Ndi matenda a Klippel-Trenaunay-Weber chiyani?

Monga mukudziwa, vuto lobadwa ali kudutsa chibadwa kwa makolo kapena uka kwa kuphwanya embryogenesis. Anomalies ikhoza kupangidwa kwa minofu kapena chiwalo. Nthawi zambiri amapezeka nthawi yomweyo pambuyo pa kubadwa, koma nthawi zina zimapezeka patapita nthawi yaitali. Chitsanzo ndi kobadwa nako anomaly syndrome, Klippel-Trenaunay-Weber. Matendawa amatanthauza kudwala a dongosolo mtima, sukhala makamaka m'munsi nthambi mtsempha.

Mosiyana ndi zina zambiri anomalies kobadwa nako, Klippel-Trenaunay-Weber syndrome mwina ndi madokotala ananena zawo yabwino kwa moyo. Iye zimatheka ndi yake chithandizo opaleshoni ya matenda.

Kudwala: syndrome, Klippel-Trenaunay-Weber - ndichiyani?

matenda akhala kuyambira m'zaka zoyambirira za m'ma 20, pamene woyamba kufotokoza asayansi awiri - ndipo Klippel Trenaunay. Kenako Dr. Weber anawonjezera kale zambiri za matenda amenewa. Amadziwika kuti matenda ali dzina lina - osteogipertrofichesky venous nevus.

Syndrome Klippel-Trenaunay-Weber anapeza makamaka pakati anthu wamwamuna. Pa nthawi, nkhani chimayambitsa matenda ndi EPIDEMIOLOGY ake sikokwanira. Zizindikiro zazikulu za kudwala ndi yaikulu "timadontho-timadontho" (nevi) pa miyendo, osiyana awiri m'munsi nthambi varicosity. Ngakhale woyambirira wa zizindikiro matenda ndi zovuta zotupa, zina, matenda ndi mankhwala. Ubwino Izi zimatheka chifukwa cha kuonekera latsopano m'munda wa njira mtima opaleshoni.

Zimayambitsa vuto

Popeza matenda amanenedwa ndi Posachedwapa (pafupifupi zaka 100 zapitazo), pakadali pano palibe zolondola zokhudza etiology ake. Komabe, pali maganizo ambiri, chimene osauka syndrome Klippel-Trenaunay-Weber. The zimayambitsa matenda kugwirizana ndi mbiri banja. Iwo ankadziwa kuti waukulu etiological Chinthu ndi kusintha jini. Malinga ndi chiphunzitso china, chifukwa cha matenda lagona kuphwanya embryogenesis mimba msanga. Mchikakamizo cha zinthu zachilengedwe (mankhwala poizoni, psycho-maganizo nkhawa, kukhudzana ndi cheza) limapezeka aplasia dongosolo venous. underdevelopment ake unakambidwanso mogwirizana ndi wothandizila opatsirana. Malinga ndi mfundo imeneyi, chifukwa cha matenda ndi mabakiteriya kapena mavairasi otsekeredwa mu thupi la mayi ali woyembekezera.

The matenda chithunzi cha matenda

Mawonetseredwe loyamba la syndrome Klippel-Trenaunay-Weber kale noticeable mu nthawi neonatal. Nthawi zina, iwo amaoneka kenako - paubwana. Classic zizindikiro za matenda ndi zizindikiro zotsatirazi:

1. Angioma - kangaude mawanga. Nthawi zambiri, zilipo pa mwendo umodzi ndi kuphimba ndi lalikulu. mtundu Angiomas zingasiyane kuwala mdima wofiirira. Mchikakamizo cha zinthu thupi (mikangano, zimakhudza) khungu mu "birthmarks" a mosavuta kuonongeka, pali magazi.
2. mitsempha Varicose wa miyendo ya pamwamba. Matenda mawonetseredwe izi khalidwe - tortuosity, thickening, kuchuluka odwala ndi zotengera za nthambi m'munsi.
3. Hypertrophy wa mwendo akhudzidwa. Chifukwa zoipa za mitsempha kwambiri ndi kuchuluka nthambi ukuwonjezeka awo awiri. Nthawi zina, njira za mafupa ndi dongosolo ali. Kenako mwendo akhudzidwa mwina kupitilira nthambi wathanzi.

Nthawi zina munthu wa zizindikilo za matenda si anadzionetsera kapena kunja (mwachitsanzo, varicose zachiphamaso mitsempha). Mu zina, kudwala amakula mwamsanga, ndi kugonjetsedwa kwa anapeza kuchokera kumalekezero kwa thunthu ndi thupi chapamwamba.

diagnostics

Akuganiza Klippel-Trenaunay-Weber syndrome n'zotheka ndi Angioma kwambiri. chizindikiro Zikuoneka choyamba. Kenako anagwirizana ndi mitsempha varicose ndi hypertrophy ya nthambi. Mbali khalidwe la matenda - mmodzi ndiye amaganiza kugonja. Ngati mukukayikira matenda ikuchitika zasayansi ndi diagnostics malangizo. Choyamba anachita ultrasound ndi Doppler ultrasound wa miyendo m'munsi. Nkhani ina syndrome ichi ndi kupanda malumikizanidwe pakati kuthamanga venous ndi ntchito mtima. Mkhalidwe umenewu umaonekera mwa mzere wolunjika pa venography.

Trenaunay Syndrome Klippel-Weber: zochizira matenda

Kucotsa matenda chimadalira pa mlingo chiwonongeko cha mtsempha kwambiri. Ndi kuchepetsa kwambiri a kulambalala imagwiridwa. Ngati waukulu Vienna nakantha lalikulu, chotengera anapanga kupanga zinthu kupatsidwa zina. Monga mankhwala zowonjezera ntchito sclerotherapy (mankhwala "Trombovar", "Wayne Fibro"), atavala kabudula psinjika. Komanso amafuna phwando nthawi zonse anticoagulants (mankhwala "Warfarin"). Nthawi zina, opaleshoni mtima angathe kuchitidwa ndi laser a.