He aha ka ka maʻi pale o Klippel-Trenaunay-Weber?

E like me oe i ike, ua hala hanau mā ma genetically mai makua paha e ku mai ka 'aʻe' ana o embryogenesis. Anomalies hiki e ua loaʻa mai kekahi'aʻaʻa a hui. Loa pinepine i loaʻa ka mea, mahope koke iho o ka hanau ana, akā, i kekahi manawa, hoike mai ai ia lakou iho he nui hope. An hana mea he congenital anomaly maʻi pale, Klippel-Trenaunay-Weber. Ka maʻi pili i ka pathology o ka vascular'ōnaehana, ka mea e loli aiʻano nui ka lalo lala aa koko.

Akä naÿe, e na mea he nui 'ē aʻe congenital anomalies, Klippel-Trenaunay-Weber maʻi pale hiki i ka maikaʻi prognosis no ke ola. He ua loaʻa ma ka 'āwīwīʻoki lāʻau lapaʻau lapaau o ka maʻi.

Pathology: maʻi pale, Klippel-Trenaunay-Weber - aha ka ia?

I ka maʻi, ua hoikeia mai ke kakahiaka nui 20th kenekulia, ka wā e i ho'ākāka 'ia ma mua e nā kānakaʻepekema - a Klippel Trenaunay. Ma hope, Dr. Weber hou mua i 'ike e pili ana i kēia maʻi. Ua ua ike ia i ka maʻi i kekahi inoa - osteogipertrofichesky venous nevus.

Maʻi pale Klippel-Trenaunay-Weber loaʻaʻano nui i waena o ka kāne heluna. Ma ka manawa, 'ike e pili ana i ke kumu kumu o ka maʻi a me kona epidemiology mea,ʻaʻole lawa. I ka papa kuhikuhiE symptoms o ka pathology e nui "iole" (nevi) ma luna o nā wāwae, kekahi anawaena haʻahaʻa lala varicosity. I loko nō o ka kakahiaka nui kūpono 'ana o ka lapaʻau na hoailona a me ka oolea o lesions, ma kekahi hihia, i ka maʻi pale mea treatable. Ua pono ua kō i ke aloha a hiki i ka muli o hou i loko o ka mahinaʻai o vascularʻoki kino 'ana i kūpono.

Nā kumu o ka pilikia

No ka mea i ho'ākāka 'ia ma ka maʻi kŘpa nei (ma kahi o 100 mau makahiki aku nei), aole mea a ianoiyuaa aʻaʻohe ike loea' ike e pili ana i kona etiology. Aka hoi, eia kekahi mau theories, e like me a ulu Klippel-Trenaunay-Weber maʻi pale. Nā kumu o ka maʻi pili me ka ohana mōʻaukala. Ma ka hoʻomaopopo aku i ka papa kuhikuhiE etiological ololi o ka'ōewe mutation. E like me kekahi kumumanaʻo, i ke kumu o ka maʻi moe i loko o ka 'aʻe' ana o embryogenesis i loko o kakahiaka hāpai keiki. Ma lalo o ka aoao o ke? Aneie aʻe (Nine Inch kaʻapu kōheoheo, psycho-mea naʻau stress, waiū e pāhawewe) ia lŘlŘ aplasia venous kahua. Kona underdevelopment ua hoi me infectious konohiki pili. E like me ia i kēia kumumanaʻo, i ke kumu o ka maʻi o koʻohune a me nā mea hoʻomaʻi trapped i loko o ke kino o ka makuahine, i ka i hāpai keiki.

Ka lapaʻau kiʻi o ka maʻi

Ka mua hoike ana mai o ka maʻi pale Klippel-Trenaunay-Weber ia kekahi 'i loko o ka neonatal wā. Ma pohihihi keia hoopii, e hōʻike hope - i loko o kamaliʻi. Classic symptoms o ka maʻi i ka i kēia symptoms:

1. Angioma - punawelewele, kiko. Loa pinepine, ka mea i loaʻa ma luna o kekahi wāwae, a uhi i ka nui wahi. Angiomas waihoʻoluʻu i hoano e mai ka malamalama i ka pouli poni. Ma lalo o ka aoao o ke kino ai 'aʻe (kū'ē, hopena) ili, ma o ka "e ola," o ka hoopuni poino, malaila e kahe ana ke koko.
2. Varicose aa koko o na wawae o kaʻili kai. Lapaʻau hoike ana mai o keia ano - tortuosity, ai hanaʻia naʻe, mahuahua morbidity a me nā ipu o ka lalo lala.
3. Hypertrophy o ka hoʻohuli ai i nā wāwae. Aie i ka poino o ka hohonu aa koko a me kā lākou ka hoʻonuiʻana lala piʻina ma anawaena. Ma kekahi mau hihia, i ke kaʻina hana o ka iwi, a me ka'ōnaehana mea ma luna o. A laila, i ka hoʻohuli ai i nā wāwae i ia hou ma mua o ka mea ola ka lala.

I kekahi manawa, kekahi o nāʻano o ka maʻi ua i hoike ole externally (e laʻa me, varicose superficial aa koko). Ma kekahi mau hihia, o ka pathology progresses hoʻomaka koke, a me ke pio ana o i loaa mai, mai nāʻaoʻao a hiki i ke kumu a me ka hoaka o ke kino.

lapaʻau '

Manaʻo pü 'Klippel-Trenaunay-Weber maʻi pale mea hiki ma ka nui Angioma. Kēia symptom ikeia mua. Hope hui e varicose aa koko a me hypertrophy o ka lala. Ke ano hiʻona o keia maʻi - hoʻokahi-kapakahi pio. Inā e manaʻo ai he maʻi lawe mai keʻena hoʻokolohua, a vocal lapaʻau 'ana. Mua i hana ultrasound a me Doppler ultrasound o ka lalo lala. A hiʻona o keia maʻi pale o ka nele o ka mea hoʻopili ma waena o venous puʻe a me cardiac aayoaeuiinoe. Keia ano i ka lewa ma ka laina pololei i venography.

Trenaunay maʻi pale Klippel-Weber: ka iapaau ana o ka maʻi

Hoʻopau o ka maʻi hilinaʻi nui ma ke degere o ka make o ka hohonu aa koko. Me ka nui hou o ka holo alaʻokoʻa ua hana. Inā i ka papa kuhikuhiE Vienna hahau i ka nui ele ele, ka moku i kemikala mea transplantation. E like me ka hou lapaʻau noi sclerotherapy (ai ' "Trombovar", "Wayne Fibro"), ua hoʻokomoʻia mīkini'opihia huihui. Kekahi pono i kekahi a eia i? Eaia o anticoagulants (lapaau "Warfarin"). Ma kekahi mau hihia, hiki ke hana vascularʻoki kino me ke kukuna.