Porphyria - he mate o te taata hou

mate porphyria Ko te ahua motuhake o pathologies ate i te taumata ira, i roto i nei i reira he kōtuitanga pūtoto tino hē. Kei te whakapono te reira e reira e waru ngā pera-ka karanga kaupae enzymatic i te biosynthesis o pūtoto. He pakaru i te wā o tetahi o ratou e kua arahi ki te raruraru pērā i porphyria. te tikanga whakawhanake tere te mate. I roto i tēnei tuhinga ka aparau i tatou i te taipitopito tino e ko tenei mate, me te aha ko nga tikanga hou o te maimoatanga.

Porphyria - he mate o te taata hou. He aha te mea te huarahi o te mate?

I te tīmatanga, pūmua-kore wahi o te pūtoto - Kei te tahuri (te kore heme) ki te taonga paitini nei, i roto i te tahuri, ngā wā kai atu tino te kiko subcutaneous katoa. Na, ko te kiri āta riro te kauruku hāurakaho, hoko te kikokore ki te wā, me i pakū te rā tika. Ko te aha tūroro ki tenei mate, ka taea e kitea e koe maha tuwhenua me pokapoka. Na āta whakawhanake porphyria. Mate, mōhio tatou te ngaro o te maimoatanga wā, te tikanga hopu nga wāhi hou katoa o te kiri.

take

ofatiraa raupapa o heme biosynthesis, i kōrerotia i runga ake ahua, arata'i ki whakaeminga nui o porphyrins i te rauropi, me te pera ka karanga precursors (hei tauira, Ala me porphobilinogen). Ko te toenga whakamutunga he pānga paitini kaha i runga i te rauropi, rite te hua o take whakawhanake kē tohu haumanu. Ko te take mo te taua maumaui ko te irakētanga o te ira haepapa mo te mahi o etahi whākōkī whai wāhi i roto i te kōtuitanga maha-taahiraa o heme. I te taime, whai wāhi kaipūtaiao e i roto i te ako atu taipitopito o enei tukanga me tenei mate rite porphyria.

tohu mate

• Tūkino ki te pūnaha io pokapū.
• Mimi he hue te kotukutuku.
• mariao maha me tohu i runga i te tinana.
• Mamae me te hūhi i roto i te rohe puku.
• pēhanga toto Nui ake.

He aha kia tukinotia?

Mea oto, i te kau, tohunga taea kore whakahere tikanga whai hua o te therapy pathogenetic o tenei mate. I roto i ētahi wā (hei tauira, uroporfiriya erythropoietic) e taea e whai hua maimoatanga. porphyria taratahi (he mate i roto i te atamira whakapeka) tino kore e tukua te whakamahi o te dipyrone me tranquilizers, rite meinga ratou te exacerbation o tohu. I roto i te aroaro o te mamae i roto i taua mate rite porphyria, rite ki te tikanga, te paremata i roto i nga raau taero kaha.

mutunga

mate Porphyria (tau pikitia 1 whakaatu tetahi o ona faaiteraa), kāore pāngia te i roto i enei ra atu pinepine. Roto i te whakamutunga, kia tuhia te reira e tenei ahua o te mate tonu tonu tenei ra ruarua mōhiotia. Scientists i huri noa i te ao tonu ki te tirotiro i te take pakiaka o, tohu haumanu tuatahi, me te tikanga ki te aro i taua raruraru kino. E noho hauora!