Fungidzira uchirwadziwa nemudumbu kamwe kamwe, uchirwadziwa pasina chikonzero chakajeka, pamwe chete nekunzwa kurwadza kana kusanzwa simba mumakumbo ako, kushaya simba kwetsandanyama, uye kuda kurutsa. Kunyangwe mushure mekuona vanachiremba vakati wandei uye kuedzwa kakawanda, chikonzero chikuru chinoramba chiripo. Iwe kana mumwe munhu waunoziva akambosangana neizvi here? Chingave chirwere chisingawanzoitika chemajini chinowanzo furatirwa. Nhasi, tiri kuongorora chirwere ichi: Acute Hepatic Porphyria , kana AHP.
Chii chinonzi Acute Hepatic Porphyria (AHP)?
AHP imhuri yezvirwere zvisingawanzoitiki zvemajini zvinokanganisa tsinga dzako uye dzimwe nguva ganda rako. Hazviwanzoitiki, zvichikanganisa vanhu vashanu pavanhu 100,000 vega vega. Chirwere ichi chinogona kukonzera matambudziko ehutano anongoerekana aitika, akakomba, uye anogona kutyisidzira hupenyu.
Kuti tinzwisise chikonzero, ngatitarisei ropa rako. Masero edu matsvuku eropa ane puroteni inonzi Hemoglobin . Basa rayo guru nderekutora okisijeni kubva mumapapu ako woiendesa kunhengo dzako dzemuviri nenyama—sebasa rakagadzirirwa kuendesa okisijeni.
Kuti ugadzire puroteni iyi, muviri wako unoda chinhu chakakosha chinonzi Heme .
Kune vanhu vane AHP, enzyme chaiyo inodiwa pakugadzirwa kweHeme inenge isina kukwana kana kuti isipo. Funga nezvazvo sekuedza kubika resipi asi usina chinhu chakakosha; chigadzirwa chekupedzisira hachigone kugadzirwa nemazvo.
Kushaikwa kwema enzyme uku kunonyanya kuitika muchiropa , icho chinonziwo hepatic system. Ndosaka chirwere ichi chichinzi Acute Hepatic Porphyria .
Kana muviri wako usingakwanise kugadzira Heme, zvinhu zvinovaka makemikari—kunyanya zvinhu zvine chepfu zvakaita sePorphobilinogen (PBG) neAminolevulinic acid (ALA)—zvinotanga kuungana muchiropa. Chepfu idzi dzinopinda muropa uye dzinogona kufamba mumuviri wese, zvichikanganisa tsinga dzako. Kukuvadzwa uku kwetsinga ndiko kunotungamira kuzviratidzo zvakaita sekurwadziwa kwakanyanya , kusanzwa , uye kuda kurutsa .
Ndeapi Mhando Huru dzeAHP?
Kune mhando huru ina dzeAHP. Kunyange hazvo imwe neimwe ichisanganisira kushomeka kweenzyme yakasiyana mukugadzirwa kweHeme, dzose ina dzinonyanya kukanganisa chiropa netsinga.
Porphyria Isingaperi Isingaperi (AIP)
Iyi ndiyo mhando inonyanya kuwanikwa, ichibata 80% yevarwere veAHP. Inokonzerwa nekushaikwa kweHydroxymethylbilane synthase (HMBS) enzyme, inokonzerwa nekuchinja kwemajini eHMBS. Izvi zvinogona kugarwa nhaka kubva kumubereki, kana kuti zvinogona kuitika sekuchinja kwemajini pasina nhoroondo yemhuri yekare.
AHP Inonzwa Sei?
Zviratidzo zveAHP zvinosiyana zvakanyanya. Vamwe vanhu vangasamboona zviratidzo, nepo vamwe vangangotambura nekurwiswa kwakanyanya. Kuziva zviratidzo izvi kwakakosha.
| Sisitimu yeMuviri Yakakanganiswa | Zviratidzo Zvakajairika |
|---|---|
| Dumbu | Kurwadziwa kwakanyanya mudumbu ( chiratidzo chikuru ), kuda kurutsa, kurutsa, uye kuvimbiwa. |
| Sisitimu Yetsinga | Kunzwa kusanzwa kana kukwenya makumbo, kushaya simba kwetsandanyama, kurwadziwa kwetsandanyama, uye, kashoma, kuoma mitezo. |
| Hutano hwepfungwa | Kusagadzikana, kuzvidya mwoyo, kuvhiringidzika, uye kuona zvinhu zvisipo. |
| Mwoyo & Kumanikidzwa Kweropa | Kumhanya kwemoyo nekukurumidza (tachycardia) uye BP yakakwira. |
| Zvimwe | Weti yakasviba, yakatsvuka, kana kuti yakatsvukuruka (kunyanya panguva yekurwiswa). |
Nekuti AHP haiwanzoitiki, zviratidzo zvayo zvinowanzoonekwa sezvirwere zvakajairika, izvo zvinogona kunonoka kuongororwa.
Chii Chinokonzera Kurwiswa?
Vanhu vazhinji vane AHP vanorarama hupenyu hwakajairika, asi zvimwe zvinhu zvinogona kukonzera kurwiswa kamwe kamwe. Kuziva izvi kwakakosha:
- Mimwe Mishonga: Kunyanya mabarbiturates nemaantibiotic esulfonamide. Kana uine AHP, gara uchibvunza chiremba wako usati watanga chero mushonga mutsva.
- Doro: Chinhu chikuru chinokonzera kurwiswa kweAHP.
- Kuderedza macalorie/Kutsanya: Kudya zvine macalorie mashoma uye makabhohaidhiretsi mashoma kunogona kukonzera zviratidzo.
- Kuchinja kwemahormone: Kunyanya izvo zvine chekuita nekuenda kumwedzi kuvakadzi.
- Kushushikana uye Utachiona: Kushushikana kwemuviri, fivha, kana zvirwere zvakajairika sefuruwenza zvinogona kukonzera chirwere.
Unofanira Kuona Chiremba Rini?
Kana ukasangana nezvinotevera, usazviramba. Tsvaga rubatsiro rwechiremba kana:
- Une kurwadziwa kwakanyanya mudumbu kunoramba kuchiitika, kusingatsananguriki.
- Unonzwa zviratidzo zvine chekuita netsinga dzakadai sekunzwa kurwadza kana kushaya simba kwemakumbo.
- Une nhoroondo yemhuri inozivikanwa yePorphyria.
- Unoona kuti weti yako yashanduka kuita tsvuku kana kuti brown.
Kana ukasangana nedambudziko, rakadai sekurwadziwa kusingatsungiririki, kuoma kufema, kuvhiringidzika kamwe kamwe, kana kutadza kufambisa makumbo ako, tsvaga rubatsiro nekukasika kudhipatimendi rekukurumidzira riri pedyo newe kana kufonera 911.
Pane Kurapwa kweAHP Here?
Kunyange hazvo pasina mushonga wechirwere ichi chemajini, kune mishonga inoshanda zvikuru iripo yekudzivirira nekudzivirira kurwiswa.
Zvirwere zvakakomba zvinoda kuti munhu apinde muchipatara kuti agare zvakanaka.
- Nzira huru ndeyekuziva nekubvisa chinokonzera.
- Kurapa marwadzo zvine simba kunoshandiswa kudzora kusagadzikana kwakanyanya.
- Mishonga inonyorerwa kuderedza kuda kurutsa nekurutsa.
- Kupinza shuga mumuviri netsinga kunogona kubatsira kudzikamisa kugadzirwa kwezvinokonzeresa chepfu muchiropa.
- Kurapa Kwakananga: Kuisirwa Hemin kunoshanda sekutsiva zvakananga, zvichiratidza chiropa kuti chirege kugadzira Heme inokuvadza zvakanyanya.
- Pamusoro pezvo, kune avo vanowanzo sangana nekurwiswa, mishonga yepamusoro inomisa majini yava kuwanikwa.
Chiremba wako ndiye achasarudza chirongwa chekurapa chakanyatsokodzera zvaunoda.
Mharidzo Yekuenda Nayo Kumba
- Chirwere cheAcute Hepatic Porphyria (AHP) chirwere chisingawanzoitiki chemajini, asi nekurapwa, vanhu vazhinji vanorarama hupenyu hwakanaka uye hwakajairika.
- Chiratidzo chikuru kurwadziwa kwemudumbu kwakanyanya, kunoramba kuchiitika pamwe nematambudziko etsinga (kushaya simba, kusasimba).
- Kudzivisa zvinhu zvinokukonzera kurwara—zvakadai semishonga, doro, uye kutsanya kwenguva refu—zvakakosha.
- Kana uchifungidzira kuti une zviratidzo, kana kuti une nhoroondo yemhuri, bvunza chiremba wako nekukasika.
- Mishonga inoshanda iripo yekudzivirira zviratidzo uye kudzivirira kurwiswa mune ramangwana. Hapana chikonzero chekuvhunduka; shanda pamwe chete nemupi wehutano hwako.
AHP, Acute Hepatic Porphyria, Porphyria, kurwadziwa mudumbu, zvirwere zvetsinga, zvirwere zvemajini, heme, hemoglobin, porphyrin, zvirwere zvisingawanzoitiki
