Ciwon ƙwayar cuta

Sashin launin jini, da kuma iyawar safarar iskar oxygen, sun kasance ne a gaban kasancewar hem. Hakanan kuma, an kafa wannan fili na biochemical saboda enzymes, samar da abin da ke buƙatar porphyrin. Lokacin da waɗannan abubuwa sun kasa raguwa, porphyria tasowa, a matsayin mai mulkin, rashin daidaituwa ya zama dalilin.

Genetics da porphyria

Babban mahimmancin da ke tattare da ci gaba da cutar ita ce kasancewar masu sintiri na HLA B7 da HLA A3 cikin jini. Suna jawo rashin samar da enzymes wanda ya haifar da shi, da kuma rashin cin zarafi na mahaifa.

Bugu da ƙari, irin kwayoyin cututtuka, rashin ciwon hepatitis B, C da A (a cikin mummunan siffar) tare da rage lalata parenchyma, lalacewar kasusuwa na da muhimmanci.

Cutar cututtuka na porphyria

An kafa mahadi a cikin hanta da hawan kashi. Sabili da haka, alamun porphyria ya bambanta dangane da kwayar da aka shafi, da magunguna daban-daban.

Nau'in hawan ƙwayar cuta yana da ƙananan raƙuman ƙasa da halayen asibiti.

1. Raba. Maganin da aka fi sani shine zafi mai tsanani a yankin yankin. Bugu da kari, akwai tachycardia, m yanayin kwakwalwa, hauhawar jini .
2. Tsarin tsaka. Hanya ya yi kama da nau'in haɗin kai, amma yana rinjayar tsarin tsarin kula da tsakiya da na gefe. Wannan yana tare da hallucinations, cututtuka na kwakwalwa, delirium da nakasa na tsokoki na ƙwayar bronchi, huhu.
3. Chester. An sami sunansa saboda matsayi na geographic (yawancin marasa lafiya na garin Chester a Ingila) da kuma dangantaka ta asali. Hakazalika da siffar porphyria ta baya, amma yana da mummunan cututtuka, wani lokacin ma yana da rauni tare da gazawar koda.
4. Coproprotoporphyria. Yana haɗu da bayyanuwar cututtuka daga cikin nau'ikan da ke dauke da su a cikin asibiti guda uku da ke dauke da su, halayyar mazaunan Afirka ta Kudu. Haka kuma cutar tare da rashes a kan fuskar epidermis.
5. Urokopproporfiriya. Ana iya gano shi a matsayin fata porphyria - an nuna alamar cututtuka a matsayin cin zarafin pigmentation da kuma samuwar kananan kumfa a cikin hannu da fuska. Sau da yawa yakan rinjayar mazaunan Rasha da ƙasashen tsohon CIS.

Idan kira na porphyrins yana damuwa a cikin kututtukan kasusuwa, waɗannan nau'o'in ciwon suka ci gaba:

1. Eropthropoietic uroporphyria. Halin rashin lafiyar mafi hatsari, tun da yake yana da wuya ya ba ka damar rayuwa zuwa girma. Yana da lalacewa da fata (tare da marasa lafiya), hakora, haɓakawa ga hasken rana, da cigaba - ta hanyar kamuwa da cutar jini.
2. Erythropoietic coproporphyria. Babban alama alama ce mai karfin gaske ga ultraviolet, wanda ke haifar da kumburi, tayarwa da hyperemia na epidermis. Bayan an shafe rana mai tsawo, akwai kuma abin da ke faruwa na vesicles, haushin haɗari, ulcers a kan fata da kuma scars.
3. Erythropoietic protoporphyria. An kwatanta daidai da wannan asibiti a matsayin nau'in pathology na baya, an gano shi ta hanyar gwajin jini (ƙaddamar da protoporphin yana ƙaruwa a cikin erythrocytes).

Jiyya na cutar porphyria

Har zuwa yau, babu hanyoyin da za a iya magance cutar. Mahimmin maganin ya shafi amfani da ƙaddamar da makirci don kawar da babban alamar cututtuka:

• rage yawan karfin jini;
• Kayan aiki;
• plasmapheresis ;
• shan magungunan ƙwayoyin cuta da cututtuka;
• Tsarin dindindin daga radiation ultraviolet;
• sakawa na lokaci-lokaci na magunguna tare da bayani glucose, gabatarwar riboxin, adenosine monophosphate, delagila.