---|---|
| Port-wine a ni | Hei hi KTS chhinchhiahna hmasa ber a ni thei. Vun hnuaia thisen kalna (capillaries) te tak te a hring (swelling) avanga lo awm a ni. A rawng hi light pink atanga dark wine-red thlengin a danglam thei. I tar deuh deuh chuan he hmun hi a dum emaw, a eng emaw a ni thei. A chang chuan a hmunah hian blister te te a lo lang a, chu chu a puak thei a, thisen a chhuak thei bawk. |
| Varicose veins a awm thin | Hetiang dinhmun hian KTS vei zawng zawng deuhthaw a nghawng thei a ni. Heng harsatnate hi vun chunglama awm vein-ah a lo awm chuan ke ruh, a bik takin groin leh thighs-a vein te chu a rawng a sen vek thin. A châng chuan, taksa chhungril thuk taka kal vein-te pawh a nghawng thei bawk. Chutiang a nih chuan thisen zam (Deep Vein Thrombosis - DVT) a awm theihna a awm. |
| Kut emaw ke emaw a lo thang dan pangngai lo | Vawi tam tak chu kut emaw, ke pakhat emaw chauh chu a dang aiin a lian zawk thin. Hei hi nausen lai atanga tan theih a ni. Ke pakhat a nghawng tam ber. Ke pakhat chu a dang aiin a sei emaw, a thuk emaw pawh a ni thei. Hei hian kea kal leh che a harsat phah thei a ni. |
| Lymphatic system lama harsatna awm thei | Mi thenkhat chuan lymph vessels extra emaw abnormally shaped emaw an nei thei. Hengte hian hna a thawh that loh chuan lymphatic fluid a leak thei a, keah a hring thei a, pelvic, bladder emaw bowel problem a thlen thei bawk. |
| Na | Veins te hi a itch emaw, a na emaw thei. Thisen kal dan lama harsatna a awm chuan ke a ti na a, a na thei bawk. Tin, ke lian zawk chu a rit emaw, a na emaw a ni thei bawk. |
Engvangin nge hetiang dinhmun hi a lo awm tak tak?
A tam zawkah chuan KTS hi kan gene pakhat a inthlak danglam vang a ni. A dik tak chuan PIK3CA an tih gene-a mutation awm vang a ni.
A pawimawh ber chu hei hi nu atanga fate rochun thil a ni lo. He genetic mutation hi a awm fo thin a, chhan awm lovin a thleng thin. Chuvang chuan nu leh pa kan nih angin he thila lungngaihna emaw, mahni intihhlumna chhan tur a awm lo.
A chang chuan PIK3CA gene-a inthlak danglamna nei lote pawhin KTS an nei ve bawk a, chuvangin zirchiangtute chuan genetic inthlak danglamna dang pawh hi a mawhphurtu a ni thei niin an ngai.
KTS vang hian eng harsatna dang nge awm thei?
KTS hi uluk taka enkawl a nih loh chuan harsatna engemaw zat a awm thei a, chu chu hriat chian a pawimawh hle.
- Thisen zam: Ke ruh thûk takah thisen zam (DVT) a lo awm thei a. A hlauhawm ber chu he thisen zam hi a lo chhuak a, lung chhunga vein pakhatah a tlakbuak chuan nunna atana hlauhawm (Pulmonary Embolism) a ni thei.
- Vun natna: Vun hnuaia bacteria infection (cellulitis) a awm thei.
- Swelling: Lymphatic system lama harsatna a awm chuan taksaah tui a awm khawm a, swelling (Lymphedema) a thlen thei.
- Internal bleeding: Pum, intestine, bladder atanga thisen chhuak a ni thei a, hmeichhiaah chuan reproductive system atanga thisen a chhuak thei bawk.
A tlem hle a, KTS vei thenkhat chuan nau piangsualna an nei thei bawk a, chu chu kut zungtang emaw ke ruh emaw a ni.
- Kut zungtang dang neih (Polydactyly) .
- Syndactyly (kut zungtang inzawm khawm) .
Engtin nge doctor hian KTS hi dik taka a hriat theih ang?
Doctor chuan naute taksa pianzia enin a natna hi a hre hmasa ang. A hmaa kan sawi tawh chhinchhiahna lian pathum (birthmarks, spider veins, leh enlarged limbs) zinga pahnih tal a awm chuan KTS a ni thei ang tih rinhlelh a ni. Heng chhinchhiahnate hi pian tirh atanga hmuh theih a nih fo avangin damdawi in atanga naute a haw hmaa hriat theih hun a awm thin.
Doctor chuan he natna hi finfiah turin test hrang hrang a ti thei a, taksaah eng nge a nghawng tih chiang takin a hre thei bawk.
| Fiahna | Hemiah hian eng nge i hmuh? |
|---|---|
| CT scan emaw MRI scan emaw hmanga tih theih a ni | Taksa soft tissue leh ruh awm dan te, a lo than dan pangngai lo nasat zia te enfiah thin ang che. |
| MRA (Magnetic Resonance Angiogram) hmanga chhut a ni. | Hei hi MRI scan chi khat bik a ni. Thisen kalna kawng leh thisen kalna kawngte dinhmun chiang takin a hmu thei a ni. |
| Doppler hmanga Ultrasound hmanga tih a ni | Vein leh arteries-a thisen kal dan en la, khawi hmunah pawh blockage emaw backflow emaw a awm em tih en rawh. |
KTS enkawl dan tur chu engte nge ni?
KTS hi damdawi a awm lo. Mahse, miten an natna lan chhuah dan leh an harsatnate an enkawl theih nan leh nun pangngaia an nun theih nan enkawlna tam tak a awm. Enkawlna chu mimal natna lan chhuah danah a innghat a ni.
| Enkawl dan tur | Eng thil nge thleng? |
|---|---|
| Thisen tithianghlimtu (Anticoagulants) . | He damdawi hian thisen zam a tihziaawm theih nan a pui a ni. |
| Damdawi chu Sirolimus a ni | He damdawi hian thisen kalna kawng dik lote chu a tikhawtlai emaw, a titawp emaw a, chu chuan natna lanchhuahna leh harsatna awmte a tiziaawm a ni. |
| Compression Stockings te chu a awm a | Hengte hian ke ruh natna leh natna tihziaawmna atan a pui a, thisen zam awm theihna a tiziaawm bawk. Ke atanga thinlung thlenga thisen kal turin an pui thin. |
| Kekawrte chawi sang | I ke a sei zawng a inang lo a nih chuan pheikhawk pakhatah section sang zawk dah belh chuan kal a ti awlsam thei a ni. Hei hian scoliosis vei theihna a tihziaawm bawk. |
| Laser hmanga enkawl a ni | He treatment hi vunah `port-wine stain` birthmark rawng tihthianghlim nan an hmang thin. |
| Sclerotherapy hmanga enkawl a ni | Buaina nei vein-ah hian tui bik an inject a, chu chuan a ti deactivate a, a seal bawk. Hei hi varicose veins enkawlna tha tak a ni. |
| Inzai | Vein lama harsatna lian tham siam that nan te, ke sei zawng inang turin emaw, tissue tam lutuk lakchhuahna hmanga kut/ke lian zawk lian tham tak tihtlem nan surgery tih theih a ni. |
KTS nena kan awm hian eng thilte nge fimkhur bik tur?
KTS nena kan khawsak hian i taksa hriatthiamna tha tak neih a pawimawh hle a, thil engemaw tak enkawl a pawimawh hle.
- I vun enkawl tha rawh: I vun thianghlim leh hliam awm lova vawn reng hian vun natna vei theihna a ti tlem thei a ni.
- A theih ang anga thawkrim rawh: Hmun khatah rei tak awm suh. Exercise neih fo leh kea kal hian thisen zam a tihziaawm thei a ni.
- Doctor-in a tih angin compression garment ha rawh: Doctor-in a pek a nih chuan socks bik emaw, hliam tihziaawmna tur thawmhnaw emaw ha fo ang che.
- Naupai venna kawng thenkhat pumpelh rawh: Naupai venna pill ang chi hormone estrogen awmna te ei loh tur chungchang sawipui la, thisen zam theihna a tipung a ni.
- Naupai lai leh zai hma: Heng hun lai hian thisen zam tur venna atan thisen tihthianghlimna damdawi ei a ngai mai thei, thisen zam theihna a sang zawk avangin. Hemi chungchang hi i doctor nen sawiho la, thurawn la rawh.
Engtikah nge Doctor ka hmuh ang? ETU-a kal hun tur chu engte nge ni?
KTS vei tan chuan a dam chhungin check-up neih fo a pawimawh hle. Hei hi a dinhmun enfiah theih hun a ni a, enkawlna a thawk tha tih hmuh theih a ni a, a tul anga tihdanglam theih a ni bawk.
Mahse, a chang chuan emergency-ah damdawi in pan a ngai mai thei. Heng symptoms te hi fimkhur rawh.
Hengte hi ETU (Emergency Treatment Unit)-a kal vat a ngaihna tur dinhmunte chu a ni:
* Thisen zam chhinchhiahna i hmuh chuan: Ke pakhat a na thut a, a hring a, a sen a, khawih pawhin a lum a nih chuan deep vein thrombosis (DVT) a ni thei.
* Lung chhunga thisen zam (blood clot) chhinchhiahna i neih chuan: I rilru natna, thawk harsa, thawk rang, emaw thisen khuh emaw i neih chuan pulmonary embolism a ni thei. Hei hi emergency a ni.
* Thisen chhuak nasa tak a awm chuan: Taksa chhung khawi hmun atang pawha thunun theih loh thisen chhuak a awm chuan.
Heng chhinchhiahnate hi i hmuh chuan hun khawhral lo la, damdawi in hnai ber Emergency Department (ETU)-ah kal nghal rawh.
KTS hi dinhmun khirh tak a nih avangin zawhna tam tak i nei mai thei. Doctor i hmuh apiangin i rilru a thil awm zawng zawng chu ngaithla thin ang che. "Hei hi ka hmachhawn ngai lo buaina mai a ni," tiin ngaihtuah suh. Doctor hnenah inhawng leh rinawm taka sawi a tha ber.
Take-Home Thuchah a ni
- Klippel-Trenaunay Syndrome (KTS) hi piansualna hmuh tur awm lo tak a ni a, nu leh pa te tihsual vang a ni lo.
- A lan chhuah dan ber pathum chu: pian chhinchhiahna dum dum (port-wine stain), varicose veins, leh kut emaw ke pakhat emaw chu a dang aiin a lian zawk a ni.
- He natna hi tihdam kim theih ni lo mah se, i natna lan chhuah dan tur enkawl thei tur leh nun tha tak nei thei tur enkawlna tam tak a awm a ni.
- Complication fimkhur tur pawimawh ber chu thisen zam (DVT, Pulmonary Embolism) leh thisen chhuah tam lutuk hi a ni. Hengte vaukhânna chhinchhiahnate hi hre reng ang che.
- I dam chhung zawnga doctor nena inzawmna nei reng tur leh checkup neih reng a pawimawh hle.
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