Isifo sePorphyria

Umbala obomvu wegazi, kwakunye nokukwazi ukuthutha i-oksijeni, ngenxa yobungqina be-heme. Ngaloo ndlela, le ngqungquthela ye-biochemical yenziwa ngenxa yeezyme, ukuveliswa kwayo okufuna i-porphyrin. Xa ezi zinto zingekho, i-porphyria ikhula, njengomthetho, i-heredity iba yimbangela.

Genetics kunye ne-porphyria

Into ephambili ebangela ukuqhubela phambili kwesi sifo kukukho kweentsimbi ze-antigen HLA B7 kunye ne-HLA A3 egazini. Baqumbisa ukuveliswa okwaneleyo kwee-enzymes ezenza i-heme, kunye nokuphulwa kwe-oxidation ye-compid compounds.

Ukongeza kwimizimba ye-genetics, i-hepatitis B, i-C kunye ne-A engafanelekiyo (ngefom ephezulu) kunye nokuchithwa kwesibindi se-parenchyma, umonakalo womnatha wethambo kubalulekile.

Iimpawu ze-porphyria

Iimveliso ze-enzyme eziyimfuneko zenziwe kwintliziyo yesibindi kunye nomnatha wethambo. Ngako oko, iimpawu ze-porphyria ziyahlukahluka kuxhomekeke kwinqanaba elichaphazelekayo, kwakunye neentlobo ezahlukeneyo zokugula.

Udidi oluthile luba neenkqutyana ezilandelayo kunye nezibonakaliso zezibonakaliso:

1. Hereditary. Isibonakaliso esona sivakalisi sisona sibuhlungu obukhulu kwingingqi ye-epigastric. Ukongezelela, kukho i-tachycardia, ukuphazamiseka kwengqondo emnene, uxinzelelo lwegazi .
2. Efanelekileyo. Ikhosi iyafana nefom yefa, kodwa ichaphazela inkqubo yesistim neyomhlaba. Oku kuhambelana neengcamango, ukuhluthwa kwesisu, i-delirium kunye nokukhubazeka kwemisipha ephosakeleyo ye-bronchi, imiphunga.
3. Chester. Ufumene igama ngenxa yendawo yokuhlala (abantu abaninzi abagulayo besixeko saseChester eNgilandi) kunye nobuhlobo bobuhlanga. Ifana nefomu yangaphambilini ye-porphyria, kodwa inempawu ezinzima kakhulu, ngamanye amaxesha zineentlungu ezingaphumeleli.
4. ICoproprotoporphyria. Idibanisa ukubonakaliswa kwekliniki kwezi ziintlobo ezintathu ezikhankanywe ngentla yesifo, uphawu lwabemi baseMzantsi Afrika. Esi sifo sihamba kunye nokuqhuma kwi-epidermis.
5. Urokopproporfiriya. Ngokuqhelekileyo ifunyaniswa ngokuba yi-skin porphyria - iimpawu zibonakaliswa njengephula umthetho we-pigmentation kunye nokwakhiwa kwamabhulebhu amancinci kwindawo yezandla nobuso. Ihlala ichaphazela abahlali baseRashiya kunye namazwe asekuqaleni kweCIS.

Ukuba ukuhlanganiswa kwe-porphyrins kuphazamiseka kwintsipho yethambo, ezi ntlobo zezifo zikhula:

1. Erythropoietic uroporphyria. Uhlobo olubungozi kakhulu lokugula, kuba akunakukuvumela ukuba uphile kubantu abadala. Ubonakaliswe ngumonakalo kwesikhumba (kunye nokungabikho kokuphulukisa izilonda zesilonda), amazinyo, ukuqonda ukukhanya kwemini kunye nokuqhubela phambili-ngokusuleleka kwegazi.
2. I-coproporphyria ye-Erythropoietic. Uphawu oluphambili lubuchule obunamandla kwi-ultraviolet, obangela ukuvuvukala, ukuvumba kunye ne-hypermia ye-epidermis. Emva kokuhamba kwelanga ixesha elide, kukho kwakhona ukuvela kwe-vesicles, ukugqithisa okukhulu, izilonda kwesikhumba kunye namacwecwe.
3. Proryporphyria. Ichanekile ngqo kwikliniki efanayo njengoluhlobo lwangaphambili lwentsholongwane, lufunyaniswa ngolu vavanyo lwegazi (ukuxilongwa kweprotoporphin kwanda kwi-erythrocytes).

Ukunyangwa kwesifo se porphyria

Kuza kubekho, akukho ndlela efanelekileyo yokuphatha esi sifo. Utyando lwempawu luquka ukusetyenziswa isikimu esiyinkimbinkimbi sokuphelisa iimpawu eziphambili:

• ukunciphisa uxinzelelo lwegazi;
• splenectomy;
• plasmapheresis ;
• ukuthatha amayeza anqabileyo kunye nezigulane;
• ukhuselo olusisigxina kwi-ultraviolet radiation;
• isilungiso sexesha le-droppers nesisombululo se-glucose, ukufakwa kweerboxin, adenosine monophosphate, delagila.