GEMOFILIYA

GEMOFILIYA (Haemophilia; az gemo… va philia – mayl), bemorii irsi, ki ba on khunravii az etidol ziyod khos ast; yake az shaklhoi diatezi khunrez. G. az norasoii omile, ki baroi bastani khun zarur ast, paydo meshavad. Ba G. faqat mardon giriftor meshavand, zero ki onro az modari zohiran solimi khud meros megirand. G. se nav meshavad: A, V va S. Hususan G.-i A beshtar duchor meoyad (80 – 90%).
Dar mavridi G. binobar norasoii omilhoi VIII, IH va HI-i lakhtabandi khun shakh nameshavad. G.-i A binobar norasoii omili VIII, G.-i V dar natijai kambudii omili IX va G.-i S az sababi narasidani omili HI ba amal meoyad. Darajai vazninii G. ba norasoii omile, ki onro paydo kardaast, vobastagi dorad.
Alomathoi G. dar ovoni kudaki paydo shuda, bo mururi vaqt tadrijan noayon meshavand. Baze shaklhoi sabuki G. dar kalonsoli zohir megardad. Andake lat khurdani bemori G. boisi khunravii ziyodi zeri pust, dokhili mushak va daruni bandu bugumho meshavad. Hayoti shakhsi giriftori G. hatto az andak buridani pust, kandani dandon va g. niz zeri khavf mondanash mumkin ast.
G. dar mardhoe, ki az ovoni kudaki giriftori khunshori meboshand, ba mushohida merasad. Tashkhisi nihoi badi tadqiqi laboratorii khun guzoshta meshavad (badi muqarrar kardani muhlati lakhtabandii khun, mavjudiyati omili muvofiqi lakhtabandi va g.)
Dar davrai khunshori gemoterapiya tain karda meshavad: hangomi G.-i A az kriopresipitat (konsentrati tozai omili VIII-i lakhtabandii khun), hamchunin usuli guzarondani plazmai nav yo plazmai ziddi G. istifoda mekunand; dar mavridi G.-i V va S plazma va dorui PPSB (majmui omilhoi II, VII, IX va X-i lakhtabandii khun) kor farmuda meshavad. Dar asnoi zarurat ba jarrohi marizoni G.-ro dar bemorkhonahoi makhsus khobonda, qablan norasoii omili lakhtabandii khunro barqaror mekunand. Dar davrai shadid budani jarayoni gemartrozho az gemoterapiyai umumi istifoda karda meshavad; dar inguna holatho mumkin bandro surokh karda, khuni jamgashtaro berun orand.
Solhoi okhir dar tabobat va peshgirii orizahoi G. komyobihoi nazarras ba dast omadaand. Az plazmai khuni odamoni solim konsentrati moddahoe, ki ba bemoroni G. namerasand, tayyor karda meshavand. Istemoli onho khunraviro peshgiri mekunad. Baroi muolijai bugumhoi illatyofta usulhoi samarabakhshi varzishi shifoi daryoft shudaand. Dar jumhuri Markazi ilmii khun faoliyat dorad, ki baroi tabobati giriftoroni G. mavodi zaruri omoda mesozad.
Kasoni giriftori G. khudro boyad az har guna zarbu lat va hatto zakhmi khurd ehtiyot kunand. Kormandoni tib boyad pesh az jarrohi tadbirhoi peshgirii khunraviro andeshand. Bemori G. barobari sar shudani khunravi boyad hatman ba dukhtur murojiat kunad.