Porphyria: yintoni na, iimpawu kunye nendlela unyango ngayo

Umxholo

• Iimpawu zePorphyria
• Unyango lwenziwa njani

I-Porphyria ihambelana neqela lezifo zemfuza nezinqabileyo ezibonakaliswa kukuqokelelwa kwezinto ezivelisa i-porphyrin, eyiprotheyini ejongene nokuthuthwa kweoksijini egazini, ibalulekile ekwenzeni ihemme kwaye, ngenxa yoko, ihemoglobin. Esi sifo ikakhulu kuchaphazela luvo, ulusu kunye namanye amalungu.

I-Porphyria ihlala izuzwa njengelifa, okanye ifunyenwe kubazali, nangona kunjalo, kwezinye iimeko, umntu unokufumana utshintsho kodwa angasiphuhlisi esi sifo, ibizwa ngokuba yi-latent porphyria. Ke, ezinye izinto zokusingqongileyo zinokukhuthaza ukubonakala kweempawu, njengokuvezwa lilanga, iingxaki zesibindi, ukusetyenziswa kotywala, ukutshaya, uxinzelelo lweemvakalelo kunye nentsimbi egqithisileyo emzimbeni.

Nangona kungekho lunyango lwe-porphyria, unyango luyanceda ukunciphisa iimpawu kunye nokuthintela i-flare-ups, kwaye ingcebiso kagqirha ibalulekile.

Iimpawu zePorphyria

I-Porphyria inokuhlelwa ngokwembonakalo yeklinikhi ibe yingozi kunye nengapheliyo. I-porphyria ebukhali ibandakanya iintlobo zesifo esibangela iimpawu kwinkqubo ye-luvo kwaye ezibonakala ngokukhawuleza, ezinokuhlala phakathi kweveki e-1 ukuya kwezi-2 kwaye ziphucule ngokuthe ngcembe. Kwimeko ye-porphyria engapheliyo, iimpawu azisahambelani nolusu kwaye zinokuqala ngexesha lobuntwana okanye kwinqanaba lokufikisa kwaye zihlala iminyaka eliqela.

Iimpawu eziphambili zezi:

• I-porphyria efanelekileyo

  • Intlungu ebukhali nokudumba esiswini;
  • Iintlungu esifubeni, imilenze okanye umqolo;
  • Ukuqunjelwa okanye urhudo;
  • Ukuphalaza;
  • Ukuphuthelwa, ixhala kunye nokuphazamiseka;
  • Ukubetha kwentliziyo kunye noxinzelelo lwegazi oluphezulu;
  • Utshintsho lwengqondo, olunjengokudideka, ukubona izinto ezingekhoyo, ukudideka okanye ukuphambana;
  • Iingxaki zokuphefumla;
  • Ubuhlungu bemisipha, ukubetha, ukuba ndindisholo, ubuthathaka okanye ukukhubazeka;
  • Umchamo obomvu okanye omdaka.

• I-porphyria engapheliyo okanye ekhethiweyo:

  • Ubuntununtunu elangeni kunye nokukhanya okungeyonyani, ngamanye amaxesha kubangela iintlungu kunye nokutsha eluswini;
  • Bomvu, ukudumba, iintlungu kunye ukurhawuzelela ulusu;
  • Amadyungudyungu kulusu oluthatha iiveki ukuphola;
  • Ulusu olubuthathaka;
  • Umchamo obomvu okanye omdaka.

Ukuxilongwa kwe-porphyria kwenziwa ngeemviwo zeklinikhi, apho ugqirha ejonga khona iimpawu ezichaziweyo kunye nokuchazwa ngumntu, kunye novavanyo lwebhubhoratri, ezinje ngegazi, isitulo kunye neemvavanyo zomchamo. Ukongeza, njengoko sisifo semfuza, uvavanyo lwemfuza lunokucetyiswa ukuba luchonge utshintsho olunoxanduva lwe-porphyria.

Unyango lwenziwa njani

Unyango luyahluka ngohlobo lomntu lwe-porphyria. Kwimeko ye-porphyria ebukhali, umzekelo, unyango lwenziwa esibhedlele kusetyenziswa amayeza ukunciphisa iimpawu, kunye nolawulo lwe-serum ngqo kumthambo wesigulana ukunqanda ukomisa emzimbeni kunye neenaliti ze-hemin ukuthintela imveliso ye-porphyrin.

Kwimeko ye-porphyria ecekeceke, kuyacetyiswa ukuthintela ukubekwa elangeni kunye nokusebenzisa amayeza, anjenge-beta-carotene, izongezo ze-vitamin D kunye namayeza okunyanga isifo seengcongconi, njenge Hydroxychloroquine, enceda ukufunxa i-porphyrin egqithisileyo. Ukongeza, kulo mzekelo, igazi linokukhutshwa ukunciphisa inani lesinyithi esijikelezayo kwaye, ngenxa yoko, isixa se-porphyrin.