Mene ne ciwo na Klippel-Trenaunay-Weber?

Kamar yadda ka sani, haihuwa lahani sun shige a kan genetically daga iyaye ko tashi daga take hakkin embryogenesis. Anomalies za a iya generated daga duk wani nama ko zuciya da sauransu. Mafi sau da yawa an same su nan da nan bayan haihuwa, amma wani lokacin bayyana kansu yawa daga baya. An misali ne a nakasar anomaly ciwo, Klippel-Trenaunay-Weber. Haka kuma cutar tana nufin wani Pathology na jijiyoyin bugun gini tsarin, shi yafi shafar ƙananan reshe jannayẽnsa.

A bambanci ga mutane da yawa da sauran nakasar anomalies, Klippel-Trenaunay-Weber ciwo iya samun m hangen nesa ga rayuwa. Ya aka samu da dace m magani na cutar.

Pathology: ciwo, Klippel-Trenaunay-Weber - abin da yake da shi?

Cutar da aka sani tun farkon karni na 20th, a lõkacin da aka farko aka bayyana ta biyu masana kimiyya - da kuma Klippel Trenaunay. Daga baya, Dr. Weber kara riga ya bayani game da wannan cuta. An sani cewa cutar yana da wani sunan - osteogipertrofichesky venous nevus.

Syndrome Klippel-Trenaunay-Weber samu yafi tsakanin namiji yawan jama'a. A lokacin, bayanai game da tushen da cutar da kuma ta epidemiology bai isa ba. Babban bayyanar cututtuka na Pathology ne m "moles" (nevi) a kan kafafu, daban-daban diamita ƙananan reshe varicosity. Duk da farkon ci gaban na asibiti da ãyõyinMu, kuma mai tsanani daga raunuka, a wasu lokuta, da ciwo ne daman a. Wannan riba aka samu godiya zuwa fitowan da sabon a cikin filin na jijiyoyin bugun gini tiyata dabaru.

A Sanadin matsalar

Tun da cutar da aka bayyana a gwada kwanan nan (game da 100 da suka wuce), akwai a halin yanzu babu abin dogara bayani game da etiology. Duk da haka, akwai da dama theories, bisa ga abin tasowa Klippel-Trenaunay-Weber ciwo. A Sanadin cutar hade tare da iyali tarihi. An gane cewa, babban etiological factor ne gene maye gurbi. A cewar wani ka'idar, a cikin hanyar cutar ta'allaka da take hakkin embryogenesis a farkon daukar ciki. Ƙarƙashin rinjayar muhalli dalilai (sinadaran da guba, Psycho-wani tunanin danniya, daukan hotuna zuwa radiation) ya auku aplasia venous tsarin. Its underdevelopment ne ma dangantawa da cututtuka jamiái. A cewar wannan ka'idar, a cikin hanyar cutar kwayoyin ko ƙwayoyin cuta kama a tarko a cikin uwa tasa ta jiki a lokacin daukar ciki.

The asibiti hoto da cutar

A farko manifestations na ciwo Klippel-Trenaunay-Weber riga m, a cikin neonatal lokaci. A rare lokuta, sun bayyana daga baya - a cikin shimfiɗar jariri. Classic bayyanar cututtuka da cutar ne da wadannan cututtuka:

1. Angioma - gizo-gizo aibobi. Mafi sau da yawa, su ne samuwa a kan daya kafa da kuma rufe babban yanki. Angiomas launi iya bambanta daga haske zuwa duhu m. Ƙarƙashin rinjayar jiki dalilai (gogayya, bugu) fata a cikin "birthmarks" na sauƙi lalace, akwai aka zub da jini.
2. Varicose veins na kafafu na surface. Clinical bayyananen wannan hali - tortuosity, thickening, ya karu wajen kisa da kwanoni na ƙananan reshe.
3. Hypertrophy na shafa kafa. Saboda rauni na zurfin jijiyoyinmu da su yaduwa reshe ƙaruwa a diamita. A wasu lokuta, da aiwatar da kashi da kuma tsarin ne a kan. Sa'an nan ya shafa kafa iya zama fiye da lafiya reshe.

Wani lokacin daya daga cikin matakai ne na cutar da ake ba bayyana ko externally (msl, varicose na waje jijiyoyinmu). A wasu lokuta, da Pathology progresses hanzari, da kuma shan kashi na Saide daga extremities ga akwati da kuma babba jiki.

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Ake zargi da Klippel-Trenaunay-Weber ciwo ne zai yiwu ta m Angioma. Wannan alama bayyana farko. Daga baya shiga ta varicose veins da hypertrophy na reshe. A halayyar alama da wannan cuta - daya mai gefe shan kashi. Idan ka zargin wata cuta da za'ayi dakin gwaje-gwaje da kuma instrumental bincikowa. Da farko ya yi duban dan tayi da kuma Doppler duban dan tayi na ƙananan wata gabar jiki. A fasalin da wannan ciwo ne da rashin hulda a tsakanin venous matsa lamba da kuma cardiac aiki. Wannan halayyar da aka nuna ta mike layin a lokacin venography.

Trenaunay Syndrome Klippel-Weber: da magani daga cututtuka

Kawar da cutar dogara a kan mataki na halakar zurfin jannayẽnsa. Tare da wani gagarumin takaita na kewaye da aka yi. Idan babban Vienna buga wani babban yanki, da jirgin ruwa sanya roba abu dasawa. Kamar yadda wani ƙarin magani amfani sclerotherapy (kwayoyi "Trombovar", "Wayne Fibro"), sanye da matsawa tufafi. Har ila yau na bukatar akai liyafar na anticoagulants (magunguna "Warfarin"). A wasu lokuta, jijiyoyin bugun gini tiyata za a iya yi tare da Laser.