Koj tus menyuam daim tawv nqaij puas liab thiab o? Cia peb kawm txog Klippel-Trenaunay Syndrome (KTS)!

Koj puas tau pom tias muaj ib txhia menyuam yug los muaj ib lub cim liab loj rau ib sab ntawm lawv lub cev, tshwj xeeb tshaj yog rau ntawm ib sab caj npab lossis ceg, thiab sab caj npab/ceg ntawd loj dua sab tod me ntsis? Los yog qee zaum koj tuaj yeem pom cov leeg ntawm sab ntawd? Nws yog ib qho ib txwm muaj kev ntshai me ntsis thaum koj pom ib yam dab tsi zoo li no. Hnub no peb yuav tham txog ib qho mob uas qhia cov tsos mob zoo sib xws, uas yog qhov tsis tshua muaj, txhais tau tias tsis yog txhua tus neeg muaj nws, thiab yog yug los. Qhov no hu ua Klippel-Trenaunay Syndrome (KTS) . Tsis txhob txhawj, peb yuav tham txog qhov no yooj yim, hauv txoj kev uas koj tuaj yeem nkag siab.

Klippel-Trenaunay Syndrome (KTS) yog dab tsi? Hais yooj yim xwb...

Xav txog seb, KTS yog ib yam mob tsis tshua muaj uas los nrog peb tus menyuam thaum nws los rau hauv lub ntiaj teb no (uas yog, ib yam mob uas muaj txij thaum yug los ). Qhov no ua rau muaj qee qhov kev hloov pauv me me hauv txoj kev uas tus menyuam cov nqaij mos, pob txha, thiab cov hlab ntsha loj hlob. Ib qho ntawm cov tsos mob tseem ceeb ntawm qhov no yog qhov tshwm sim ntawm lub cim yug liab-liab, feem ntau ntawm ib sab caj npab lossis ceg, hu ua 'port-wine stain' . Feem ntau, cov neeg uas muaj KTS no kuj muaj qee qhov teeb meem nrog lawv lub cev lymphatic . Lub cev lymphatic no yog ib qho tseem ceeb uas pab kom peb cov kua dej hauv lub cev sib npaug.

Tam sim no tsis muaj tshuaj kho tau KTS. Txawm li cas los xij, tsis txhob ntshai. Muaj ntau txoj kev kho mob zoo los tswj cov tsos mob . Yog tias kuaj pom thiab kho tus mob sai li sai tau, xws li tom qab tus menyuam yug los, cov teeb meem kev noj qab haus huv uas tuaj yeem tshwm sim vim KTS tuaj yeem txo qis heev.

Qee cov kws kho mob kuj hu qhov mob KTS ua CLVM . Qhov no yog thawj tsab ntawv ntawm plaub lo lus Askiv.

• C yog rau Capillaries - cov no yog cov hlab ntsha me me uas txuas peb cov leeg thiab cov hlab ntsha.
• L sawv cev rau Lymphatic system - Qhov no yog ib feem ntawm peb lub cev tiv thaiv kab mob. Nws nqa cov kua dej hu ua lymph thoob plaws lub cev.
• V sawv cev rau Cov leeg ntshav - cov no yog cov hlab ntsha uas nqa ntshav mus rau peb lub plawv.
• Tsab ntawv M sawv cev rau Malformation . Nws txhais tau tias ib feem ntawm lub cev tsis loj hlob li qub, lossis muaj qhov tsis zoo.

KTS tau muab lub npe rau ob tug kws kho mob Fabkis, Maurice Klippel thiab Paul Trenaunay, uas tau tshawb pom tus mob no hauv xyoo 1900. Cov kws tshaj lij kwv yees tias kwv yees li 1 ntawm 100,000 tus neeg thoob ntiaj teb raug tus mob no. Nws tuaj yeem cuam tshuam rau txhua tus neeg, tsis hais haiv neeg lossis poj niam txiv neej.

Cov tsos mob ntawm KTS yog dab tsi? Koj paub nws li cas?

Cov tsos mob ntawm Klippel-Trenaunay Syndrome (KTS) feem ntau cuam tshuam rau cov leeg ntshav, cov hlab ntsha me me, cov nqaij mos, cov pob txha, thiab cov hlab ntsha lymph hauv peb lub cev. Cia peb saib seb yuav ua li cas:

1. Kev puas tsuaj ntawm cov hlab ntsha hauv lub hlwb (CM):

Qhov no yog qhov uas 'port-wine stain' uas peb tau hais ua ntej tshwm sim. Qhov no yog tshwm sim los ntawm qhov o ntawm cov hlab ntsha hauv qab peb daim tawv nqaij. Cov cim yug no tuaj yeem yog ib qho ntawm thawj cov cim qhia tias koj muaj KTS. Cov pob no tuaj yeem muaj ntau yam xim, los ntawm xim liab dawb mus rau xim ntshav tsaus (xim liab caw). Thaum koj laus zuj zus, cov pob no tuaj yeem zoo li lub pob liab, sib dua, lossis tsaus dua.

2. Kev puas tsuaj ntawm cov leeg ntshav (VM):

Yuav luag txhua tus neeg uas muaj KTS muaj cov hlab ntsha tsis zoo. Cov no tuaj yeem cuam tshuam rau cov hlab ntsha uas nyob saum daim tawv nqaij, ua rau muaj cov hlab ntsha varicose hauv ob txhais ceg, tshwj xeeb tshaj yog hauv qhov chaw mos thiab sab nraub qaum. Lawv kuj tuaj yeem cuam tshuam rau cov hlab ntsha tob uas khiav tob hauv lub cev. Qhov no tuaj yeem ua rau muaj ntshav txhaws hauv cov hlab ntsha tob (Deep Vein Thrombosis - DVT) . DVT yog ib qho mob txaus ntshai.

3. Cov nqaij mos thiab pob txha loj hlob ntau dhau:

Qhov no yog ib qho mob uas, txij li thaum yau, ib sab caj npab lossis ceg feem ntau loj dua lwm sab. Feem ntau, qhov no tsuas cuam tshuam rau ib sab caj npab lossis ceg xwb, thiab feem ntau tsuas yog ib ceg xwb. Qee zaum ib sab ceg yuav ntev dua lwm sab. Qhov no tuaj yeem ua rau poob qhov sib npaug, nyuaj rau kev taug kev, thiab tsis tshua muaj peev xwm txav mus los.

4. Kev Ua Tsis Zoo ntawm Cov Lymphatic (LM):

Ib txhia neeg uas muaj KTS tej zaum yuav muaj cov hlab ntsha lymph ntxiv rau hauv lawv lub cev lymphatic, lossis cov hlab ntsha lymph uas twb muaj lawm tej zaum yuav tsis zoo li qub. Vim tias cov hlab ntsha lymphatic ntxiv no tsis ua haujlwm zoo, cov kua lymphatic tuaj yeem xau , ua rau o ntawm ob txhais ceg, tshwj xeeb tshaj yog ko taw, lossis muaj teeb meem hauv lub duav, lub zais zis, lossis cov hnyuv qis.

Ua cas thiaj ua rau KTS? Vim li cas qhov no thiaj tshwm sim?

Feem ntau, KTS yog tshwm sim los ntawm kev hloov pauv hauv cov noob hu ua PIK3CA . Qhov kev hloov pauv caj ces no tshwm sim qee zaus, txhais tau tias tsis muaj qhov ua rau paub. Nws tsis yog tau txais los ntawm niam txiv . Qhov no txhais tau tias txawm tias tsis muaj niam txiv twg muaj KTS, tus menyuam tuaj yeem muaj KTS vim yog qhov kev hloov pauv caj ces no.

Muaj ib txhia neeg muaj KTS yam tsis muaj qhov kev hloov pauv ntawm cov noob caj noob ces PIK3CA no. Yog li ntawd, cov kws tshawb fawb ntseeg tias KTS tej zaum yuav yog los ntawm kev hloov pauv ntawm ntau lwm cov noob caj noob ces. Kev tshawb fawb txog qhov no tseem tab tom ua mus.

Cov teeb meem uas yuav tshwm sim ntawm KTS yog dab tsi?

KTS muaj peev xwm ua rau muaj ntau yam teeb meem. Yog vim li ntawd kev kho mob sai sai yog qhov tseem ceeb. Cia peb saib seb cov teeb meem ntawd yog dab tsi:

• Cov ntshav txhaws: Muaj peev xwm tshwm sim, tshwj xeeb tshaj yog nyob rau hauv cov leeg ntshav tob (DVT).
• Cellulitis: Qhov no yog ib qho kab mob bacterial uas tshwm sim hauv qab daim tawv nqaij.
• Cov kua dej sib sau ua ke thiab o (Lymphedema): Ua rau muaj kev ua haujlwm tsis zoo ntawm lub cev lymphatic.
• Kev los ntshav sab hauv: Kev los ntshav tuaj yeem tshwm sim hauv txoj hnyuv plab (GI), lub zais zis, lossis lub cev poj niam.
• Pulmonary embolism: Qhov no yog ib qho mob uas ua rau tuag taus uas cov ntshav txhaws uas tsim nyob rau hauv cov leeg ntshav tob tawg thiab thaiv cov hlab ntsha hauv lub ntsws.

Tsis tshua muaj neeg mob KTS uas yug los muaj qhov tsis zoo ntawm lawv txhais tes lossis taw. Piv txwv li:

• Muaj cov ntiv tes ntxiv (Polydactyly)
• Cov ntiv tes lo ua ke (Syndactyly)

KTS puas ua rau mob?

Yog lawm, KTS tuaj yeem mob heev.

• Cov leeg ntshav varicose tuaj yeem ua rau khaus lossis mob.
• Teeb meem nrog kev ncig ntshav tuaj yeem ua rau o thiab mob, tshwj xeeb tshaj yog nyob rau hauv cov ceg qis.
• Kev loj hlob ntau dhau ntawm ib lub cev, xws li ib ceg, tuaj yeem ua rau koj mob thiab hnyav.

Cov kws kho mob kuaj mob KTS li cas? (Kev kuaj mob)

Cov kws kho mob xub kuaj mob KTS raws li cov cim qhia ntawm lub cev. KTS raug xav tias muaj teeb meem yog tias muaj teeb meem tsawg kawg yog ob ntawm peb qhov chaw tseem ceeb uas peb tau tham ua ntej - cov hlab ntsha, cov leeg ntshav, lossis kev loj hlob ntawm ceg. Vim tias ntau cov tsos mob ntawm KTS pom tau thaum yug los, nws yuav ua tau kom kuaj mob KTS ua ntej koj tus menyuam tawm hauv tsev kho mob.

Cov kev kuaj twg siv los kuaj mob KTS?

Nyob ntawm seb cov tsos mob zoo li cas, tus kws kho mob yuav ua cov kev kuaj mob xws li cov no kom paub meej ntxiv txog qhov mob thiab txiav txim siab qhov teeb meem loj npaum li cas:

• Cov duab CT scans lossis MRI scans: Saib seb cov nqaij mos thiab pob txha zoo li cas.
• Magnetic Resonance Angiogram (MR angiogram): Qhov no yog kev kuaj MRI tshwj xeeb uas tuaj yeem pom tseeb txog cov hlab ntsha thiab cov leeg ntshav.
• Doppler ultrasound: Qhov no pab pom cov ntshav ntws los ntawm cov leeg thiab cov hlab ntsha.

Cov kev kho mob rau KTS yog dab tsi?

Kev kho mob rau Klippel-Trenaunay Syndrome (KTS) sib txawv nyob ntawm cov tsos mob uas txhua tus neeg muaj.Tsis yog txhua tus neeg tau txais kev kho mob zoo ib yam. Lub hom phiaj tseem ceeb ntawm kev kho mob yog los tswj cov tsos mob thiab txo qhov kev pheej hmoo ntawm cov teeb meem. Cia peb saib seb cov txheej txheem kho mob tseem ceeb yog dab tsi:

• Cov tshuaj uas ua rau cov ntshav nyias / Cov tshuaj Anticoagulants: Piv txwv li, cov tshuaj xws li Heparin . Cov no txo ​​qhov kev pheej hmoo ntawm cov ntshav txhaws hauv ob txhais ceg (DVT) thiab pulmonary embolism.
• Sirolimus: Cov tshuaj no feem ntau yog siv los tiv thaiv lub cev kom tsis txhob tsis lees txais lub cev uas tau hloov lawm. Txawm li cas los xij, nws kuj tau pom tias yuav tiv thaiv cov hlab ntsha kom tsis txhob loj hlob tuaj.
• Cov thom khwm nruj: Cov no yog cov thom khwm tshwj xeeb uas pab cov ntshav ntws rov qab los ntawm ob txhais ceg mus rau lub plawv. Qhov no tuaj yeem pab txo qhov o, mob, thiab kev pheej hmoo ntawm cov ntshav txhaws.
• Kev phais mob kub ntawm lub qhov ntswg: Qhov no suav nrog kev siv cov zog uas tsom mus rau qhov chaw kom kaw cov hlab ntsha uas muaj teeb meem los ntawm sab hauv. Qhov no ua tiav thaum cov hlab ntsha tseem nyob ntawd. Qhov no ua rau lub sijhawm rov zoo tsawg dua thiab mob tsawg dua.
• Kev kho mob siv laser: Cov teeb zog muaj zog thiab tsom mus rau qhov chaw tuaj yeem siv los rhuav tshem lossis tshem tawm cov nqaij mos uas tsis xav tau. Kev kho mob siv laser no yog siv los ua kom cov xim ntawm lub cim yug 'port-wine stain' dawb dua.
• Kev kho mob Sclerotherapy: Hauv qhov no, ib qho tshuaj tshwj xeeb raug txhaj ncaj qha rau hauv cov leeg ntshav, cov hlab ntsha me me, lossis cov hlab ntsha lymphatic uas muaj teeb meem. Cov hlab ntsha ces kaw. Qhov no yog kev kho mob zoo heev rau cov leeg ntshav varicose.
• Kev Nqa Khau: Yog tias koj ob txhais ceg tsis ntev ib yam, koj tuaj yeem nqa siab dua rau ib lub khau kom kho qhov no. Qhov no kuj tseem tuaj yeem pab tiv thaiv scoliosis .
• Kev Phais: Kev phais yuav ua tau los kho cov teeb meem nrog cov leeg ntshav thiab kom sib npaug qhov ntev ntawm ob txhais ceg. Los yog, kev phais yuav ua tau los tshem tawm cov rog lossis cov ntaub so ntswg ntau dhau los txo qhov loj ntawm caj npab lossis ceg uas loj dhau. Tsis tshua muaj, yog tias tus ntiv taw loj dhau ua rau nws nyuaj rau hnav khau lossis taug kev, tus ntiv taw yuav raug phais tshem tawm.

Puas tiv thaiv tau KTS?

Tu siab kawg li, vim KTS tshwm sim yam tsis paub tias yuav tshwm sim li cas, tsis muaj ib txoj hauv kev los tiv thaiv nws kom tsis txhob loj hlob . Txawm li cas los xij, raws li tau hais ua ntej lawm, kev kho mob kom zoo tuaj yeem pab cov neeg uas muaj KTS kom muaj lub neej zoo dua.

Ib tug neeg uas muaj KTS yuav xav tau lub neej yav tom ntej zoo li cas?

Txawm hais tias tsis muaj tshuaj kho tau KTS los, cov tsos mob tuaj yeem tswj tau. Feem ntau, cov neeg uas muaj tus mob no nyob tau ib lub neej zoo li qub .

Txawm li cas los xij, qhov kev cia siab rau KTS yuav txawv ntawm ib tus neeg mus rau ib tus neeg. Nws nyob ntawm seb koj cov hlab ntsha puas loj npaum li cas. Cov no tuaj yeem hnyav zuj zus mus. Yog li ntawd, yog tias koj muaj kev los ntshav hauv plab (GI bleeding), cov hlab ntsha ntshav tob (deep vein thrombosis), lossis pulmonary embolism , koj yuav tsum nrhiav kev kho mob tam sim ntawd . Cov ntshav txhaws lossis los ntshav ntau dhau tuaj yeem ua rau tuag taus.

Kev qhia thiab kho mob tsis tu ncua tuaj yeem txo qhov kev pheej hmoo ntawm cov teeb meem.

Yog tias kuv muaj KTS, kuv yuav saib xyuas kuv tus kheej li cas? / Kuv yuav saib xyuas kuv tus menyuam li cas?

Yog koj lossis koj tus menyuam muaj KTS, ceev faj txog cov no:

• Saib xyuas koj daim tawv nqaij kom zoo: Nws yog ib qho tseem ceeb kom tiv thaiv kev kis kab mob.
• Feem ntau tsis pom zoo kom siv cov tshuaj hormones uas muaj estrogen: Lawv tuaj yeem ua rau muaj kev pheej hmoo ntawm cov ntshav txhaws. Tham nrog koj tus kws kho mob txog qhov no.
• Noj tshuaj kom tiv thaiv cov ntshav txhaws thaum cev xeeb tub: Koj tus kws kho mob yuav qhia koj txog qhov no.
• Noj cov tshuaj uas ua rau cov ntshav nyias ua ntej phais: Txo qhov kev pheej hmoo ntawm cov ntshav txhaws.

Kuv yuav tsum mus ntsib kws kho mob thaum twg?

Mus ntsib koj tus kws kho mob tsis tu ncua thoob plaws hauv koj lub neej kom tau txais kev kuaj mob . Qhov no yuav cia koj tus kws kho mob saib xyuas koj tus mob thiab kho txhua yam teeb meem tshiab uas yuav tshwm sim. Kev kuaj mob tsis tu ncua yuav pab koj tus kws kho mob pom tias koj txoj kev kho mob ua haujlwm zoo npaum li cas thiab hloov pauv koj txoj kev npaj kho mob yog tias tsim nyog.

Kuv yuav tsum mus rau Chav Kho Mob Thaum Muaj Xwm Ceev (ETU) thaum twg?

Yog koj xav tias koj muaj cov ntshav txhaws (piv txwv li, DVT, Cov tsos mob ntawm Pulmonary Embolism) lossis yog tias koj los ntshav ntau (los ntshav ntau), koj yuav tsum mus rau chav kho mob ceev tam sim ntawd.

Cov lus nug tseem ceeb uas yuav nug tus kws kho mob yog dab tsi?

Yog tias koj lossis koj tus menyuam muaj KTS, tej zaum koj yuav xav nug koj tus kws kho mob cov lus nug xws li:

• Cov kev kho mob twg zoo tshaj plaws rau kuv/kuv tus menyuam?
• Kuv yuav tsum tuaj kuaj mob ntau npaum li cas?
• Muab kuv qhov xwm txheej tam sim no, koj tuaj yeem hais li cas txog kuv lub neej yav tom ntej (kev kwv yees / kev cia siab)?

KTS puas muaj kev phom sij rau lub neej?

KTS nws tus kheej tsis cuam tshuam ncaj qha rau lub neej ntev. Txawm li cas los xij, qee qhov teeb meem uas tuaj yeem tshwm sim vim KTS, xws li kev los ntshav sab hauv lossis pulmonary embolism, tuaj yeem ua rau tuag taus . Kev kho mob tsis tu ncua rau cov yam txaus ntshai no tuaj yeem txo qhov kev pheej hmoo ntawm cov teeb meem.

KTS puas yog ib qho kev tsis taus?

Nws tuaj yeem tshwm sim. Yog tias koj ua tsis tau haujlwm vim muaj teeb meem ntawm KTS, xws li mob ntshav hauv cov hlab ntsha tob (DVT) lossis mob ntsws embolism, koj yuav tsim nyog tau txais cov txiaj ntsig kev tsis taus. Koj kuj tseem yuav tsim nyog tau txais cov txiaj ntsig xws li daim ntawv cim chaw nres tsheb rau cov teeb meem xws li kev nyuaj siab taug kev vim yog ceg loj.

Nws yuav nyuaj rau nkag siab txog txhua yam tsos mob thiab teeb meem ntawm KTS ib zaug. Txawm li cas los xij, koj tus kws kho mob tuaj yeem piav qhia rau koj txhua yam thiab pab koj kho koj cov teeb meem. Tsis txhob ntshai qhia koj tus kws kho mob txog koj txoj kev xav thiab cov tsos mob tshiab uas koj tab tom ntsib . Kev tham qhib siab li no yuav ua rau nws yooj yim dua los thov kev pab yog tias koj muaj teeb meem.

Peb xav coj dab tsi los ntawm zaj dab neeg no (Cov Lus Qhia Txog Kev Coj Mus Tsev)

Zoo, yog li peb tau tham ntau txog KTS, puas yog? Nov yog ob peb yam uas yuav tsum nco ntsoov:

• KTS yog ib yam mob uas tsis tshua muaj, uas muaj txij thaum yug los . Yog li ntawd, tsis txhob txhawj, koj tsis yog tib leeg xwb.
• Cov tsos mob tseem ceeb yog lub cim yug 'port-wine stain', caj npab/ceg loj, thiab teeb meem ntawm cov leeg ntshav .
• Txawm hais tias tsis muaj kev kho mob tag nrho rau qhov no los xij, muaj kev kho mob zoo heev los tswj cov tsos mob .
• Nws yog ib qho tseem ceeb heev uas yuav tsum kuaj mob thiab pib kho sai li sai tau .
• Nws yog ib qho tseem ceeb uas yuav tsum tau txais kev saib xyuas kho mob tas li thiab kev kho mob tsim nyog thoob plaws lub neej.
• Sib txuas lus zoo nrog koj tus kws kho mob. Nug txhua yam lus nug uas koj muaj, thiab qhia rau lawv paub koj xav li cas.

Vam tias cov ntaub ntawv no tau pab koj nkag siab zoo dua txog KTS. Yog tias koj lossis ib tus neeg koj paub muaj cov tsos mob no, nws yog qhov zoo tshaj plaws los nrhiav kev pab kho mob.

Klippel -Trenaunay Syndrome, KTS, xim av port-wine, cim yug los, cov hlab ntsha tsis zoo, lub cev lymphatic, ceg loj hlob ntau dhau, teeb meem txij thaum yug los, cim yug los liab, cov hlab ntsha tsis zoo, lub cev lymphatic, ceg loj hlob ntau dhau, kab mob caj ces, CLVM