I-Porphyria: kuyini, izimpawu nokuthi ukwelashwa kwenziwa kanjani

-Delile

• Izimpawu zePorphyria
• Yelashwa kanjani

I-Porphyria ifana neqembu lezifo zofuzo nezingajwayelekile ezibonakala ngokuqongelelwa kwezinto ezikhiqiza i-porphyrin, okuyi-protein ebhekele ukuthuthwa komoya-mpilo egazini, okubalulekile ekwakheni i-heme futhi, ngenxa yalokho, i-hemoglobin. Lesi sifo sithinta kakhulu isimiso sezinzwa, isikhumba nezinye izitho.

I-Porphyria ivame ukuzuzwa njengefa, noma izuzwe njengefa kubazali, noma kunjalo, kwezinye izimo, umuntu angaba nokuguquka kwesifo kodwa angasitholi lesi sifo, kubizwa ngokuthi yi-latent porphyria. Ngakho-ke, ezinye izinto zemvelo zingakhuthaza ukuvela kwezimpawu, njengokuvezwa yilanga, izinkinga zesibindi, ukusetshenziswa kotshwala, ukubhema, ingcindezi engokomzwelo kanye nensimbi eyeqile emzimbeni.

Yize lungekho ikhambi le-porphyria, ukwelashwa kusiza ukunciphisa izimpawu nokuvikela ukuqhuma, futhi izincomo zikadokotela zibalulekile.

Izimpawu zePorphyria

I-Porphyria ingahlukaniswa ngokusho kokubonakaliswa komtholampilo ibe yi-acute futhi engapheli. I-acute porphyria ifaka izinhlobo zesifo esidala izimpawu ohlelweni lwezinzwa futhi ezivela ngokushesha, ezingahlala phakathi kweviki elilodwa kuya kwamabili bese zithuthuka ngokuqhubekayo. Endabeni ye-porphyria engapheli, izimpawu azisahlobene nesikhumba futhi zingaqala ngesikhathi sobuntwana noma ebusheni futhi zihlala iminyaka eminingana.

Izimpawu eziyinhloko yilezi:

• I-porphyria enamandla

  • Ubuhlungu obukhulu nokuvuvukala esiswini;
  • Ubuhlungu esifubeni, emilenzeni noma emuva;
  • Ukuqunjelwa noma uhudo;
  • Ukuhlanza;
  • Ukuqwasha, ukukhathazeka nokuxakaniseka;
  • Ukushaya ngonyawo kanye nomfutho wegazi ophakeme;
  • Izinguquko zengqondo, njengokudideka, ukusangana, ukudideka noma ukuphanjaniswa ingqondo;
  • Izinkinga zokuphefumula;
  • Ubuhlungu bemisipha, ukuqaqamba, ukuba ndikindiki, ubuthakathaka noma ukukhubazeka;
  • Umchamo obomvu noma onsundu.

• I-porphyria engapheli noma enqamulelayo:

  • Ukuzwela ilanga nokukhanya okufakelwayo, kwesinye isikhathi kubangele ubuhlungu nokushisa esikhunjeni;
  • Ububomvu, ukuvuvukala, ubuhlungu nokunwaya kwesikhumba;
  • Amabhamuza esikhunjeni athatha amasonto ukuphola;
  • Isikhumba esithambile;
  • Umchamo obomvu noma onsundu.

Ukuxilongwa kwe-porphyria kwenziwa ngokuhlolwa komtholampilo, lapho udokotela abona khona izimpawu ezethulwe futhi ezichazwe ngumuntu, nangokuhlolwa kwelabhorethri, njengokuhlolwa kwegazi, indle kanye nomchamo. Ngaphezu kwalokho, njengoba kuyisifo sofuzo, kunganconywa ukuhlolwa kofuzo ukukhomba ukuguquka kwesibopho okubhekele i-porphyria.

Yelashwa kanjani

Ukwelashwa kuyahluka ngohlobo lomuntu lwe-porphyria. Endabeni ye-porphyria ebucayi, ngokwesibonelo, ukwelashwa kwenziwa esibhedlela kusetshenziswa imithi yokwelapha izimpawu, kanye nokuphathwa kweserum ngqo emthanjeni wesiguli ukuvimbela ukuphelelwa amandla kwamanzi nokujova kwe-hemin ukuze kuncishiswe ukukhiqizwa kwe-porphyrin.

Endabeni ye-porphyria enqunyiwe, kunconywa ukuthi uvikele ukutholakala kwelanga futhi usebenzise imishanguzo, njenge-beta-carotene, izengezo zikavithamini D namakhambi okwelapha umalaleveva, njenge-Hydroxychloroquine, esiza ukumunca i-porphyrin eyeqile. Ngaphezu kwalokho, kulokhu, kungakhishwa igazi ukuze kuncishiswe inani lensimbi elijikelezayo futhi, ngenxa yalokho, inani le-porphyrin.