Hemophilia ndi matenda ochotsa magazi . Munthu yemwe ali ndi hemophilia akusowa magazi omwe amafunika kuti aphimbe magazi omwe amachititsa kuti magazi asatulukidwe.
Zizindikiro
Anthu omwe ali ndi hemophilia nthawi zina amatchedwa "azungu omasuka" amatanthauza kuti iwo amayamba magazi mosavuta. Malingana ndi kuuma kwake, magazi amapezeka mosavuta (popanda kuvulala) kapena atatha opaleshoni kapena kupwetekedwa mtima.
Zizindikiro zikuphatikizapo:
- Mphuno ya nosebleeds
- Kutsekemera kuchokera ku chingamu
- Mikwingwirima yayikulu
- Kuchetsa kwa nthawi yaitali pambuyo pochita opaleshoni kapena kuwombera, kuphatikizapo mdulidwe
- Kutupa kwa ziwalo zazikulu (mapewa, mabala, mawondo, mabotolo) kuchokera kutuluka m'magazi
- Ululu wochokera m'magazi mu mgwirizano kapena minofu
Ndani Ali Pangozi?
Amuna obadwira m'mabanja omwe ali ndi mbiri ya hemophilia mwa achibale ena ali pangozi. Kuti timvetse cholowa cha hemophilia, tiyenera kulankhula pang'ono. Amuna ali ndi X chromosome kuchokera kwa amayi awo ndi Y chromosome kuchokera kwa atate wawo. Akazi amaulandira X chromosome kuchokera kwa bambo ndi mayi awo onse. Chilema cha hemophilia chimapezeka pa X chromosome, kutanthauza amayi (omwe ali zonyamulira matendawa) apasitsa chilema cha chibadwa kwa ana awo; izi zimatchedwa cholowa chogwirizana ndi X. Chifukwa chakuti ali ndi ma chromosomes aŵiri, ana ambiri samakhudzidwa (koma akhoza kukhala osavuta).
Kudziwa
Hemophilia imakayikira pamene mnyamata kapena munthu ali ndi magazi omwe amawoneka ovuta.
Amapezeka ndi kuyesa zigawo za coagulation (mapuloteni amafunika kuti aziphimba magazi).
Dokotala wanu akhoza kuyamba ndi mayesero a ma laboratory kuti awonetsere dongosolo lonse la coagulation. Izi zimatchedwa prothrombin nthawi (PT) komanso nthawi ya thromboplastin (PTT). Mu hemophilia, PTT imakhala yaitali.
Ngati PTT ikulitali (pamwamba payomweyo), kusowa kwa coagulation chifukwa kungakhale chifukwa. Kenaka dokotala wanu aziyambitsa zokhazokha (mapuloteni m'magazi omwe amasiya magazi) 8, 9, ndi 11. Kupatula ngati pali mbiri yakale ya banja, nthawi zambiri zinthu zitatu zimayesedwa panthawi yomweyo. Mayeso awa apatseni dokotala wanu gawo limodzi la ntchito iliyonse, yomwe ili yochepa mu hemophilia. Kusanthula kungatsimikizidwe ndi kuyesedwa kwa majini.
Mitundu
Hemophilia ikhoza kusankhidwa ndi coagulation china chosowa.
- Hemophilia Zotsatira za kusowa kwa chigawo 8.
- Hemophilia B (yomwe imatchedwanso Khrisimasi) imachokera ku kusowa kwa factor 9.
- Hemophilia C (yomwe imatchedwanso Rosenthal syndrome) chifukwa cha kusowa kwa factor 11.
Hemophilia ikhoza kuwerengedwanso ndi kuchuluka kwa coagulation factor. Chochepa cha coagulation chomwe muli nacho, ndibwino kuti muzitsuka magazi.
- Wofatsa: 6 - 40%
- Zosasintha: 1-5%
- Zowonjezera: <1%
Chithandizo
Hemophilia imathandizidwa ndi factor concentrates. Izi zimaphatikizapo kudzera mu mitsempha (IV). Hemophilia imatha kuchiritsidwa m'njira ziwiri: pakufunidwa kokha (pamene magazi amapezeka m'magazi) kapena prophylaxis (kulandira chinthu kamodzi, kawiri, kapena katatu pamlungu popewera magazi m'magazi).
Momwe mumachitiridwa zimatsimikiziridwa ndi zinthu zambiri kuphatikizapo kuuma kwa hemophilia. Kawirikawiri, anthu omwe ali ndi hemophilia yochepa amatha kuchiritsidwa ngati akusowa magazi. Mwamwayi, mankhwala ambiri a hemophilia amaperekedwa kunyumba. Makolo angaphunzire momwe angathandizire ana awo kupyolera mu mitsempha panyumba kapena namwino wodwala kunyumba angathe kutero. Ana omwe ali ndi hemophilia angaphunzire momwe angagwiritsire ntchito ntchito yawo, nthawi zambiri asanakhale achichepere.
Ngakhale kuti chofunika ndi chithandizo chovomerezeka, mankhwalawa sapezeka m'mayiko onse.
Hemophilia ingathenso kuthandizidwa ndi mankhwala. Zovuta zokwanira 8 zimatha kuchiritsidwa ndi cryoprecipitate (mawonekedwe a plasma). Mazira a pulasitiki atsopano angagwiritsidwe ntchito pochiza chinthu cha 8 ndi kusowa kwafakitale 9.
Odwala ochepetsedwa, mankhwala otchedwa desmopressin acetate (DDAVP) amatha kuperekedwa kudzera mu mitsempha kapena minofu. Zimachititsa kuti thupi limasulire masitolo a 8 kuti athetse magazi.