Konke Okufanele Ukwazi NgePorphyria Cutanea Tarda

-Delile

• Izimpawu
• Izithombe ze-porphyria cutanea tarda
• Izimbangela
• Izici zobungozi
• Isigameko
• Ukuxilongwa
• Ukwelashwa
• Umbono

Ukubuka konke

I-Porphyria cutanea tarda (PCT) uhlobo lwe-porphyria noma ukuphazamiseka kwegazi okuthinta isikhumba. I-PCT ingenye yezinhlobo ezivame kakhulu ze-porphyria. Kwesinye isikhathi kubhekiselwa kukho ngokujwayelekile njengesifo se-vampire. Lokho kungenxa yokuthi abantu abanalesi simo bavame ukuhlangabezana nezimpawu kulandela ukuvezwa yilanga.

Izimpawu

Izimpawu eziningi ze-porphyria cutanea tarda zivela esikhumbeni. Izimpawu ezijwayelekile zifaka:

• amabhamuza esikhunjeni avele elangeni, kufaka phakathi izandla, ubuso, nezingalo
• photosensitivity, okusho ukuthi isikhumba sakho siyazwela elangeni
• isikhumba esincane noma esintekenteke
• ukukhula kwezinwele okukhulayo, imvamisa ebusweni
• uqweqwe nokusikeka kwesikhumba
• ububomvu, ukuvuvukala, noma ukulunywa kwesikhumba
• izilonda ezikhula ngemuva kokulimala okuncane esikhunjeni
• i-hyperpigmentation, okusho ukuthi amabala esikhumba aba mnyama
• umchamo omnyama kunobunsundu obujwayelekile noma obubomvu
• ukulimala kwesibindi

Ngemuva kokuqhuma kwamabhulethi esikhunjeni sakho, isikhumba singakhula. Kuvamile nokuthi isibazi sivele lapho amabhamuza eselaphekile.

Amachashazi e-Hyperpigmentation ngokuvamile avela ebusweni, ezandleni nasentanyeni.

Izithombe ze-porphyria cutanea tarda

Izimbangela

I-Porphyria cutanea tarda ingabangelwa izinto ezahlukahlukene. Izimbangela zivame ukuhlukaniswa njengezakhi zofuzo noma ezitholakele.

Izimbangela zofuzo ezivame kakhulu zifaka:

• umlando womndeni we-porphyria cutanea tarda
• Ukushoda njengefa kwe-enzyme yesibindi uroporphyrinogen decarboxylase
• umlando womndeni wesifo sesibindi noma umdlavuza wesibindi
• insimbi eningi yesibindi kunokujwayelekile

Izimbangela ezivame kakhulu ezitholakele zifaka:

• ukuphuza utshwala
• usebenzisa ukwelashwa kwe-estrogen
• usebenzisa izinto zokuvimbela inzalo ngomlomo
• ukuvezwa kwezinto ezithile zemvelo noma amakhemikhali, njenge-Agent Orange
• ukuthatha insimbi eningi kakhulu
• ukubhema
• ukuba nesifo sokusha kwesibindi kohlobo C
• une-HIV

Kwezinye izimo, akunakwenzeka ukunquma imbangela ye-porphyria cutanea tarda.

Izici zobungozi

Usengozini enkulu ye-porphyria cutanea tarda uma ubhema noma usebenzisa utshwala. Kungenzeka futhi ukuthi uthole lesi simo uma une-hepatitis C noma i-HIV.

Ukuvezwa kumakhemikhali athile, njenge-Agent Orange, nakho kungandisa ubungozi bakho. Kungenzeka ukuthi wavezwa yileli khemikhali uma ungumakadebona owayesebenza endaweni eyayino-Agent Orange.

Isigameko

I-Porphyria cutanea tarda ingathinta abesilisa nabesifazane. Kuvame ukuvela ngemuva kweminyaka yobudala engama-30, ngakho-ke akuvamile phakathi kwezingane noma intsha.

IPorphyria cutanea tarda ithinta abantu emhlabeni jikelele futhi ayigcini esifundeni esithile noma ezweni elithile. Kulinganiselwa ukuthi umuntu oyedwa kwabangu-10 000 kuya ku-25,000 unalesi simo.

Ukuxilongwa

Udokotela wakho angenza ukuhlolwa ngokomzimba, ahlole izimpawu, futhi aqophe umlando wakho wezokwelapha. Ngaphezu kwalokho, bangasebenzisa izivivinyo ezilandelayo ukuxilonga i-porphyria cutanea tarda:

• ukuhlolwa kwegazi
• ukuhlolwa komchamo
• indle ukuhlolwa
• isikhumba biopsy

Udokotela uzohlola amazinga akho e-porphyrin ne-enzyme yesibindi. Ukuhlolwa kofuzo kunganconywa kubantu abanomlando womndeni walesi simo.

Ukwelashwa

Ukwelashwa kwe-porphyria cutanea tarda kugxile ekulawuleni nasekumiseni izimpawu. Izinguquko zokuphila, njengokunciphisa ukusetshenziswa kotshwala nokungabhemi, nakho kungasiza.

Izinketho zokwelashwa ezivamile zifaka:

• i-phlebotomy, okungukususwa kwegazi ukunciphisa i-iron
• i-chloroquine (Aralen)
• i-hydroxychloroquine (i-Plaquenil)
• imithi yezinhlungu
• ama-iron chelators
• ukwelapha izifo ezibanga i-porphyria cutanea tarda, efana ne-HCV noma i-HIV

I-Phlebotomy ingenye yezindlela zokwelashwa ezivame kakhulu ze-porphyria cutanea tarda. Amaphilisi we-antimalarial nawo asetshenziswa kaningi.

Izinguquko zokuphila ezivamile zokwelapha i-porphyria cutanea tarda zifaka:

• ukugwema utshwala
• ukungabhemi
• ukugwema ukukhanya kwelanga
• usebenzisa i-sunscreen
• ukugwema ukulimala esikhumbeni
• ukungathathi ama-estrogens

Kungase kudingeke ukuthi ugqoke okokuvikela ilanga, imikhono emide nesigqoko ukuze uvikele ilanga.

I-Porphyria cutanea tarda ingakhuphula ubungozi besifo somdlavuza wesibindi noma i-cirrhosis, okuyisibazi sesibindi. Yingakho kubalulekile ukuthi ungaphuzi utshwala uma unalesi simo.

Umbono

I-Porphyria cutanea tarda ivame ukuthinta abantu abadala abaneminyaka engaphezu kwengama-30. Yisifo segazi esithinta kakhulu isikhumba. Isikhumba sakho singazwela kakhulu elangeni, ngakho-ke kungadingeka ukuthi uthathe izinyathelo ezithile zokugwema ilanga. Amabhamuza ajwayelekile kulesi simo.

Udokotela wakho angancoma ukwelashwa okuhlukile kwe-porphyria cutanea tarda. Amaphilisi e-phlebotomy kanye ne-antimalarial yizona zindlela zokwelashwa ezivame kakhulu.

Uma ufuna ukwesekwa, hlola lolu hlu olukhethiwe lwamabhulogi amahle kakhulu wokuphazamiseka kwesikhumba onyakeni.