Hemophilia chirwere chinokonzerwa neropa . Munhu ane hemophilia inoshayika ropa rinodikanwa kuvhara ropa iro rinoguma nekubuda ropa kunowedzera.
Zviratidzo
Vanhu vane hemophilia dzimwe nguva vanotumidzwa se "bluerers" vasinganzwisisi zvinoreva kuti vaibuda ropa nyore. Zvichienderana nekuoma, kubuda ropa kunogona kuitika pasina (pasina kukuvara) kana mushure mekuvhiyiwa kana kushungurudzika.
Zviratidzo zvinosanganisira:
- Nguva refu nosebleeds
- Kubvambura kubva kune gums
- Matambudziko makuru
- Kubuda ropa kwenguva refu mushure mekuvhiyiwa kana kuputika, kusanganisira kudzingiswa
- Kufuridzira kwemajoka makuru (mapepa, mapapiro, mabvi, maziso) kubva kubuda kusvika mubatanidzwa
- Nhamo inobva kubuda ropa kusvika mujoka kana musimba
Ndiani Ari Pangozi?
Varume vakaberekerwa mumhuri vane nhoroondo yehemophilia mune dzimwe hama vari pangozi. Kuti tinzwisise nhaka yehemophilia, tinofanira kutaura zvishoma nezvinyorwa. Vanhurume vane X chromosome kubva kuna amai vavo uye Y chromosome kubva kuna baba vavo. Vakadzi vanogara nhaka X chromosome kubva kuna baba vavo namai vavo. Chiremera cheHemophilia chinowanika pa X chromosome, zvinoreva vanaamai (avo vari vatakuri vehutano) kupfuura ichi chiremera chemafuti kuvana vavo; iyi inonzi X-yakabatanidzwa nhaka. Nemhaka yokuti vane ma x chromosomes maviri, vanasikana havawanzovhiringidzi (asi vanogona kunge vari mumamiriro asingawanzo).
Kuziva
Hemophilia inofungidzirwa kana mukomana kana munhu ane ropa iro rinoratidzika kuwedzerwa.
Iyo inorwara nokuenzanisa ye coagulation zvinhu (mapuroteni anodiwa kuti avhare ropa).
Chiremba wako angangotanga neongororo yekuongorora kuti aongorore yose yekugadzirisa maitiro. Izvo zvinonzi prothrombin nguva (PT) uye nguva yechirwere chepromboplastin (PTT). Mu hemophilia, PTT inowedzera.
Kana iyo PTT ichiwedzerwa (pamusoro pehutano hwakatarwa), kushayikwa kwe coagulation chinogona kunge chikonzero. Uye ipapo chiremba wako acharonga maitiro ekugadzirisa (mapuroteni muropa anoramba kubuda ropa) 8, 9, uye 11. Kunze kwekunge pane nhoroondo yemhuri yakajeka, kazhinji zvinhu zvitatu zvinongororwa panguva imwe chete. Iyi miedzo inopa chiremba wako zvikamu zvebasa rechimwe nechimwe chezvinhu, izvo zvakaderera mune hemophilia. Kuongororwa kunogona kusimbiswa nemaitiro ekuongorora.
Aina
Hemophilia inogona kuenzanisirwa neyoyo coagulation chinhu chisina kukwana.
- Hemophilia Migumisiro yekushaikwa kwechikamu che8.
- Hemophilia B (inonziwo chirwere cheKrisimasi) inokonzerwa nekushaikwa kwechikonzero 9.
- Hemophilia C (inonziwo Rosenthal syndrome) inokonzerwa nekushaikwa kwechikonzero che11.
Hemophilia inogonawo kuenzaniswa nechiyero che coagulation chinowanikwa. Chero coagulation chinokonzera iwe, iwe unowedzera kubuda ropa.
- Unyoro: 6 - 40%
- Mutevedzeri: 1-5%
- Zvakanyanya: <1%
Kurapa
Hemophilia inobatwa ne-concentrates. Izvozvi zvinokonzera zvinoshandiswa kuburikidza nemuviri (IV). Hemophilia inogona kurapwa nenzira mbiri dzakasiyana: pazvinoda chete (apo kubuda ropa zvikamu zvinowanikwa) kana prophylaxis (kugamuchira chinhu kamwe, kaviri, kana katatu pavhiki kuvhara zvirwere zvekubuda ropa).
Maitiro ako anogadziriswa nezvinhu zvakawanda zvinosanganisira kusuruvara kwehemophilia yako. Nenzira yakawanda, vanhu vane hemophilia ine unyoro ndivo vanogona kurapwa pavanoda sezvo ivo vasina kubuda ropa. Nenzira yakanaka, ruzhinji rwemishonga ye hemophilia inotarisirwa kumba. Vabereki vanogona kudzidza kuti vagoita sei vana vavo kuburikidza nemuviri wemuviri kana kuti mukoti muimba anokwanisa kugadzirisa ichocho. Vana vane hemophilia vanogonawo kudzidza nzira yekugadzirisa nayo pfungwa, ivo vasati vava kuyaruka.
Kunyange zvazvo icho chinonyanya kuisa ndiyo inoshandiswa kurapwa, kurapwa uku hakuwaniki munyika dzose.
Hemophilia inogonawo kurapwa nemishonga yeropa. Chinhu 8 kushayikwa kunogona kurapwa ne cryoprecipitate (inogadziriswa ye plasma). Mvura yakachena yemalasma inogona kushandiswa kubata chirwere chechina uye kushandiswa kwefekitari 9.
Mune varwere vane hanya zvakanyanyisa, mushonga unonzi demopressin acetate (DDAVP) unogona kugadziriswa kuburikidza nemuviri kana musal spray. Iko inokurudzira muviri kuti ubudise zvivako zvechikamu che8 kuti ubatsire pakuguma kwekubuda ropa.