Disease Rendu-Osler mate: tātaritanga me te maimoatanga

mate Osler-Rendu - ko te ahua o tōiti toto iti (arterioles venules ranei). Ko reira nui e kore e tenei tukanga i te pūāhua inflammatory. I roto i te nuinga o te wā, whakakitea te mate te rite te whatunga iaia (ranei atú). Ko puta kino iaia e kore anake i runga i te mata o te kiri. Ano kia puta tohumate i runga i nga kiriuhi mucous o ētahi atu whēkau (ngōrahi, ihu, waha, tōngāmimi).

Disease Rendu-Osler: Clinic

Hoki te wā tuatahi whakaahuatia te mate e te Māori o France - Lui Mari Rendu. Whakaotia te pikitia o te mate whakaratohia he tohunga Ingarangi William Osler. Mōhiohio me ngā kitenga i kō atu Frederika Vebera. I kitea te reira e riro i te mate te. Tamaiti nui ki te riro i te ira o te matua. E ai syndrome mamae rite rua tamaroa e tamahine. Hoki te whanaketanga o tona whakahoki nga ira e whai ake nei: ko te tuatahi te kawenga mō te endoglin, whakahaere te hanga o te glycoprotein. E pā ana te kāwai tuarua ki te take tupu Liliu. A, no te ngaro ratou oko irakētanga elasticity, i reira he mea kohakore i roto i o ratou hononga, abnormalities i te hanganga o ngā pūtau endothelial. E tano ia tapao e te mate Rendu-Osler (haemangiomatosis tohatohaina) - e kore te mea tonu mārama he huru onge, me te āhua o tona takenga. Ko te nuinga o te mate e whai ake nei: pānga reira 1 tangata ia 5,000.

Ko te tohu tuatahi o te mate

Ka rite ki te tikanga, ki runga ki te 6 E kore tau tohu kitea o te mate (ahakoa he tuku te mate Rendu-Osler). Ki runga ki pa tetahi tau (6-10 tau) i reira ko nga tohu tuatahi. I runga i te kiri kiriuhi mucous o te tahi atu whēkau ranei e taea te kite i te angiomas putanga (i.e., pukupuku iaia). Ka rite ki te hua, e taea te hanga e hē o tōiti me oko toto whero (kauruku puru ranei) wahi e tahe. āhuatanga Disease Rendu-Osler-Weber me e auau iti o te upoo- toto. Ka rite ki te hua, toto kia (ara rite te microtraumas nā). I te tahi taime anake te tohu e kia tohu i te aroaro o te mate, he nosebleeds. Ano, Ka taea e te kōtiro e paheke taimaha. No te mea whanake o te toto auau anemia, taka te taumata pūtoto.

atamira o te mate

He maha ngā wāhanga Disease Rendu-Osler-Weber. Hoki ia o ratou āhuatanga e etahi tohu. I roto i He iti nga tīmatanga o pāpaka iaia, i te ahua o wahi iti. Ko te takawaenga āhuatanga e ia he tohu o uaua pūngāwerewere, "pūngāwerewere". puka nodular - ko te ahua o whero (a tawhio porotītaha ranei) nodules e protrude runga ake te mata o te torutoru mitamano. E ratou he diameter o i te 5 ki te 7 mitamano. A, no te riro nga raraunga mātauranga pēhanga-tairongo koma (momo katoa). Na ka whakakiia e ratou ki te toto.

Whakarōpū o momo o te mate

Ka rite ki kua whakahuatia, kia waiho ai o tuku mate Rendu-Osler (Ka taea te kite faaiteraa mate whakaahua i roto i tēnei tuhinga), tuku nei i ngā mātua. Ki te mamae tenei syndrome me papa, ko toku whaea, kua ka tupu i roto i te puka ake nui te mate te tamaiti. He hoki ngā take, katea e whakatika tika ki te irakētanga. Tei runga i te wahi wehewehe nga tohunga lesion te momo o te nasal (ihu toto), pharyngeal, waihakihaki (kia tahe etahi wāhanga o te kiri). āhuatanga puka visceral e toto i whekau. kia hoki kia kitea te reira me te momo whakauru, e pā e rua te kiri me whekau.

Disease Rendu-Osler (ICD-10 waehere): āhuatanga mōrearea i tātaritanga

Tuatahi o te katoa, te take mōrearea matua ko te whakapapa. Ano, Ka taea e te mate whakapataritari rongoā tangohia e te wahine hapu nga marama e toru tuatahi i roto i. Haumaru, me te hopuhopu mate whakawhitia i roto i tenei wā. Ka taea e whakapataritari toto e whai ake nei: kakahu komukumuku i runga i te wāhi pakaru, te pēhanga toto tiketike, he kai porangi. Ko te kore o ngā āhuatanga wahi faufaa i roto i te kai e taea te whakapakari i oko toto - e te tetahi o nga take mo to ratou ngoikore. Ko rawa pono o kaitautoko o te kaimangatanga tenei.

tikanga o te tātaritanga

Waihanga tātaritanga haumanu o mate Osler-Rendu i runga i te whakamātautau o te huru tinana o te manawanui. Ko te tikanga i runga i te mata, tumuaki, ka taea e kite mucous telangiectasia. Tenei hanganga o te tae whero, e mahi i runga i te mata kiri. kia kia tūnga taua hē i runga i te whekau. E hinaaro tatou i te whakamātautau toto noa e taea tohu i te aroaro o te anemia. hanga tātari matüora taea reira ki te kitea tahi-mate, me te mate. Reira tā hoki he whakamātautau mimi. Ki te kitea pūtau toto whero reira, he reira taea ki te mea e te tākihi whakawhanake toto, roma mimi. whakahaere te mātanga te raupapa o ngā whakamātautau. Whakawiri te tauira hanga reira taea ki te whakawa i te pakaruhanga toto subcutaneous. Hoki te whakamātautau te kiri i raro i te pehitanga paewai mo te ia. whakarato taua mōhiohio me te pukupuku whakamātautau (te superimposed reira i runga i te kikowhiti mō te meneti torutoru). Ki te kahore he māharahara o te mate Rendu-Osler, e kore he tātaritanga kahore e mau ana i roto i whakamātautau i runga i te tere o te upoo- toto, toto roanga (wehenga maihao). taua ana kōhao tinana mā te whakamahi i te endoscope. Ki te he mea motu nga tohu waho, ka kia tomography computed. taea tēnei aratuka ki a koutou e kite i tetahi maumaui i roto i te whekau.

maimoatanga o te mate

Rendu-Osler mate maimoatanga, whakarato e whai ake. Tuatahi, ko te therapy momo tenei. takoto Ko tōna ngako i roto i te whakamahi o inhibitors o irrigation mata e toto. Enei rongoā tautāwhi i te resorption o te katinga toto, me te aukati i te toto na. Ka rite ki whakaaturanga mahi, e whai hua ana ētahi atu raau taero. E mea pinepine, e hiahiatia ana pokanga te. I roto i te hui nekehia te wahi pakaru o te oko. Ka taea e whakakapi te reira i te prosthesis (ki te tika). A, no te homai nosebleeds cryocautery he hua pai. Mā te hauota wai, wahi mauanga tio. Iti iho whai hua ko te whakamahi o te taiaho mō cauterization. I roto i ētahi wā, he therapy taiki.

Toto huhu (gemokomponentnaya therapy)

I roto i te wā i reira te mate Rendu-Osler ai mate toto nui, titau te whakamahi o te toto takoha. Hē o wāhanga haepapa mō te upoo-, hanga ake mo te huhu o te wē (tio hou). Kei te whakamahia i tēnei therapy mō ngā āhuatanga e whakawehi te ora tangata. Hoki i roto i ngā take pēnei i, kia hiahia koe i te huhu o platelets kaituku. I roto i te wā i reira te manawanui he ahua nui o anemia (taumata tino iti o te pūtoto) ranei haere mai pōroa, anemic (mate o mahara e tika ana ki te kore o te hāora ki te roro), e huhu o ngā pūtau toto whero i te kaituku toto. E mea pinepine e tukua e ratou i pūmua kitea i runga i te mata.

pōauautanga taea o te mate

mate Disease Rendu-Osler, te pathogenesis o te whanaketanga me te kore o te maimoatanga whai hua e taea te arahi ki te maha o ngā āhuatanga kino. Tuatahi, whakawhanake anemia. Ka taea e mate toto nui arahi ki pōroa anemic. Ko te tangata i roto i tenei take, ko te mauri, me te kore e urupare ki akiaki waho. Ko reira ano hoki e huaki ana taea o te pāpāngia. Ka taea e te mate arahi ki matapo (mo te toto ki te etine). ino roa State o whekau tino. pānga taha kino o te mate e taea e te pakaruhanga toto roro. Heoi, mate undiagnosed me mātau therapy taea ki te whakaora hauora me ora o tūroro.

āraihia

E hiahiatia ana mehua ārai Paraimere i mua i kitea te raruraru te. Mau utuafare i roto i i pāngia ki te mate Rendu-Osler kotahi, neke atu rānei ngā mema, kia kōrero ki te geneticist. He take i reira ki te kupu tohutohu te whakamanawanui i hapu te tamaiti. Me hoki ki te kia āwangawanga e pā ana ki te ope haumaru o te tinana. ka whai hīkoi auau mahi pai i roto i te huarahi, whakapakeke o te tinana. Ko tika hoki tika kai tōtika ki te kai whārite.

ārai Tuarua Ko whakamātautau auau i tohunga. Ko tōna whakaaro, ko te kite wawe o te mate. Ki te mea kua kua pāngia te syndrome, kia kia wā, me te oti maimoatanga. Ki te tika, pokanga te manawanui me tika kōrero i te hematologist. Tenei ka tukua ki te tiaki i te tangata i pōauautanga i roto i te pokanga (toto nui). kia hoatu ia manawanui me ana whanaunga ngā mōhiohio i runga i te āhua o ki te whakarato āwhina tuatahi mō te toto (e rua ā, me te waho). E tūtohutia ana ki te mahi i te huhu o wāhanga toto kaituku anake i roto i te wā o te tikanga tino.