Kab mob Porphyria

Cov xim liab ntawm cov ntshav, as Well as nws lub peev xwm xa cov pa oxygen, yog vim muaj kev puas siab ntsws. Hauv lem, no cov biochemical compound yog tsim los ntawm cov enzymes, zus tau cov uas yuav tsum tau porphyrin. Thaum cov tshuaj no tsis muaj txhij, porphyria tsim, raws li txoj cai, heredity yog qhov ua rau.

Genetics thiab porphyria

Lub hauv paus tseem ceeb tshaj plaws rau qhov kev mob ntawm tus kab mob yog qhov muaj cov tshuaj tua tau HLA B7 thiab HLA A3 hauv cov ntshav. Lawv ua rau muaj kev tsim cov khoom tsis zoo uas tsim tawm hauv lub cev, nrog rau kev ua txhaum ntawm oxidation ntawm lipid compounds.

Ntxiv nrog rau cov noob caj noob ces, kab mob siab B, C thiab A (nyob rau hauv daim ntawv loj) nrog qhov degradation ntawm daim siab parenchyma, pob txha pob txha puas yog qhov tseem ceeb.

Cov tsos mob ntawm porphyria

Qhov tsim nyog cov zom tau tsim muaj nyob hauv daim siab thiab pob txha pob txha. Raws li, cov cim qhia ntawm porphyria sib txawv nyob ntawm seb puas cuam tshuam cov khoom, thiab ntau yam ntawm pathology.

Hom hepatic muaj cov nram qab subgroups thiab cov cwj pwm mob tshwm sim:

1. Hereditary. Cov kab mob tshaj plaws yog qhov mob siab heev hauv cheeb tsam epigastric. Tsis tas li ntawd, muaj cov tachycardia, mob hlwb, mob ntshav siab , mob ntshav siab .
2. Mob sib quas ntus. Lub chav kawm yog me ntsis zoo ib yam li daim ntawv cog lus, tab sis nws cuam tshuam rau hauv nruab nrab thiab peripheral hlwb. Qhov no yog nrog los ntawm kev pom kev, kev qaug dab peg, kev ua tsis ncaj thiab kev tuag ntawm cov nqaij ntshiv ntawm bronchi, lub ntsws.
3. Chester. Tau txais nws lub npe vim yog thaj chaw ntawm thaj chaw (feem ntau cov neeg mob hauv nroog Chester hauv Asmeskas) thiab kev nrhiav kev sib raug zoo. Zoo li yav dhau los ntawm porphyria, tab sis nws muaj cov tsos mob nyuab dua, qee zaus nws yog fraught nrog lub raum tsis ua hauj lwm.
4. Coproprotoporphyria. Nws combines cov kev soj ntsuam ntawm peb hom abovementioned ntawm tus kab mob, yam ntxwv ntawm cov inhabitants ntawm South Africa. Tus kab mob no yog nrog cov pob liab liab nyob rau saum npoo ntawm cov kab mob.
5. Urokopproporfiriya. Nws yog feem ntau kuaj raws li daim tawv nqaij porphyria - cov tsos mob tau hais tias yog ua txhaum ntawm pigmentation thiab tsim cov npuas me hauv cheeb tsam tes thiab ntsej muag. Nws feem ntau cuam tshuam cov neeg nyob hauv Russia thiab lub teb chaws ntawm tus qub CIS.

Yog hais tias tus synthesis ntawm porphyrins yog ntxhov hauv cov pob txha pob txha, cov hom kab mob no tsim:

1. Erythropoietic uroporphyria. Qhov feem ntau txaus ntshai ntawm kev mob, vim nws tsis tshua muaj kev tso cai rau koj mus nyob rau cov neeg laus. Nws yog tsiag ntawv los ntawm kev puas tsuaj rau ntawm daim tawv nqaij (nrog rau cov neeg tsis muaj kab mob loj), cov hniav, rhiab heev rau hnub ci tawg, thiab kev vam meej - los ntawm kev kis tau ntshav.
2. Erythropoietic coproporphyria. Cov tsos mob tseem ceeb yog ib qho muaj zog heev rau ultraviolet, uas ua rau o, khaus thiab hyperemia ntawm cov epidermis. Tom qab lub hnub ntev raug, muaj qhov tshwm sim ntawm cov hlwv, hemorrhagic pob, mob rau ntawm daim tawv nqaij thiab nti.
3. Erythropoietic protoporphyria. Cwj pwm raws nraim los ntawm tib lub tsev kho mob raws li yav dhau los ntawm hom pathology, nws yog kuaj los ntawm txoj kev kuaj ntshav (qhov concentration ntawm protoporphin tsub kom nyob rau hauv erythrocytes).

Kev kho mob ntawm kab mob porphyria

Txog rau hnub tim, tsis muaj txoj hauv kev zoo ntawm kev kho tus kab mob. Txoj kev kho mob uas yuav siv tau yog siv ntawm txoj kev npaj kom tshem tawm cov tsos mob tseem ceeb:

• txo cov ntshav siab;
• splenectomy;
• plasmapheresis ;
• noj cov tshuaj tiv thaiv thiab cov tshuaj tsis sib haum;
• tiv thaiv tas li tiv thaiv ultraviolet hluav taws xob;
• periodic installation ntawm droppers nrog kua qab zib, qhov qhia txog ntawm riboxin, adenosine monophosphate, delagila.