Thaum koj tus menyuam yug los, koj puas tau pom ib lub cim loj, xim liab tsaus, xim cawv txiv hmab ntawm koj tus menyuam lub cev? Los yog, thaum koj tus menyuam loj hlob, ib ceg puas zoo li ntev dua thiab tuab dua lwm ceg? Koj kuj tseem yuav pom tias muaj cov leeg ntshav uas ntswj. Yog tias koj tus menyuam muaj ib lossis ntau yam ntawm cov tsos mob no, nws yog ib qho tseem ceeb rau koj kom paub txog tus mob tsis tshua muaj uas peb tab tom tham txog hnub no hu ua Klippel-Trenaunay Syndrome (KTS). Txawm hais tias nws yuav zoo li nyuaj, cia peb ua kom yooj yim.
Hais yooj yim xwb, Klippel-Trenaunay Syndrome (KTS) yog dab tsi?
KTS yog ib yam mob uas tshwm sim thaum yug los. Nws tsuas cuam tshuam li ib ntawm ib lab tus tib neeg thoob ntiaj teb xwb. Yog li ntawd, nws tsis yog ib qho uas tshwm sim ntau heev. Muaj peb yam teeb meem tseem ceeb uas pom tau rau ib tus neeg uas muaj tus mob no.
1. Ib lub cim liab ntawm daim tawv nqaij: Qhov no kuj hu ua "Port-wine stain" vim nws zoo li xim ntawm cawv me ntsis.
2. Cov leeg ntshav varicose: Qhov no yog qhov mob uas peb hu ua "cov leeg ntshav varicose".
3. Ib sab caj npab lossis ib ceg loj dua lwm sab: Ib sab ceg tshwj xeeb yuav loj dua lossis tuab dua lwm sab.
Ntxiv rau peb yam tsos mob tseem ceeb no, qee tus neeg kuj tseem yuav muaj teeb meem nrog lub cev lymphatic, uas pab tswj kom cov kua dej hauv peb lub cev sib npaug.
Cov kws kho mob qee zaum hu qhov no tias "CLVM." Qhov no txhais tau tias qhov mob no cuam tshuam rau ntau qhov ntawm peb lub cev.
- C (Capillaries): Cov no yog cov hlab ntsha me me uas txuas peb cov leeg thiab cov hlab ntsha. Lub cim yug liab ntawd yog tshwm sim los ntawm kev hloov pauv ntawm cov no.
- L (Lymphatic system): Qhov ntawd txhais tau tias lymphatic system. Ib feem ntawm peb lub cev tiv thaiv kab mob.
- V (Cov leeg ntshav): Qhov ntawd txhais tau tias cov leeg ntshav. Cov hlab ntsha uas nqa ntshav mus rau lub plawv.
- M (Malformation): Qhov no txhais tau tias ib feem tsis tau loj hlob li qub.
Tus mob no tau tshawb pom thawj zaug los ntawm ob tug kws kho mob Fabkis xyoo 1900. Yog vim li ntawd tus mob no thiaj li raug muab lub npe rau lawv.
Cov tsos mob ntawm ib tus neeg uas muaj KTS yog dab tsi?
Cia peb los saib seb qhov mob no cuam tshuam li cas rau cov hlab ntsha, cov nqaij mos, cov pob txha, thiab cov hlab ntsha lymphatic ntawm tus neeg uas muaj KTS. Yuav kom nkag siab cov tsos mob no kom meej, saib lub rooj hauv qab no.
| Cov tsos mob | Ib qho lus piav qhia yooj yim |
|---|---|
| Xim av cawv txiv hmab | Qhov no yog thawj qhov cim qhia ntawm KTS. Nws yog tshwm sim los ntawm o ntawm cov hlab ntsha me me (capillaries) hauv qab daim tawv nqaij. Nws tuaj yeem hloov xim los ntawm xim liab dawb mus rau xim liab tsaus. Thaum koj laus zuj zus, qhov chaw no yuav tsaus dua lossis sib dua. Qee zaum, cov pob me me tshwm sim ntawm qhov chaw, uas tuaj yeem tawg thiab los ntshav. |
| Cov leeg ntshav varicose | Tus mob no tuaj yeem cuam tshuam rau yuav luag txhua tus neeg uas muaj KTS. Thaum cov teeb meem no tshwm sim hauv cov leeg ntshav uas nyob rau ntawm daim tawv nqaij, cov leeg ntshav hauv ob txhais ceg, tshwj xeeb tshaj yog hauv qhov chaw mos thiab sab nraub qaum, dhau los ua xim xiav. Qee zaum, nws kuj tseem tuaj yeem cuam tshuam rau cov leeg ntshav uas khiav tob hauv lub cev. Yog tias qhov no tshwm sim, muaj kev pheej hmoo ntawm cov ntshav txhaws (Deep Vein Thrombosis - DVT). |
| Kev loj hlob tsis zoo ntawm ib txhais caj npab lossis ceg | Feem ntau, tsuas yog ib sab caj npab lossis ceg loj dua lwm sab. Qhov no tuaj yeem pib thaum yau. Nws feem ntau cuam tshuam rau ib ceg. Ib ceg yuav ntev dua lossis tuab dua lwm ceg. Qhov no tuaj yeem ua rau muaj teeb meem taug kev thiab txav mus los. |
| Cov teeb meem ntawm lub cev lymphatic | Tej zaum ib txhia neeg yuav muaj cov hlab ntsha lymph uas loj dhau los yog tsis zoo li qub. Thaum cov hlab ntsha no tsis ua haujlwm zoo, lawv tuaj yeem xau cov kua lymphatic, ua rau ob txhais ceg o, lossis ua rau lub duav, lub zais zis, lossis teeb meem plab hnyuv. |
| Mob | Cov leeg ntshav yuav khaus lossis mob. Teeb meem nrog kev ncig ntshav tuaj yeem ua rau ob txhais ceg o thiab mob. Tsis tas li ntawd, ob txhais ceg loj tuaj yeem hnov qhov hnyav lossis mob. |
Vim li cas qhov xwm txheej no thiaj tshwm sim tiag tiag?
Feem ntau, KTS yog tshwm sim los ntawm kev hloov pauv hauv ib qho ntawm peb cov noob caj noob ces. Yog hais kom meej, nws yog tshwm sim los ntawm kev hloov pauv hauv cov noob caj noob ces hu ua PIK3CA.
Qhov tseem ceeb yog qhov no tsis yog ib yam dab tsi uas tau txais los ntawm niam mus rau menyuam. Qhov kev hloov pauv caj ces no tshwm sim qee zaus, tsis muaj laj thawj dab tsi. Yog li ntawd, ua niam txiv, tsis muaj laj thawj yuav tu siab lossis liam koj tus kheej rau qhov no.
Qee zaum cov neeg uas tsis muaj kev hloov pauv hauv PIK3CA gene kuj muaj KTS, yog li cov kws tshawb fawb ntseeg tias lwm yam kev hloov pauv caj ces yuav yog lub luag haujlwm.
Muaj lwm yam teeb meem dab tsi tshwm sim vim yog KTS?
Yog tias KTS tsis raug tswj hwm kom zoo, qee qhov teeb meem tuaj yeem tshwm sim, thiab nws yog ib qho tseem ceeb kom paub txog lawv.
- Cov Ntshav Txhaws: Cov ntshav txhaws (DVT) tuaj yeem tsim nyob rau hauv cov leeg ntshav tob ntawm ob txhais ceg. Qhov kev pheej hmoo loj tshaj plaws yog tias yog cov ntshav txhaws no tawg thiab daig rau hauv cov leeg ntshav hauv lub ntsws, nws tuaj yeem ua rau muaj kev phom sij rau lub neej (Pulmonary Embolism).
- Kab mob ntawm daim tawv nqaij: Cov kab mob ntawm cov kab mob uas tshwm sim hauv qab daim tawv nqaij (cellulitis) tuaj yeem tshwm sim.
- Kev o: Teeb meem nrog lub cev lymphatic tuaj yeem ua rau cov kua dej sib sau ua ke hauv lub cev thiab ua rau o (Lymphedema).
- Kev los ntshav sab hauv: Kev los ntshav yuav tshwm sim los ntawm lub plab, txoj hnyuv, zis, lossis rau cov poj niam, lub cev kev yug me nyuam.
Tsis tshua muaj, qee tus neeg uas muaj KTS kuj tseem yuav muaj teeb meem yug los uas cuam tshuam nrog cov ntiv tes lossis cov ntiv taw.
- Muaj cov ntiv tes ntxiv (Polydactyly)
- Syndactyly (cov ntiv tes sib koom ua ke)
Tus kws kho mob yuav kuaj mob KTS li cas kom raug?
Tus kws kho mob yuav xub kuaj mob los ntawm kev saib tus menyuam lub cev zoo li cas. Yog tias muaj tsawg kawg yog ob ntawm peb yam tseem ceeb uas peb tau tham ua ntej (cov cim yug menyuam, cov leeg ntshav, thiab cov ceg loj), nws yuav xav tias nws yog KTS. Vim tias cov cim no feem ntau pom thaum yug los, muaj qee lub sijhawm uas nws tuaj yeem kuaj mob ua ntej tus menyuam mus tsev los ntawm tsev kho mob.
Tus kws kho mob yuav xaj ntau yam kev kuaj mob kom paub meej tias muaj tus mob no thiab nrhiav seb muaj dab tsi cuam tshuam rau lub cev.
| Kev Ntsuas | Koj pom dab tsi hauv qhov no? |
|---|---|
| Kev luam theej duab CT lossis MRI | Tshawb xyuas seb cov nqaij mos thiab pob txha ntawm lub cev zoo li cas, thiab seb puas loj hlob tsis zoo. |
| Kev Siv Tshuaj Ntsuam Xyuas Lub Hlwb Hlau Sib Nqus (MRA) | Qhov no yog ib hom kev luam theej MRI tshwj xeeb. Nws tuaj yeem pom cov hlab ntsha thiab cov leeg ntshav kom meej heev. |
| Doppler Ultrasound | Saib seb cov ntshav ntws li cas los ntawm cov leeg ntshav thiab cov hlab ntsha, thiab saib seb puas muaj qhov twg thaiv lossis ntws rov qab. |
Cov kev kho mob rau KTS yog dab tsi?
Tsis muaj tshuaj kho tau KTS. Txawm li cas los xij, muaj ntau txoj kev kho mob uas tuaj yeem pab tib neeg tswj lawv cov tsos mob thiab teeb meem thiab ua neej nyob li qub. Kev kho mob nyob ntawm tus neeg cov tsos mob.
| Txoj kev kho mob | Ua li cas rau nws? |
|---|---|
| Cov tshuaj ua kom cov ntshav nyias (Anticoagulants) | Cov tshuaj no pab txo qhov kev pheej hmoo ntawm cov ntshav txhaws. |
| Cov tshuaj Sirolimus | Cov tshuaj no ua rau cov hlab ntsha tsis zoo loj hlob qeeb lossis nres, yog li ntawd txo cov tsos mob thiab teeb meem. |
| Cov thom khwm nias | Cov no pab txo qhov o thiab mob ntawm ob txhais ceg, thiab txo qhov kev pheej hmoo ntawm cov ntshav txhaws. Lawv pab cov ntshav ntws los ntawm ob txhais ceg mus rau lub plawv. |
| Cov khoom nqa khau | Yog tias koj ob txhais ceg tsis ntev ib yam, ntxiv ib ntu siab dua rau ib lub khau tuaj yeem ua rau kev taug kev yooj yim dua. Qhov no kuj pab txo qhov kev pheej hmoo ntawm scoliosis. |
| Kev kho mob laser | Txoj kev kho no yog siv los ua kom cov xim ntawm "port-wine stain" ntawm daim tawv nqaij dawb dua. |
| Kev kho mob sclerotherapy | Yuav muab ib yam kua tshwj xeeb txhaj rau hauv cov leeg ntshav uas muaj teeb meem, uas yuav ua rau lawv tsis ua haujlwm thiab kaw. Qhov no yog ib txoj kev kho mob zoo rau cov leeg ntshav varicose. |
| Kev phais mob | Kev phais mob yuav ua tau los kho cov teeb meem loj ntawm cov leeg ntshav, ua kom ceg ntev sib npaug, lossis txo qhov loj ntawm caj npab/ceg uas loj los ntawm kev tshem tawm cov nqaij ntau dhau. |
Muaj tej yam dab tsi uas yuav tsum tau ceev faj tshwj xeeb thaum nyob nrog KTS?
Thaum nyob nrog KTS, nws yog ib qho tseem ceeb heev kom nkag siab zoo txog koj lub cev thiab saib xyuas qee yam.
- Saib xyuas koj daim tawv nqaij kom zoo: Kev ua kom koj daim tawv nqaij huv thiab tsis raug mob tuaj yeem txo qhov kev pheej hmoo ntawm kev kis kab mob ntawm daim tawv nqaij.
- Ua kom koj lub cev nquag li sai tau: Tsis txhob nyob hauv tib qho chaw ntev dhau. Kev tawm dag zog thiab kev taug kev tsis tu ncua tuaj yeem txo qhov kev pheej hmoo ntawm cov ntshav txhaws.
- Hnav cov khaub ncaws nruj raws li koj tus kws kho mob qhia: Yog tias koj tus kws kho mob tau sau ntawv kom hnav, ib txwm hnav cov thom khwm tshwj xeeb lossis cov khaub ncaws uas txo qhov o.
- Tsis txhob siv qee yam tshuaj tiv thaiv kev yug me nyuam: Tham nrog koj tus kws kho mob txog kev tsis txhob siv cov tshuaj tiv thaiv kev yug me nyuam uas muaj cov tshuaj hormones estrogen, vim tias lawv ua rau muaj kev pheej hmoo ntawm cov ntshav txhaws.
- Thaum cev xeeb tub thiab ua ntej phais: Thaum lub sijhawm no, koj yuav tsum noj tshuaj uas ua rau cov ntshav nyias kom tiv thaiv cov ntshav txhaws, vim tias qhov kev pheej hmoo ntawm cov ntshav txhaws ntau dua. Tham nrog koj tus kws kho mob txog qhov no thiab tau txais kev qhia.
Kuv yuav tsum mus ntsib kws kho mob thaum twg? Lub sijhawm twg mus rau ETU?
Nws yog ib qho tseem ceeb rau ib tus neeg uas muaj KTS kom mus kuaj mob tsis tu ncua thoob plaws lawv lub neej. Qhov no yog thaum uas tus mob tuaj yeem saib xyuas tau, kev kho mob tuaj yeem pom tias ua haujlwm tau zoo, thiab txhua yam kev hloov pauv tsim nyog tuaj yeem ua tau.
Txawm li cas los xij, qee zaum koj yuav tsum mus rau tsev kho mob thaum muaj xwm txheej ceev. Nco ntsoov txog cov tsos mob no.
Nov yog cov xwm txheej thaum koj xav tau mus rau ETU (Emergency Treatment Unit) sai sai:
* Yog tias koj pom cov cim qhia tias muaj ntshav txhaws: Yog tias ib ceg cia li mob, o, liab, thiab sov so thaum kov, nws yuav yog qhov mob ntshav txhaws tob (DVT).
* Yog tias koj muaj cov tsos mob ntawm cov ntshav txhaws hauv lub ntsws: Mob hauv siab tam sim ntawd, ua pa nyuaj, ua pa sai, lossis hnoos ntshav, nws yuav yog pulmonary embolism. Qhov no yog qhov xwm txheej ceev.
* Yog tias muaj kev los ntshav hnyav heev: Yog tias muaj kev los ntshav uas tsis tuaj yeem tswj tau los ntawm txhua qhov chaw hauv lub cev.
Yog koj pom ib qho ntawm cov cim no, tsis txhob nkim sijhawm thiab mus rau Chav Kho Mob Ceev (ETU) ze tshaj plaws tam sim ntawd.
KTS yog ib qho mob nyuaj heev, yog li koj yuav muaj ntau lo lus nug. Txhua zaus koj mus ntsib koj tus kws kho mob, mloog txhua yam uas nyob hauv koj lub siab. Tsis txhob xav tias, "Qhov no tsuas yog ib qho teeb meem uas kuv tsis tas yuav daws." Nws yog qhov zoo tshaj plaws kom qhib siab thiab ncaj ncees nrog koj tus kws kho mob.
Cov Lus Qhia Txog Kev Coj Mus Tsev
- Klippel-Trenaunay Syndrome (KTS) yog ib qho mob tsis tshua muaj thaum yug los uas tsis yog los ntawm qhov txhaum ntawm niam txiv.
- Peb yam tsos mob tseem ceeb yog: lub cim yug me nyuam liab tsaus (xim av caw), cov leeg ntshav varicose, thiab ib sab caj npab lossis ceg loj dua lwm sab.
- Txawm hais tias tus mob no tsis tuaj yeem kho tau tag nrho los xij, muaj ntau yam kev kho mob uas tuaj yeem pab koj tswj koj cov tsos mob thiab ua lub neej zoo heev.
- Cov teeb meem tseem ceeb tshaj plaws uas yuav tsum tau ceev faj yog cov ntshav txhaws (DVT, Pulmonary Embolism) thiab los ntshav ntau dhau. Nco ntsoov paub txog cov cim ceeb toom ntawm cov no.
- Nws yog ib qho tseem ceeb heev uas yuav tsum sib txuas lus nrog koj tus kws kho mob thoob plaws hauv koj lub neej thiab mus kuaj mob tsis tu ncua.
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Tsis muaj lus teb tau muab tso tawm. Ntxiv koj cov lus tawm ntawm no thawj zaug.
Ntxiv koj cov lus