Mate Urbach-Vite o: tohumate, maimoatanga, waitohunga

Disease Urbach-Vite - he mate ira tino onge, tuku i roto i te tikanga ngoikore autosomal. Meinga o te mate - he irakētanga i runga i te pūira tuatahi i roto i ECM1 ira. I roto i te tuhinga hauora i reira he ingoa tetahi mo te mate - proteinosis lipoid.

History o whakahura

rēhita Ko te take tuatahi o te mate, he rite ki te mate Urbach-Vite nei tohumate, i roto i 1908. whakaahuatia te reira i te orometua Swiss me tākuta Friedrich Siebenmann. I roto i te 1925 he dermatologist Swiss me histologist Iogann Misher fakamatala'i take ōrite e toru.

Heoi, mana ko reira i korerotia te mate ira ngoikore onge kua anake i roto i te tau 1929 e te dermatologist me Urbach otolagingologom Vita. Te reira i huaina Erich Urbach māuiuitanga proteinosis lipoid, no te mea i whakapono ia e te pā reira ki te tāne i o lipid me te pūmua putunga i roto i te kopa o te tinana.

Kua Mai kua rēhitatia te whakatuwheratanga rite ki rerekē puna i toru rau ki te wha rau take o te mate te ao.

tohu

I roto i te tohu tino noa o te mate - ponana huri noa nga kamo rite papules (he ano ratou e wātea ana i runga i Luba me te kaki), riro te manawanui reo whango, i reira he he whakaiti i roto i te kaha whakaora o te kiri, mucosa thickening me induration o te kiko roro i roto i te rohe pae tino. Ko te tino mōrearea te tohu whakamutunga no te mea e taea e arahi i te reira ki te haurangi taupa pae tino.

Ko te mate tuatahi faaite ake i roto i detstkom tau (3-7 tau). Ko reira ka e reira he rashes runga te kiri, me te whango.

Me kï reira e he rangirua enei tohu me kia rerekē nui i manawanui ki te manawanui. Ka rite ki te mate autosomal Urbach-Vite tuku te ngoikore, e kore e faaite mai i te reira iho, ki te te tohuira o te irarā tonu ngoi. E mea pinepine e kore e te iwi ara ite e he ratou nga kawe o te ira mutant. Ētahi pāpāho he kua kahore tohu i te mate onge. A anake, ki te whai ngā mātua e rua te ira kawenga mō te proteinosis lipoid, ka taea e te mate faaite mai ano i roto i te uri.

tātaringa

He uaua ki te kia pāngia mate Urbach-Vite o no te tohu e kore he reira ao. I roto i te take o te hunga ponana (papules) i runga i nga kamo, ponana papular i runga i te kiri o te kaki me te rae, tātaritanga kau toro dermatologist.

Hoki te mate whakapae Urbach-Vite whakaritea MRI kahore rerekē e taea kitea haaputuputuraa o te konupūmā i roto i te tinana. Heoi, e kore e tenei tohu tika tohu proteinosis lipoid. He āhuatanga o te herpes encephalitis simplex me hoki Calcification.

He tūranga matua i roto i te tātaritanga o whakaari rangahau ira. E hanga ana e ia taea te reira ki te whakatau mo te tino ranei reira ko te ira irakētanga ECM1, a ki te pera, ahakoa i tenei irakētanga mata ki te mate Urbach-Vite.

maimoatanga

Ahakoa te meka i e kua whakatuwheratia te proteinosis lipoid mo te wa roa, ko reira he mate onge, rite kore e taea te ora te nuinga o abnormalities ira. Heoi, i te whanaketanga hou i roto i te mara o te mataora tukua te hiki i maha tohu o te mate.

Ko te kupu, kitea te au pai i ka te fariiraa "heparin" me "sulfoxide dimethyl", ahakoa kahore i roto i te wā katoa. whakahaere Oral o pota iti "dimethylsulfoxide" (Dimexidum) e tika ana ki ngā āhuatanga anti-inflammatory o te raau taero, te whakaiti faaiteraa mate dermotropic.

Ko te whakamahi o "D-penicillamine" ano ko te angitu, engari e kore e whakamahia whānuitia. Rēhita me take o tūroro "Tigasonom", noa te i whakaritea mo te psoriasis.

Mai te konupūmā te ruiatanga i roto i te kopa roro e taea arahi ki te whakatupuranga o ngātere hiko te taikaha me whakaeke hunga haurangi, anticonvulsants tūroro whakaritea ( "Metindion" "ethosuximide").

outlook

mate Urbach-Vite ki maimoatanga tika, rite ki te tikanga, e kore te mea mōrearea. Iwi e mate ana i tenei mate e taea te ora i te ora roa, me te ki tonu. Otiia e kore e ti'a ia tatou ia tau'a ore toronga tākuta auau. Nā ki te thickening o te membrane mucous i roto i ētahi wā whakawhanake airway tukuna ai. Taua tūroro ki te whakahoki hiahiatia patency o te airway te tracheotomy te whakamahi i te taiaho hauhā ranei.

I roto i te wā i reira arahi putunga konupūmā ki haurangi hunga haurangi, he mea e tika ana ki te kite i te mātanga-epileptolog e kōwhiria e te maimoatanga tika, i runga i te pakeke o tohu rānei.