Chii urwere pakati Klippel-Trenaunay-Weber?

Sezvaunoziva, pakupona vari kuvabereki kubva vabereki kana muka kubva kutyora embryogenesis. Anomalies anogona yakawanikwa kubva chero zvinyama kana nenyere. Kakawanda zvinowanika pakarepo pashure pokuberekwa, asi dzimwe nguva anozviratidza zvikuru gare gare. Muenzaniso vachiberekwa anomaly chirwere, Klippel-Trenaunay-Weber. The chirwere zvinoreva zvokurwara pakati vascular ezvinhu, zvinokanganisawo kazhinji rezasi richiripo tsinga.

Kusiyana nevamwe vakawanda vachiberekwa anomalies, Klippel-Trenaunay-Weber chirwere angava yakanaka fungidziro upenyu. Iye zvinoitwa nenguva kuvhiya pakurapa chirwere.

Zvokurwara: urwere, Klippel-Trenaunay-Weber - chii?

The chirwere rave richizivikanwa kubvira pakutanga kwezana remakore rechi20, apo pakutanga kurondedzerwa nemasayendisiti maviri - uye Klippel Trenaunay. Gare gare, Dr. Weber akawedzera kare aiva mashoko nezvechirwere ichi. Zvinozivikanwa kuti chirwere ane rimwe zita - osteogipertrofichesky venous nevus.

Syndrome Klippel-Trenaunay-Weber akawana kunyanya pakati hono vanogara. Panguva iyoyo, mashoko pamusoro chinokonzera chirwere wayo epidemiology hakuna kukwana. The chikuru Zviratidzo zviri zvokurwara vari zvikuru "moles" (nevi) pamusoro makumbo, yakasiyana dhayamita ezasi richiripo varicosity. Pasinei mangwanani kukura kunotoda kurapwa zviratidzo nekuomarara maronda, mune zvimwe zviitiko, chirwere iri rinorapika. mukana Izvi zvinoitwa nemhaka chidzoke idzva mumunda vascular kuvhiya nzira.

Zvinokonzera dambudziko

Sezvo chirwere akarondedzerwa tichishandisa yapfuura (anenge 100 apfuura), pari zvino hakuna ruzivo yakavimbika pamusoro wayo etiology. Kunyange zvakadaro, pane zvakawanda pfungwa, maererano iyo dzichiri Klippel-Trenaunay-Weber syndrome. The zvinokonzera chirwere chokuita nenhoroondo mhuri. Zvinonzi kunzwisisa kuti huru etiological chinokonzera ndiko Gene mutation. Maererano neimwe pfungwa, mhaka chirwere iri kutyora embryogenesis mukutanga pamuviri. Nokupesvedzera kwezvakatipoteredza zvinhu (namakemikari chepfu, psycho-mupfungwa, kugara nemwaranzi) rinowanika aplasia venous hurongwa. underdevelopment Its kunewo chokuita noutachiona nevamiririri. Maererano nepfungwa iyi, chinokonzera chirwere utachiona kana mavhairasi inobatwa muviri ramai panguva yepamuviri.

The kunotoda Mufananidzo chirwere

The kuratidzwa rokutanga chirwere Klippel-Trenaunay-Weber atova kuoneka mune iri neonatal munguva. Mune zvisingawanzoitiki nguva vanoonekwa gare gare - muuduku. Classic zviratidzo chirwere ndedzinotevera zviratidzo:

1. Angioma - dandemutande mavara. Kakawanda, vari pa rimwe gumbo uye kufukidza nzvimbo huru. Angiomas ruvara dzinosiyana chiedza nerima pepuru. Nokupesvedzera panyama zvinhu (kukweshera, rova) ganda mu "birthmarks" nyore kukuvaradzwa, kune kubuda ropa.
2. Varicose mutsinga ari makumbo pemvura. Clinical kuratidzwa ichi hunhu - tortuosity, thickening, vakawedzera hwevanhu vanorwara uye nemidziyo rezasi richiripo.
3. Hypertrophy of zvakakanganisa gumbo. Nokuda zvakuvadza mutsinga yakadzika uye avo kuwanda muzvidimbu kunowedzera dhayamita. Mune zvimwe zviitiko, muitiro pfupa uye hurongwa iri. Zvino njodzi gumbo kungava kunopfuura utano richiripo.

Dzimwe nguva mumwe zviratidzo hosha haina kwakaratidzwa kana externally (semuenzaniso, varicose asingafungi mutsinga). Mune zvimwe zviitiko, zvokurwara mberi nokukurumidza, uye kukundwa Ndakatengesa kumigumo kusvika hunde uye yokumusoro muviri.

Diagnostics

Fungira Klippel-Trenaunay-Weber chirwere zvinogoneka kuburikidza rakakura Angioma. chiratidzo ichi anooneka kutanga. Gare gare varicose mutsinga uye hypertrophy yacho richiripo. The maitiro kuchiitwa chirwere ichi - mumwe-mativi kukundwa. Kana uchifungidzira chirwere kuitwa rabhoritari uye richishanda Diagnostics. First akaita Ultrasound uye Doppler Ultrasound repasi maoko nemakumbo. A chiri chirwere ichi ndicho kushaya kuwirirana pakati venous kumanikidzwa uye cardiac basa. mamirire Izvi zvinoratidzwa yakarurama mutsetse panguva venography.

Trenaunay Syndrome Klippel-Weber: mishonga yezvirwere

Kubviswa hosha kunoenderana mwero nokuparadzwa yakadzika tsinga. With chinokosha aienda pamusoro yokunzvenga rinoitwa. Kana huru Vienna akatema nzvimbo huru, mudziyo wakaita chakaita zvokugadzirwa zvinhu transplantation. Sezvi- kurapwa akashandisa sclerotherapy (zvinodhaka "Trombovar", "Wayne Fibro"), akapfeka compression andapeni. Uyewo zvinoda kuramba negashiro anticoagulants (mishonga "Warfarin"). Mune zvimwe zviitiko, vascular kuvhiya kunogona kuitwa chete Laser.