Kodi khungu la mwana wanu ndi lofiira komanso lotupa? Tiyeni tiphunzire za matenda a Klippel-Trenaunay Syndrome (KTS)!

Kodi munayamba mwaonapo kuti ana ena amabadwa ndi chizindikiro chofiira chachikulu mbali imodzi ya thupi lawo, makamaka pa mkono kapena mwendo, ndipo mkono/mwendowo ndi waukulu pang'ono kuposa mbali inayo? Kapena nthawi zina mumatha kuona mitsempha mbali imeneyo? Ndizachilendo kumva mantha pang'ono mukaona chinthu chonga ichi. Lero tikambirana za vuto lomwe limasonyeza zizindikiro zofanana, zomwe sizimachitika kawirikawiri, kutanthauza kuti si aliyense amene amaliyambitsa, ndipo amabadwa nalo. Izi zimatchedwa Klippel-Trenaunay Syndrome (KTS) . Musadandaule, tikambirana izi mosavuta, m'njira yomwe mungamvetse.

Kodi Klippel-Trenaunay Syndrome (KTS) ndi chiyani? Mwachidule...

Tangoganizirani, KTS ndi vuto losowa lomwe limabwera ndi mwana wathu akabwera m'dziko lino (ndiko kuti, vuto lobadwa nalo ). Izi zimayambitsa kusintha pang'ono momwe minofu yofewa ya mwana, mafupa, ndi mitsempha yamagazi imakulira. Chimodzi mwa zizindikiro zazikulu za izi ndikuwoneka kwa chizindikiro chofiira chofiirira, nthawi zambiri pamanja kapena miyendo, chotchedwa 'port-wine stain' . Nthawi zambiri, anthu omwe ali ndi KTS iyi amakhala ndi mavuto ena ndi dongosolo lawo la lymphatic . Dongosolo la lymphatic ili ndi dongosolo lofunikira lomwe limathandiza kuti madzi amthupi lathu azikhala bwino.

Pakadali pano palibe mankhwala a KTS. Komabe, musachite mantha. Pali njira zambiri zochizira zizindikiro . Ngati vutoli lapezeka ndi kuchiritsidwa msanga, monga mwana akabadwa, mavuto azaumoyo omwe angabwere chifukwa cha KTS angachepe kwambiri.

Madokotala ena amatchanso vuto la KTS CLVM . Ili ndi chilembo choyamba cha mawu anayi a Chingerezi.

• C ndi ya ma Capillaries - awa ndi mitsempha yaying'ono yamagazi yomwe imalumikiza mitsempha yathu ndi mitsempha yathu.
• L imayimira dongosolo la Lymphatic - Ichi ndi gawo la chitetezo chathu chamthupi. Chimanyamula madzi otchedwa lymph m'thupi lonse.
• V imayimira Mitsempha - iyi ndi mitsempha yamagazi yomwe imanyamula magazi kupita kumtima kwathu.
• Chilembo M chikuyimira Kusakhazikika . Zimatanthauza kuti gawo lina la thupi silikukula bwino, kapena kuti pali kupunduka.

KTS idatchulidwa dzina lake potengera madokotala awiri aku France, Maurice Klippel ndi Paul Trenaunay, omwe adapeza vutoli mu 1900. Akatswiri akuti pafupifupi munthu m'modzi pa anthu 100,000 padziko lonse lapansi ali ndi vutoli. Lingakhudze aliyense, mosasamala kanthu za fuko kapena jenda.

Kodi zizindikiro za KTS ndi ziti? Kodi mumazindikira bwanji?

Zizindikiro za Klippel-Trenaunay Syndrome (KTS) zimakhudza kwambiri mitsempha, mitsempha yamagazi, minofu yofewa, mafupa, ndi mitsempha yamagazi m'thupi lathu. Tiyeni tiwone momwe izi zingachitikire:

1. Kusokonekera kwa Mitsempha ya Capillary (CM):

Apa ndi pomwe 'dothi la vinyo wa port-wine' lomwe tatchula kale limapezeka. Izi zimachitika chifukwa cha kutupa kwa mitsempha yamagazi yomwe ili pansi pa khungu lathu. Zizindikiro zobadwa nazo izi zitha kukhala chimodzi mwa zizindikiro zoyambirira zakuti mungakhale ndi KTS. Madontho awa akhoza kukhala ndi mitundu yosiyanasiyana, kuyambira pinki yopepuka mpaka yofiirira yakuda (vinyo wofiira). Mukakula, madontho awa amatha kukhala ngati matuza, opepuka, kapena akuda.

2. Kusokonekera kwa Mitsempha (VM):

Pafupifupi aliyense amene ali ndi KTS ali ndi vuto la mitsempha yamagazi. Izi zimatha kukhudza mitsempha yomwe ili pamwamba pa khungu, zomwe zimayambitsa mitsempha yotupa m'miyendo, makamaka m'mimba ndi m'ntchafu. Zingathenso kukhudza mitsempha yakuya yomwe imayenda mkati mwa thupi. Izi zitha kupangitsa kuti magazi aziundana m'mitsempha yakuya (Deep Vein Thrombosis - DVT) . DVT ndi vuto loopsa.

3. Minofu yofewa ndi mafupa zimakula kwambiri:

Imeneyi ndi vuto lomwe, kuyambira ali aang'ono, mkono kapena mwendo umodzi nthawi zambiri umakula kuposa wina. Nthawi zambiri, izi zimakhudza mkono kapena mwendo umodzi wokha, ndipo nthawi zambiri mwendo umodzi wokha. Nthawi zina mwendo umodzi ungakhale wautali kuposa wina. Izi zingayambitse kutayika kwa mphamvu, kuvutika kuyenda, komanso kuyenda pang'ono.

4. Kusokonekera kwa Mitsempha (LM):

Anthu ena omwe ali ndi KTS akhoza kukhala ndi mitsempha yowonjezera ya lymph mu dongosolo lawo la lymphatic, kapena mitsempha ya lymph yomwe ilipo ikhoza kukhala ndi mawonekedwe osazolowereka. Chifukwa mitsempha yowonjezera iyi ya lymph sigwira ntchito bwino, madzi a lymphatic amatha kutuluka , zomwe zimayambitsa kutupa m'miyendo, makamaka mapazi, kapena mavuto m'chiuno, chikhodzodzo, kapena m'matumbo apansi.

Kodi n’chiyani chimayambitsa KTS? N’chifukwa chiyani izi zimachitika?

Nthawi zambiri, KTS imayamba chifukwa cha kusintha kwa majini otchedwa PIK3CA . Kusintha kwa majini kumeneku kumachitika pang'onopang'ono, zomwe zikutanthauza kuti palibe chifukwa chodziwika. Sikochokera kwa makolo . Izi zikutanthauza kuti ngakhale makolo onse awiri anali ndi KTS, mwana akhoza kukhala ndi KTS chifukwa cha kusintha kwa majini kumeneku.

Anthu ena ali ndi KTS popanda kusintha kwa majini a PIK3CA. Chifukwa chake, ofufuza amakhulupirira kuti KTS ikhoza kukhala chifukwa cha kusintha kwa majini ena angapo. Kafukufuku pa izi akupitirirabe.

Kodi mavuto omwe angakhalepo chifukwa cha KTS ndi ati?

KTS ingayambitse mavuto osiyanasiyana. Ichi ndichifukwa chake chithandizo chachangu n'chofunika. Tiyeni tiwone mavuto amenewo:

• Magazi amaundana: Amatha kuchitika, makamaka m'mitsempha yakuya (DVT).
• Cellulitis: Iyi ndi matenda a bakiteriya omwe amapezeka pansi pa khungu.
• Kuchulukana kwa madzi ndi kutupa (Lymphedema): Kumachitika chifukwa cha kusokonekera kwa ntchito ya lymphatic system.
• Kutuluka magazi mkati: Kutuluka magazi m'mimba (GI), m'chikhodzodzo, kapena m'njira yoberekera ya akazi.
• Kutsekeka kwa magazi m'mapapo: Uwu ndi matenda omwe angawononge moyo pomwe magazi oundana omwe amapangika m'mitsempha yakuya amasweka ndikutseka mtsempha wamagazi m'mapapo.

Kawirikawiri, anthu omwe ali ndi KTS amakhala ndi zilema zobadwa nazo m'manja kapena m'mapazi. Mwachitsanzo:

• Kukhala ndi zala zina (Polydactyly)
• Zala zolumikizidwa pamodzi (Syndactyly)

Kodi KTS imayambitsa ululu?

Inde, KTS ikhoza kukhala yopweteka.

• Mitsempha ya varicose ingayambitse kuyabwa kapena kupweteka.
• Mavuto okhudza kuyenda kwa magazi angayambitse kutupa ndi kupweteka, makamaka m'miyendo ya m'munsi.
• Kukula kwambiri kwa chiwalo, monga mwendo, kungayambitse kumva kulemera ndi kupweteka mwendowo.

Kodi madokotala amazindikira bwanji matenda a KTS? (Kuzindikira)

Madokotala amayamba kuzindikira KTS potengera zizindikiro zakuthupi. KTS imaganiziridwa ngati pali mavuto m'malo awiri mwa atatu akuluakulu omwe takambirana kale - mitsempha yamagazi, mitsempha, kapena kukula kwa miyendo. Popeza zizindikiro zambiri za KTS zimawonekera pobadwa, n'zotheka kuzindikira KTS mwana wanu asanatuluke kuchipatala.

Ndi mayeso ati omwe amagwiritsidwa ntchito pozindikira KTS?

Kutengera ndi zizindikiro, dokotala angachite mayeso ngati awa kuti atsimikizire vutoli ndikupeza kukula kwa vutoli:

• Kujambula kwa CT kapena MRI: Kuwona momwe minofu ndi mafupa ofewa alili.
• Magnetic Resonance Angiogram (MR angiogram): Iyi ndi njira yapadera ya MRI yomwe imatha kuona bwino momwe mitsempha yamagazi ndi mitsempha ilili.
• Doppler ultrasound: Izi zimathandiza kuona kuyenda kwa magazi kudzera m'mitsempha ndi m'mitsempha.

Kodi mankhwala a KTS ndi otani?

Chithandizo cha Klippel-Trenaunay Syndrome (KTS) chimasiyana malinga ndi zizindikiro zomwe munthu aliyense ali nazo.Si aliyense amene amapatsidwa chithandizo chofanana. Cholinga chachikulu cha chithandizo ndikuwongolera zizindikiro ndikuchepetsa chiopsezo cha zovuta. Tiyeni tiwone njira zazikulu zochizira:

• Mankhwala ochepetsa magazi / Mankhwala oletsa magazi kuundana: Mwachitsanzo, mankhwala monga Heparin . Izi zimachepetsa chiopsezo cha magazi kuundana m'miyendo (DVT) ndi pulmonary embolism.
• Sirolimus: Mankhwalawa nthawi zambiri amagwiritsidwa ntchito poletsa thupi kukana chiwalo chomwe chaikidwa m'thupi. Komabe, zapezekanso kuti amaletsa zolakwika za mitsempha yamagazi kuti zisakule kwambiri.
• Masokisi opondereza: Awa ndi mitundu yapadera ya masokosi omwe amathandiza magazi kubwerera kuchokera ku miyendo kupita kumtima. Izi zingathandize kuchepetsa kutupa, kupweteka, komanso chiopsezo cha magazi kuundana.
• Kuchotsa kutentha kwa thupi: Izi zimaphatikizapo kugwiritsa ntchito mphamvu zokhazikika kuti zitseke mitsempha yamagazi yomwe ili mkati. Izi zimachitika mitsempha ikadalipo. Izi zimapangitsa kuti munthu asamavutike kwambiri komanso kuti ululu ukhale wochepa.
• Chithandizo cha laser: Mphamvu yolunjika komanso yolimba ingagwiritsidwe ntchito kuwononga kapena kuchotsa minofu yosafunikira. Chithandizo cha laser ichi chimagwiritsidwa ntchito kuwunikira mtundu wa chizindikiro chobadwa nacho cha 'port-wine stain'.
• Sclerotherapy: Mu izi, njira yapadera imabayidwa mwachindunji m'mitsempha yovuta, m'mitsempha yamagazi, kapena m'mitsempha ya lymphatic. Kenako mitsempha imatseka. Iyi ndi njira yothandiza kwambiri yothandizira mitsempha ya varicose.
• Kukweza nsapato: Ngati miyendo yanu si yofanana kutalika, mutha kuyika kukweza kwakukulu pa nsapato imodzi kuti mukonze izi. Izi zingathandizenso kupewa scoliosis .
• Opaleshoni: Opaleshoni ingachitike kuti akonze mavuto a mitsempha ndi kulinganiza kutalika kwa miyendo. Kapena, opaleshoni ingachitike kuti achotse mafuta ochulukirapo kapena minofu kuti achepetse kukula kwa mkono kapena mwendo wokulirapo. Nthawi zambiri, ngati chala chachikulu kwambiri chimapangitsa kuti zikhale zovuta kuvala nsapato kapena kuyenda, chalacho chingachotsedwe mwa opaleshoni.

Kodi KTS ingapewedwe?

Mwatsoka, chifukwa chakuti KTS imachitika mwachisawawa, palibe njira yoletsera kuti isakule . Komabe, monga tanenera kale, chithandizo choyenera chingathandize anthu omwe ali ndi KTS kukhala ndi moyo wabwino kwambiri.

Kodi munthu amene ali ndi matenda a KTS angayembekezere tsogolo lotani?

Ngakhale kuti palibe mankhwala a KTS, zizindikiro zake zimatha kuchiritsidwa. Nthawi zambiri, anthu omwe ali ndi vutoli amakhala ndi moyo wautali .

Komabe, chiyembekezo cha KTS chingasiyane malinga ndi munthu ndi munthu. Zimatengera momwe matenda anu a mitsempha yamagazi alili oopsa. Izi zitha kuipiraipira pakapita nthawi. Chifukwa chake, ngati mukuyamba kutuluka magazi m'mimba, kutsekeka kwa mitsempha yamagazi, kapena pulmonary embolism , muyenera kupita kuchipatala nthawi yomweyo . Kutsekeka kwa magazi kapena kutuluka magazi kwambiri kumatha kupha.

Uphungu ndi chithandizo chamankhwala nthawi zonse zingathandize kuchepetsa kwambiri chiopsezo cha mavuto.

Ngati ndili ndi matenda a KTS, ndimadzisamalira bwanji? / Kodi ndimasamalira bwanji mwana wanga?

Ngati inu kapena mwana wanu ali ndi matenda a shuga (KTS), dziwani izi:

• Samalirani bwino khungu lanu: Ndikofunikira kupewa matenda.
• Njira zolerera za mahomoni zomwe zili ndi estrogen nthawi zambiri sizikulimbikitsidwa: Zitha kuwonjezera chiopsezo cha magazi kuundana. Lankhulani ndi dokotala wanu za izi.
• Imwani mankhwala oletsa magazi kuundana panthawi ya mimba: Dokotala wanu adzakulangizani pa izi.
• Imwani mankhwala ochepetsa magazi musanachite opaleshoni: Chepetsani chiopsezo cha magazi kuundana.

Ndiyenera kupita kwa dokotala liti?

Pitani kwa dokotala nthawi ndi nthawi pa moyo wanu wonse kuti mukaone ngati muli ndi matenda . Izi zithandiza dokotala wanu kuyang'anira matenda anu ndikuchiza mavuto atsopano omwe angabuke. Kuyang'aniridwa nthawi zonse kudzathandiza dokotala wanu kuwona momwe chithandizo chanu chikugwirira ntchito ndikusintha dongosolo lanu la chithandizo ngati pakufunika kutero.

Kodi ndiyenera kupita liti ku Emergency Treatment Unit (ETU) ?

Ngati mukuganiza kuti muli ndi magazi oundana (monga DVT, zizindikiro za Pulmonary Embolism) kapena ngati mukutuluka magazi ambiri (kutuluka magazi ambiri), muyenera kupita kuchipatala mwamsanga.

Kodi ndi mafunso otani ofunika kufunsa dokotala?

Ngati inu kapena mwana wanu ali ndi KTS, mungafune kufunsa dokotala wanu mafunso monga:

• Ndi mankhwala ati omwe ndi abwino kwa ine/mwana wanga?
• Kodi ndiyenera kubwera kangati kudzayezetsa?
• Poganizira momwe zinthu zilili panopa, munganene chiyani za tsogolo langa (kuneneratu zamtsogolo/malingaliro anga)?

Kodi KTS ndi yoopsa?

KTS yokha siikhudza mwachindunji nthawi yomwe munthu amakhala ndi moyo. Komabe, mavuto ena omwe angachitike chifukwa cha KTS, monga kutuluka magazi mkati kapena pulmonary embolism, akhoza kukhala pachiwopsezo cha moyo . Kuchiza nthawi zonse zinthu zoopsazi kungachepetse chiopsezo cha mavuto.

Kodi KTS ndi chilema?

Zitha kuchitika. Ngati simungathe kugwira ntchito chifukwa cha zovuta za KTS, monga deep vein thrombosis (DVT) kapena pulmonary embolism, mutha kulandira maubwino olemala. Muthanso kulandira maubwino monga malo oimika magalimoto olemala chifukwa cha mavuto monga kuyenda movutikira chifukwa cha kukula kwa miyendo.

Zingakhale zovuta kumvetsetsa zizindikiro zonse ndi mavuto a KTS nthawi imodzi. Komabe, dokotala wanu akhoza kukufotokozerani zonsezo ndikukuthandizani kuchiza mavuto anu. Musaope kuuza dokotala wanu za momwe mukumvera komanso zizindikiro zatsopano zomwe mukukumana nazo . Kulankhula momasuka motere kudzakuthandizani kupempha thandizo ngati muli ndi vuto lililonse.

Zimene tikufuna kutenga kuchokera mu nkhaniyi (Uthenga Wopita Nawo Kunyumba)

Chabwino, takambirana zambiri za KTS, sichoncho? Nazi zinthu zingapo zomwe muyenera kukumbukira:

• Matenda a KTS ndi matenda obadwa nawo osowa kwambiri . Choncho musadandaule, simuli nokha.
• Zizindikiro zazikulu ndi chizindikiro cha kubadwa kwa 'port-wine stain', kukula kwa mkono/mwendo, ndi mavuto a mitsempha .
• Ngakhale kuti palibe mankhwala athunthu a izi, pali mankhwala abwino kwambiri oletsa zizindikiro .
• Ndikofunikira kwambiri kuzindikira matendawa ndikuyamba kulandira chithandizo mwachangu .
• Ndikofunikira kulandira chithandizo chamankhwala nthawi zonse komanso kulandira chithandizo chofunikira pa moyo wanu wonse.
• Pitirizani kulankhulana bwino ndi dokotala wanu. Funsani mafunso aliwonse omwe muli nawo, ndipo muuzeni momwe mukumvera.

Ndikukhulupirira kuti izi zakuthandizani kumvetsetsa bwino za KTS. Ngati inu kapena munthu amene mumamudziwa ali ndi zizindikirozi, ndi bwino kufunsa upangiri kwa dokotala.

Matenda a Klippel -Trenaunay, KTS, chizindikiro cha port-wine, chizindikiro cha kubadwa, vuto la mitsempha yamagazi, dongosolo la lymphatic, kukula kwa miyendo, vuto lobadwa nalo, chizindikiro chofiira cha kubadwa, vuto la mitsempha yamagazi, dongosolo la lymphatic, kukula kwa miyendo, matenda a majini, CLVM