Nhaka Dzakapiwa Neropa
Hemophilia - tsika inowanikwa nhaka yevarume kubuda ropa - chirwere chekare chakaiswa pasi pesimba mumakore makumi mashanu kana apfuura. Zvinyorwa zvechiJudha zvezana remakore rechipiri AD rinotaura kune vakomana vaida kuurayiwa pashure pokudzingiswa, uye chiremba weArab Chiremba Albucasis (1013-1106) akarondedzerawo varume mune imwe mhuri vachifa mushure mekukuvadzwa zvishoma.
Mune dzimwe nhau dzangobva kuitika, Mambokadzi Victoria weBritain mwanakomana weBritain Leopold aiva nehemophilia, uye vaviri vevanasikana vake, Alice naBeatrice, vakanga vari vatakuri vejeni.
Nadzo, hemophilia yakapfuudzwa kumhuri dzeumambo muSpain neRussia, zvichiita kune mumwe wevarume vechiduku vakakurumbira vane chirwere, Tsar Nicholas II mwanakomana wega, Alexei.
Inenge 1 mu10 000 vanhu vanoberekwa vane hemophilia A. Anenge 1 mu 50 000 vanhu vanoberekwa vane hemophilia B.
Zvinokonzera Nemhando
Kunyange zvazvo chirwere ichi chakazivikanwa uye chakanyorwa pamusoro, mumajaya akapfuura vashoma vaingofa nokuda kwaro nokuti vanachiremba vakanga vasingazivi chakave chakaita kuti chiiti kana kuti chingaitirwe sei. Mumakore ekuma1800, vanachiremba vakafunga kuti kubuda ropa kwakaitika nokuti mishonga yeropa yakanga isina simba. Muna 1937, chinhu chakawanikwa muropa rega rega raizoita hemophilic blood clot, iyo yainzi "anti-hemophilic globulin."
Muna 1944, vatsvakurudzi vakawana mune imwe nyaya kuti apo ropa kubva kune maviri ehemophiliacs akasiyana, akakwanisa kuvhara. Hapana aikwanisa kutsanangura izvi kusvikira muna 1952, apo vatsvakurudzi veEngland vaiziva kuti pane 2 maitiro e hemophilia.
Vakanga vachiri kudzidza mukomana ane makore gumi ane hemophilia ainzi Stephen Christmas uyo akanga asingaiti seane "chirwere" chaiye. Vakadana shanduro yake hemophilia B, kana kuti "Chirwere cheKrisimasi," uye yakanyanya kufanana nehemophilia A, kana kuti "classic hemophilia." Chirwere cheKrisimasi chinokanganisa 15-20% chete yevanhu vane hemophilia.
Coagulation Factors
Nekuwanikwa kweA neB B kwakauya kuziva kuti panofanira kuva nemhando dzakasiyana dze "anti-hemophilic globulin" inobatanidzwa mukuvhara. Mazita akapiwa kune aya akasiyana-siyana e "coagulation factors" nedare renyika dzakawanda muna 1962. Hemophilia A inoshaikwa ye Factor VIII, uye hemophilia B inoshayikwa ne Factor IX.
Kurapa
Kamwe pazvakajeka kuti hemophilia yakakonzerwa nekushayikwa kwekiagulation factor, kushandiswa kwechinhu chisina kukwana kwakava nzira yekurapa. Mukutanga kwema1950 mhuka yeplasma yakashandiswa. Pakazosvika 1970, coagulation factor concentrates yakaitwa kubva kumapulama evanhu aivepo. Zvinosuruvarisa, masayendisiti iyezvino anoziva kuti avo vanofungisisa vane zvirwere zvakadai sehepatitis neHIV, uye vanhu vazhinji vane hemophilia vakawana zvirwere izvi.
Nhasi, recombinant (genetically engineered) shanduro dze coagulation zvinhu zvinobudiswa, izvo zvinobvisa ngozi yehutachiona. Vana vane hemophilia vanopiwa kubatana kwechikonzero sekudzivirira kurapwa kuderedza ropa risingaperi uye kuvabatsira kurarama hurefu, hune utano, uye hunoita hupenyu.
Sources:
"Nhoroondo yeBleeding Disorders." Chii Chinonzi Kuvhiringidza Kunetseka ?. 2006. National Hemophilia Foundation.
17 Dec 2008
Shord, SS, & CM Lindley. "Coagulation mishonga uye kushandiswa kwavo." Am J Health-Syst Pharm 57 (2000): 1403-1417.