Matenda a Porphyria

Mtundu wofiira wa magazi, komanso mphamvu yake yotengera mpweya, ndi chifukwa cha kukhalapo kwa heme. Pachifukwachi, chigawochi chachilengedwe chimapangidwa chifukwa cha michere, zomwe zimapanga porphyrin. Pamene zinthu izi ziri zosafunikira, porphyria imayamba, monga lamulo, chibadwidwe chimakhala chifukwa.

Genetics ndi porphyria

Chinthu chachikulu chomwe chimapangitsa kuti matendawa apite patsogolo ndi kukhalapo kwa zonyamulira antigen HLA B7 ndi HLA A3 m'magazi. Zimayambitsa kupanga mavitamini ochepa omwe amapanga heme, komanso kuphwanya mavitamini a lipid.

Kuphatikiza pa majini, matenda a chiwindi a B, C ndi A (omwe ali ovuta kwambiri) osasinthika, ndi kuwonongeka kwa chiwindi parenchyma, kuwonongeka kwa mafupa n'kofunika.

Zizindikiro za porphyria

Mavitamini oyenerera amapangidwa m'chiwindi ndi m'mafupa. Choncho, zizindikiro za porphyria zimasiyana malingana ndi chiwalo chokhudzidwa, komanso matenda osiyanasiyana.

Mtundu wamtunduwu uli ndi zigawo zotsatirazi ndi ziwonetsero zamakono:

1. Wokonzeka. Chizindikiro chodziwika kwambiri ndikumva kupweteka kwambiri m'dera la epigastric. Komanso, pali tachycardia, matenda ovutika maganizo, matenda oopsa .
2. Zosintha bwino. Maphunzirowa ndi ofanana kwambiri ndi mawonekedwe a cholowa, koma amakhudza dongosolo loyamba la mitsempha. Izi zimaphatikizidwa ndi kukhumudwa, kutupa kwa khunyu, kukhumudwa ndi kufooka kwa minofu yofewa ya bronchi, mapapo.
3. Chester. Ali ndi dzina lake chifukwa cha malo (makamaka anthu odwala a mumzinda wa Chester ku England) ndi maukwati awo. Mofanana ndi mtundu wakale wa porphyria, koma uli ndi zizindikiro zoopsa kwambiri, nthawi zina zimadzaza ndi impso kulephera.
4. Chiproprotoporphyria. Zimaphatikizapo mawonetseredwe a chipatala cha mitundu itatu yapamwambayi ya matendawa, zomwe zimachitika ndi anthu a ku South Africa. Matendawa amaphatikizidwa ndi mphutsi pamwamba pa epidermis.
5. Urokopproporfiriya. Kaŵirikaŵiri amapezeka ngati khungu porphyria - zizindikiro zimasonyezedwa ngati kuphwanya mazira ndi kupanga mapulogalamu ang'onoang'ono m'mbali mwa manja ndi nkhope. Nthawi zambiri zimakhudza anthu a ku Russia komanso mayiko omwe kale anali a CIS.

Ngati kaphatikizidwe ka porphyrins imasokonezeka m'mafupa, matendawa amayamba:

1. Erythropoietic uroporphyria. Matenda owopsa kwambiri, chifukwa salola kuti mukhale wamkulu. Amadziwika ndi kuwonongeka kwa khungu (popanda machiritso a zilonda zam'mimba), mano, kukhudzidwa kwa miyeso ya dzuwa, ndi kupitirira - ndi matenda a magazi.
2. Erythropoietic coproporphyria. Chizindikiro chachikulu ndi mphamvu yamphamvu ya ultraviolet, yomwe imayambitsa kutupa, kuyabwa ndi ma hyperemia a epidermis. Pambuyo pa kutentha kwa dzuwa kwa nthawi yaitali, palinso zochitika za vesicles, kutupa kwa magazi, zilonda pa khungu ndi zipsera.
3. Erythropoietic protoporphyria. Chodziwika bwino ndi chipatala chomwecho monga mtundu wammbuyo wamatenda, amapezeka mwa njira yoyezetsa magazi (protoporphin yawonjezeka mu erythrocytes).

Chithandizo cha matenda a porphyria

Pakadali pano, palibe njira zothandizira matendawa. Chizindikiro cha mankhwala chimaphatikizapo kugwiritsa ntchito ndondomeko yovuta kuthetsa zizindikiro zazikulu:

• kuchepetsa kuthamanga kwa magazi;
• splenectomy;
• plasmapheresis ;
• kutenga anti-inflammatory and analgesic mankhwala;
• chitetezo chosatha ndi ma radiation;
• kuika nthawi ya droppers ndi yankho la shuga, kutsekemera kwa riboxin, adenosine monophosphate, delagila.