Porphyria: ndi chiyani, zizindikiro ndi momwe mankhwala amathandizira

Zamkati

• Zizindikiro za Porphyria
• Momwe mankhwalawa amachitikira

Porphyria imafanana ndi gulu la matenda amtundu komanso osowa omwe amadziwika ndi kuchuluka kwa zinthu zomwe zimatulutsa porphyrin, yomwe ndi protein yomwe imayendetsa mpweya wamagazi, yofunikira pakupanga heme ndipo chifukwa chake, hemoglobin. Matendawa amakhudza kwambiri dongosolo lamanjenje, khungu ndi ziwalo zina.

Porphyria nthawi zambiri amatengera, kapena kutengera kwa makolo, komabe, nthawi zina, munthuyo amatha kusintha koma osadwala, amatchedwa latent porphyria. Chifukwa chake, zinthu zina zachilengedwe zimatha kuyambitsa kuwonekera kwa zizindikilo, monga kuwonekera padzuwa, mavuto a chiwindi, kumwa mowa, kusuta, kupsinjika kwamaganizidwe ndi chitsulo chochulukirapo mthupi.

Ngakhale kulibe mankhwala a porphyria, chithandizochi chimathandiza kuchepetsa zizindikilo ndikupewa kuwonongeka, ndipo malingaliro a dokotala ndiofunikira.

Zizindikiro za Porphyria

Porphyria imatha kugawidwa molingana ndi mawonekedwe azachipatala kukhala ovuta komanso osatha. Pachimake porphyria imaphatikizapo mitundu yamatenda omwe amayambitsa zizindikiritso zamanjenje komanso zomwe zimawoneka mwachangu, zomwe zimatha kukhala pakati pa sabata limodzi mpaka 2 ndikukula pang'onopang'ono. Pankhani ya porphyria yanthawi yayitali, zizindikirazo sizogwirizana ndi khungu ndipo zimatha kuyamba ali mwana kapenaunyamata ndipo zimatha zaka zingapo.

Zizindikiro zazikulu ndi izi:

• Porphyria yovuta

  • Kupweteka kwambiri ndi kutupa m'mimba;
  • Kupweteka pachifuwa, miyendo kapena kumbuyo;
  • Kudzimbidwa kapena kutsegula m'mimba;
  • Kusanza;
  • Kusowa tulo, nkhawa komanso kusakhazikika;
  • Kupindika ndi kuthamanga kwa magazi;
  • Kusintha kwamaganizidwe, monga kusokonezeka, kuyerekezera zinthu m'maganizo, kusokonezeka m'maganizo kapena kusowa nzeru;
  • Kupuma mavuto;
  • Kupweteka kwa minofu, kulira, kufooka, kufooka kapena kufooka;
  • Mkodzo wofiira kapena wofiirira.

• Matenda osakanikirana kapena osakanikirana:

  • Kuzindikira dzuwa ndi kuwala kochita kupanga, nthawi zina kumayambitsa kupweteka komanso kuwotcha pakhungu;
  • Kufiira, kutupa, kupweteka ndi kuyabwa pakhungu;
  • Matuza pakhungu omwe amatenga milungu kuti achiritse;
  • Khungu losalimba;
  • Mkodzo wofiira kapena wofiirira.

Kuzindikira kwa porphyria kumachitika kudzera pakuwunika kwachipatala, momwe adotolo amawona zomwe zimafotokozedwa ndikufotokozedwa ndi munthuyo, komanso kudzera m'mayeso a labotale, monga kuyesa magazi, chopondapo ndi mkodzo. Kuphatikiza apo, popeza ndi matenda amtundu, kuyesa kwa majini kungalimbikitsidwe kuti muzindikire kusintha komwe kumayambitsa porphyria.

Momwe mankhwalawa amachitikira

Chithandizo chimasiyanasiyana kutengera mtundu wa porphyria. Mwachitsanzo, pachimake porphyria, chithandizo chimachitika mchipatala pogwiritsa ntchito mankhwala kuti muchepetse zizindikilo, komanso kuperekera seramu mwachindunji mumitsempha ya wodwalayo kuti ateteze kuchepa kwa madzi m'thupi ndi jakisoni wa hemin kuti muchepetse kupanga porphyrin.

Pankhani ya porphyria yodulira khungu, tikulimbikitsidwa kuti tipewe kuwonekera padzuwa komanso kugwiritsa ntchito mankhwala, monga beta-carotene, mavitamini D othandizira ndi mankhwala ochizira malungo, monga Hydroxychloroquine, omwe amathandiza kuyamwa porphyrin wochulukirapo. Kuphatikiza apo, pakadali pano, magazi amatha kutengedwa kuti achepetse kuchuluka kwa chitsulo ndipo, chifukwa chake, kuchuluka kwa porphyrin.