Kurwadziwa kwakanyanya mudumbu uye kurasikirwa nepfungwa pasina chikonzero... Ngatidzidzei nezvechirwere ichi chisingawanzoitiki? (Acute Hepatic Porphyria)

Fungidzira, pasina chikonzero chakajeka, unobva wangoerekana wava nekurwadziwa kwemudumbu kusingatsungiririki. Pamwe chete neizvozvo, nhengo dzako dzinopera simba, unonzwa sekunge uchapera simba, uye unonzwa kuda kurutsa. Kunyangwe mushure mekuona vanachiremba vakati wandei nekuita bvunzo dzakawanda, chikonzero chaicho chechirwere hachiwanikwe. Iwe kana mumwe munhu waunoziva akambosangana nedambudziko iri here? Pamwe chikonzero chacho chirwere chisingawanzoitiki chemajini icho vanhu vazhinji munyika medu vasati vatombonzwa nezvacho. Nhasi tichataura nezvechimwe chirwere chakadai, Acute Hepatic Porphyria , kana AHP.

Zvichitaurwa zviri nyore, chii chinonzi Acute Hepatic Porphyria (AHP)?

AHP chirwere chisingawanzoitiki chinokanganisa tsinga dzedu uye dzimwe nguva ganda redu. Chishoma zvekuti chinobata vanhu vanosvika vashanu pavanhu 100,000. Chinogona kukonzera zviratidzo zvinorwadza kamwe kamwe. Dzimwe nguva, izvi zvinogona kuisa upenyu panjodzi.

Kuti tinzwisise izvi, ngatitaurei zvishoma nezveropa riri mumuviri wedu. Masero edu matsvuku eropa ane puroteni inonzi " Hemoglobin ". Basa rayo guru nderekutora okisijeni inouya mumapapu patinofema uye kuiendesa kune dzimwe nhengo dzese dzemuviri. Zvakafanana ne'basa rekutumira okisijeni'.

Pane chimwe chinhu chakakosha pakugadzira puroteni iyi chinonzi ``Hemoglobin'', uye tinochidana kuti ``Heme''' .

Chinoitika mumuviri wemunhu ane chirwere cheAHP kudzikira kana kurasikirwa neenzyme inodiwa panguva imwe chete mukugadzirwa kwechinhu ichi chinonzi ``Heme''. Zvakafanana nekubika ndiro, kana chimwe chinhu chisipo, ndiro haigone kugadzirwa nemazvo.

Kushaikwa kweenzyme iyi kunowanzoitika muchiropa chedu. Tinodaidzawo chiropa kuti `(Hepatic)`. Ndosaka chirwere ichi chakatumidzwa zita rekuti `Acute Hepatic Porphyria` .

Zvino, chii chinoitika kana tisingakwanise kugadzira `(Heme)`? Zvinhu zvakashandiswa pairi, zvinoti `(Porphobilinogen – PBG)` uye `(Aminolevulinic acid – ALA)`, zvinotanga kuungana muchiropa. Uturu uhu hunoungana muropa uye hunofamba mumuviri wese, kunyanya huchikuvadza tsinga dzedu. Imhaka yekukuvara kwetsinga ikoko ndiko kunoita kuti zviratidzo zvakaita sekurwadziwa kwakanyanya , kusanzwa , uye kuda kurutsa zvibude.

Ndeapi marudzi makuru echirwere cheAHP?

Kune mhando huru ina dzeAHP. Rudzi rumwe nerumwe runokonzerwa nekushaikwa kweenzyme yakasiyana mukugadzirwa kwe``(Heme)``. Asi dzose ina dzinonyanya kukanganisa chiropa netsinga.

Porphyria Isingaperi Isingaperi (AIP)

Iyi ndiyo mhando inokanganisa 80% yevarwere veAHP, kureva kuti, vazhinji. Mune izvi, pane kushomeka kwe enzyme `(Hydroxymethylbilane synthase - HMBS)`. Chikonzero cheizvi kuchinja kwejini re`(HMBS)`. Mukurapa, tinodana izvi kuti `(mutation)` gene . Izvi zvinogona dzimwe nguva kugara nhaka kubva kuvabereki kuenda kuvana. Kana dzimwe nguva zvinogona kuitika zvisina kurongeka mumunhu asina nhoroondo yemhuri.

Zviratidzo zveAHP zvinonzwa sei?

Havasi vese vane AHP vane zviratidzo zvakafanana. Vamwe vanhu vanogona kunge vasina zviratidzo muhupenyu hwavo hwese. Asi vamwe vanogona kunge vachirwara zvakanyanya. Zvakakosha kuziva zviratidzo izvi.

Ndezvipi zvinokonzera chirwere ichi kuti chiwedzere kuipa?

Munhu ane AHP anogona kurarama hupenyu hwakajairika. Zvisinei, zvimwe zvinhu zvinogona kukonzera chirwere ichi. Zvakakosha kuziva izvi.

• Mimwe mishonga: Kunyanya mimwe mishonga yakaita semishonga inorwisa mabhakitiriya ine `(Barbiturates)` uye `(Sulfa)`. Kana uine AHP, zvakakosha kutaura nachiremba wako usati watora chero mushonga.
• Kunwa doro: Doro ndiro rinonyanya kukonzera kuwedzera kwechirwere ichi.
• Kudya zvine mwero kana kutsanya: Kudya chikafu chine macalorie mashoma nemacarbohydrate (starch) kunogona kuwedzera chirwere ichi.
• Kuchinja kwemahormone: Kuchinja kwemahormone, kunyanya kuvakadzi, kunoenderana nekuenda kwavo kumwedzi.
• Kushushikana uye Utachiona: Chero rudzi rwekushushikana mumuviri, kunyangwe chirwere chinotapukira chakadai sefivha kana dzihwa, chinogona kuwedzera chirwere chacho.

Unofanira kuona chiremba rini?

Kana uine zviratidzo izvi, usazvitore sezvinowanzoitika. Zvakakosha kutsvaga rubatsiro rwechiremba, kunyanya muzviitiko zvinotevera:

• Kana uine kurwadziwa kwakanyanya mudumbu kusingatsananguriki, kunoramba kuchiitika.
• Kana zviratidzo zvetsinga zvakaita sekunzwa kusasimba uye kushaya simba zvikaitika panguva imwe chete.
• Kana uchiziva kuti mumwe munhu mumhuri yako ane chirwere ichi `(Porphyria)`.
• Kana ukaona kuti weti yako yava kutsvuka/kusviba.

Kana ukaona zviratidzo zvakakomba, zvakaita sekurwadziwa kusingatsungiririki, kunetseka kufema, kuvhiringidzika mupfungwa, kana kurasikirwa nemakumbo, enda kuEmergency Treatment Unit (ETU) yechipatara nekukasika.

Pane mushonga weAHP here?

Sezvo ichi chirwere chinokonzerwa nemajini, hapasati pava nemushonga. Zvisinei, kune mishonga inoshanda zvikuru yekudzora nekudzivirira kuwedzera kwechirwere ichi.

Muzviitiko zvakakomba, kurara muchipatara uye kurapwa zvinodiwa.

• Chinhu chikuru ndechekuona chinokonzera chirwere ichi uye kuchimisa.
• Mishonga ine simba yekuderedza marwadzo inopihwa kudzora marwadzo akanyanya.
• Mishonga inopiwa kudzora kuda kurutsa nekurutsa.
• Nekupa muviri glucose (shuga), kureva kuti, glucose ine saline kuburikidza netsinga, kugadzirwa kwechepfu muchiropa kunogona kuderedzwa.
• Sekurapa kwakananga , majekiseni e`(Heme)` anoiswa `(Hemin infusions)`. Izvi zvinoitwa nekupa `(Heme)` kunze, kutumira chiratidzo kuchiropa chichiti, "Une `(Heme)`, hauchadi kugadzira zvimwe," uye kumisa kugadzirwa kwechepfu.
• Kunze kweizvi, ikozvino kune mishonga yemazuva ano inogadzirwa kuburikidza netekinoroji yemajini kudzivirira chirwere ichi kuti chisadzokorore kune avo vane exacerbations dzinowanzoitika.

Chiremba anokuongorora ndiye achasarudza mushonga wakakodzera iwe.

AHP, Acute Hepatic Porphyria, Porphyria, Kurwadziwa kwedumbu, Zvirwere zvetsinga, Zvirwere zvemajini, Heme, Hemoglobin, Porphyrin, Zvirwere zvisingawanzoitiki