He aha te mea te syndrome o Klippel-Trenaunay-Weber?

Ka rite ki matau koutou, e haere hē whanau i runga i ira i ngā mātua ranei ara ake i te ofatiraa o te embryogenesis. Ka taea te hanga kōhikohiko i tetahi kiko okana ranei. Nuinga o ngā wā e kitea e ratou tonu i muri i whanau, engari i ētahi wā faaite mai ratou nui i muri. He tauira ko te Pākatokato congenital syndrome, Klippel-Trenaunay-Weber. E pā ana te mate ki te māuiuitanga o te pūnaha iaia, pānga reira te nuinga te rua e alangá raro.

Roto i te rerekē ki te maha atu kōhikohiko congenital, kia whai Klippel-Trenaunay-Weber syndrome he waitohunga pai mō te ora. Kei te tutuki ia e wā maimoatanga hāparapara o te mate.

Māuiuitanga: syndrome, Klippel-Trenaunay-Weber - he aha te mea te reira?

Kua te mate kua mohiotia mai te tīmatanga o te rau tau 20, ka i whakaahuatia tuatahi reira e rua pūtaiao - ko Klippel Trenaunay. I muri mai, tāpiri Dr. Weber kē i ngā mōhiohio e pā ana ki tenei mate. Kei te mohiotia te reira e kua ingoa tetahi te mate - osteogipertrofichesky nevus venous.

Syndrome Klippel-Trenaunay-Weber kitea te nuinga i roto i te taupori tane. I te kau, e kore he nui ngā kōrero e pā ana ki te take pakiaka o te mate, me tona mātai tahumaero. He whānui nga tohu matua o te māuiuitanga "kiore" (nevi) i runga i nga waewae, rerekē diameter raro e alangá varicosity. Ahakoa te whanaketanga wawe o tohu haumanu me te pakeke o pāpaka, i roto i ētahi wā, he treatable te syndrome. tutuki tēnei painga te whakawhetai ki te putanga o hou i roto i te mara o te tikanga tafa iaia.

Ko te take o te raruraru

Mai i whakaahuatia te mate ngā tata (e pā ana ki 100 nga tau ki muri), i reira he wā kahore mōhiohio pono e pā ana ki tona etiology. Otiia, i reira e rave rahi ariā, kia rite ki nei te whakawhanake i Klippel-Trenaunay-Weber syndrome. Ko te take o te mate e pā ana ki te aamu utuafare. Kei te matau te reira e te take etiological matua ko te irakētanga ira. E ai ki tetahi atu ariā, te take o te mate e takoto i roto i te ofatiraa o te embryogenesis roto i te hapū wawe. I raro i te mana o ngā āhuatanga taiao (poisoning matū, te ahotea psycho-aronganui, te rongo ki te radiation) puta pūnaha venous aplasia. Kei te whai pānga hoki tōna underdevelopment ki āpiha hopuhopu. E ai ki tenei ariā, te take o te mate, ko te huakita ranei huaketo mau i roto i te kopu o te whaea i roto i te hapū.

Ko te pikitia haumanu o te mate

Ko te mau faaiteraa tuatahi o te syndrome Klippel-Trenaunay-Weber kua kitea i roto i te wā fā'ele'i. I roto i te wā onge, puta muri mai ratou - i roto i te tamarikitanga. tohu Classic o te mate ko te tohu e whai ake nei:

1. wahi pūngāwerewere - Angioma. Nuinga o ngā wā, e wātea ana i runga i tetahi waewae ratou, ka hipoki ai i te wāhi nui. kia rerekē Angiomas tae i te marama ki te pouri papura. I raro i te mana o ngā āhuatanga tinana (waku, pānga) kiri i roto i te "birthmarks" o ngāwari pakaru, i reira te toto.
2. uaua tīponapona o nga waewae o te mata. faaiteraa haumanu o tenei huru - tortuosity, thickening, nui haere atu tahumaero me oko o te e alangá raro.
3. Hypertrophy o te waewae pāngia. Nā ki te wharanga o uaua hohonu, me o ratou piki e alangá mo tini i roto i te whānui. I roto i ētahi wā, ko i runga i te tukanga o te wheua, me te pūnaha. Na kia roa atu i te e alangá hauora te waewae kua pā.

I te tahi taime e kore e tetahi o nga huru o te mate whakakitea ranei waho (hei tauira, uaua ausia tīponapona). I roto i ētahi wā, kauneke tere te māuiuitanga, me te hinga o puta i te topito ki te kātua me te tinana o runga.

tātaringa

Ko taea parenga syndrome Klippel-Trenaunay-Weber i te Angioma whānui. puta ana tēnei tohu tuatahi. I muri mai piri e uaua tīponapona me hypertrophy o te e alangá. Ko te āhuatanga āhuatanga o tenei mate - hinga-iu kotahi. Ki te whakapae koe he mate kawea i roto i taiwhanga me tātaritanga mauhaa. Tuatahi whakamana ultrasound me Doppler ultrasound o nga wahi o raro. He āhuatanga o tenei syndrome ko te kore o te hononga ki waenganui i te pēhanga venous me te mahi cardiac. Ka kitea tēnei āhuatanga e te raina tika i roto i venography.

Trenaunay Syndrome Klippel-Weber: te maimoatanga o mate

Whakakorenga o te mate tei runga i te tohu o te whakangaromanga o te rua hohonu. Ki te whāiti nui o te karo whakamana te. Ki te patua e te matua Vienna he wāhi nui, i hanga te oko transplantation rauemi waihanga. Ka rite ki te maimoatanga atu tono sclerotherapy (taero "Trombovar", "Wayne Fibro"), mau kōpeketanga underwear. titau hoki te fariiraa tonu o anticoagulants (rongoā "fafati"). I roto i ētahi wā, ka taea te whakamana pokanga iaia ki te taiaho.