Hemophilia ndi matenda amtundu wa hemorrhagic omwe amalepheretsa magazi kuundana, kumayambitsa kutaya magazi kwanthawi yayitali atavulala komanso nthawi zina popanda kuvulala. Zimayambitsidwa ndi kusintha kwa majini komwe kumayambitsa kusowa kapena kusowa kwa mapuloteni oundana omwe amalepheretsa kuti magazi asapangidwe mwamphamvu kuti athetse magazi.

Hemophilia imatengedwa kuti ndi matenda a anyamata ndipo imakhudza mwana mmodzi mwa anyamata 1 obadwa. Komabe, atsikana amatha kunyamula kusintha kwa majini ndikukhala ndi kachirombo kakang'ono ka matendawa. Pa chiwerengero chonse cha anthu, munthu mmodzi mwa anthu 5000 aliwonse ali ndi hemophilia. (1) Pali mitundu iwiri yayikulu ya matendawa: hemophilia A ndi B. Kuchuluka kwa hemophilia A ndikwapamwamba kuposa kwa hemophilia B (12/000 amuna motsutsana ndi 1 / 1). (6).

Hemophilia idakali matenda ofooketsa komanso oopsa kwambiri zaka makumi angapo zapitazo kuchokera paubwana ndi unyamata. Masiku ano, chithandizo chamankhwala chogwira mtima koma choletsa chimathandizira kuchepetsa magazi komanso kuchepetsa kuvulaza ndi kulumala kwa anthu omwe ali ndi haemophilia.

mwanayo. Kutaya magazi kwa nthawi yayitali kumachitika pambuyo povulala kapena kuvulala pang'ono. Zitha kukhala zodzidzimutsa (kotero kulowererapo pakalibe zoopsa) mumitundu yoopsa ya matendawa. Kutuluka magazi kungakhale mkati kapena kunja. Dziwani kuti magazi mwa munthu amene ali ndi hemophilia sakhala kwambiri, koma kuti nthawi yake ndi yaitali. Kutuluka magazi m'minofu (mikwingwirima) ndi m'malo olumikizirana mafupa (hemarthrosis), makamaka mu akakolo, mawondo ndi m'chiuno, pakapita nthawi kungayambitse kuuma ndi kulemala, zomwe zingayambitse ziwalo.

Matendawa amakhala owopsa kwambiri ngati kuchuluka kwa zinthu zomwe zimalumikizana m'magazi ndizochepa (1):

• Mawonekedwe owopsa: kukha magazi modzidzimutsa komanso pafupipafupi (50% ya milandu);
• Mawonekedwe apakati: kukha magazi kwautali wotalikirapo pambuyo povulala pang'ono komanso kukha magazi komwe kumachitika mwadzidzidzi (10 mpaka 20% ya milandu);
• Maonekedwe ang'onoang'ono: kukha magazi kwautali kwanthawi yayitali koma kusatuluka kodzidzimutsa (30 mpaka 40% ya milandu).

Magaziwo amakhala ndi mapuloteni, omwe amatchedwa kuti clotting factor, zomwe zimapangitsa kuti magazi aziundana ndipo amasiya kutuluka. Kusintha kwa majini kumalepheretsa kupanga mapuloteniwa. Ngati zizindikiro zokhudzana ndi hemophilia A ndi B ndizofanana kwambiri, mitundu iwiriyi ya matendawa imakhala ndi chiyambi chosiyana: hemophilia A imayamba chifukwa cha kusintha kwa F8 jini (Xq28) encoding the coagulation factor VIII ndi l hemophilia B ndi masinthidwe. mu F9 jini (Xq27) encoding the coagulation factor IX.

Hemophilia imayamba chifukwa cha majini omwe ali pa X chromosome. Ndi matenda obadwa nawo omwe amadziwika kuti "X-linked recessive cholowa". Izi zikutanthauza kuti munthu wodwala amapatsira jini yosinthidwa kwa ana ake aakazi okha, omwe angathe kupatsira, ndi chiopsezo cha 50%, kwa ana awo aakazi ndi anyamata. Zotsatira zake, matendawa amakhudza amuna okha, koma akazi ndi onyamula. Pafupifupi 70 peresenti ya anthu omwe ali ndi hemophilia ali ndi mbiri ya banja lawo. (1) (3)

Kuchiza tsopano kumapangitsa kuti zitheke komanso kuletsa kutuluka kwa magazi. Amakhala ndi mtsempha wopereka antihemophilic factor: factor VIII for hemophiliacs A ndi factor IX ya hemophiliacs B. Mankhwala oletsa hemophilic awa amapangidwa kuchokera kuzinthu zotengedwa m'magazi (a chiyambi cha plasma), kapena opangidwa ndi mainjiniya. genetics (zophatikizanso). Amaperekedwa ndi jakisoni wanthawi zonse komanso mwadongosolo kuti apewe kutuluka kwa magazi, kapena kutsatira kutulutsa magazi. Physiotherapy imalola anthu omwe ali ndi hemophilia kukhalabe ndi kusinthasintha kwa minofu komanso kuyenda kwa mafupa omwe avutika magazi mobwerezabwereza.