Kodi Hemophilia A ndi Chiyani?

Zamkati

• Kodi chimayambitsa hemophilia A ndi chiyani?
• Kodi hemophilia A imasiyana bwanji ndi B ndi C?
• Ndani ali pachiwopsezo?
• Kodi zizindikiro za hemophilia A ndi ziti?
• Matenda otupa magazi kwambiri
• Chiwombankhanga chochepa
• Haemophilia wofatsa
• Kodi hemophilia A imapezeka bwanji?
• Kodi zovuta za hemophilia A ndi ziti?
• Kodi hemophilia A imachiritsidwa bwanji?
• Haemophilia wofatsa A
• Matenda otupa magazi kwambiri A
• Kodi malingaliro ake ndi otani?

Hemophilia A nthawi zambiri amakhala ndi matenda am'magazi omwe amabwera chifukwa cha puloteni yotayika kapena yolakwika yotchedwa factor VIII. Amatchedwanso classical hemophilia kapena factor VIII kusowa. Nthawi zambiri, sichimatengera kubadwa, koma m'malo mwake chimayambitsidwa ndi chitetezo chamthupi m'thupi lanu.

Anthu omwe ali ndi hemophilia A amatuluka magazi ndikutunduka mosavuta, ndipo magazi awo amatenga nthawi yayitali kuti apange matumbo. Hemophilia A ndichikhalidwe chosowa kwambiri, chowopsa chomwe chilibe mankhwala, koma ndichachiritsika.

Pemphani kuti mumvetsetse bwino za matenda okhudzana ndi magazi, kuphatikizapo zomwe zimayambitsa, zoopsa, zizindikiro, komanso zovuta zomwe zingachitike.

Kodi chimayambitsa hemophilia A ndi chiyani?

Hemophilia A nthawi zambiri amakhala matenda amtundu. Izi zikutanthauza kuti zimayambitsidwa ndi kusintha (kusintha) ku jini inayake. Kusintha kumeneku ndikobadwa, kumachokera kwa makolo kupita kwa ana.

Kusintha kwa majini komwe kumayambitsa hemophilia A kumabweretsa kusowa kwa chinthu chotchedwa clotting factor chotchedwa factor VIII. Thupi lanu limagwiritsa ntchito zinthu zingapo zotseka kuti zithandizire kuundana pachilonda kapena povulala.

A clot ndi chinthu chonga gel osakaniza chopangidwa ndi zinthu mthupi lanu chotchedwa ma platelet ndi fibrin. Vundira limathandizira kuletsa kutuluka kwa magazi kuvulala kapena kudula ndikulola kuti lizichira. Popanda chinthu chokwanira VIII, magazi amatenga nthawi yayitali.

Nthawi zambiri, hemophilia A imachitika mosasintha mwa munthu yemwe alibe mbiri yabanja yamatendawo. Izi zimadziwika kuti hemophilia A. Amapeza chifukwa cha chitetezo chamthupi cha munthu popanga ma antibodies omwe amawononga factor VIII. Matenda a hemophilia amapezeka kwambiri mwa anthu azaka zapakati pa 60 ndi 80 wazaka komanso azimayi apakati. Kupeza hemophilia kwadziwika kuti kuthana nako, mosiyana ndi mawonekedwe obadwa nawo.

Kodi hemophilia A imasiyana bwanji ndi B ndi C?

Pali mitundu itatu ya hemophilia: A, B (yomwe imadziwikanso kuti Khrisimasi), ndi C.

Hemophilia A ndi B ali ndi zizindikilo zofananira kwambiri, koma zimayambitsidwa ndikusintha kwamitundu yosiyanasiyana. Hemophilia A imayamba chifukwa chosowa kwa clotting factor VIII. Hemophilia B imachokera pakuchepa kwa chinthu IX.

Kumbali inayi, hemophilia C imachitika chifukwa chosowa kwa XI. Anthu ambiri omwe ali ndi hemophilia yamtunduwu alibe zisonyezo ndipo nthawi zambiri samatuluka m'magazi ndi minofu.Kutuluka magazi nthawi yayitali kumachitika pambuyo povulala kapena kuchitidwa opaleshoni. Mosiyana ndi hemophilia A ndi B, hemophilia C imafala kwambiri ku Ashkenazi Ayuda ndipo imakhudza amuna ndi akazi mofanana.

Factor VIII ndi IX sizinthu zokhazokha zomwe thupi lanu limafunikira kuti zigwetse. Matenda ena osowa magazi amatha kuchitika pakakhala zolakwika za zinthu I, II, V, VII, X, XII, kapena XIII. Komabe, zoperewera pazinthu zina zotsekemera ndizosowa kwambiri, kotero sizambiri zomwe zimadziwika pazovuta izi.

Mitundu itatu yonseyi ya hemophilia imadziwika kuti ndi matenda osowa kwambiri, koma hemophilia A ndiofala kwambiri mwa atatuwo.

Ndani ali pachiwopsezo?

Hemophilia ndi osowa - amapezeka mwa mwana m'modzi yekha mwa amayi 5,000 aliwonse obadwa. Hemophilia A imachitika chimodzimodzi m'mitundu yonse komanso mafuko.

Amatchedwa chikhalidwe cholumikizidwa ndi X chifukwa kusintha komwe kumayambitsa hemophilia A kumapezeka pa X chromosome. Amuna amadziwitsa ma chromosomes ogonana a mwana, kupatsa X chromosome kwa ana aakazi ndi Y chromosome kwa ana amuna. Chifukwa chake akazi ndi XX ndipo amuna ndi XY.

Pamene bambo ali ndi hemophilia A, imapezeka pa X chromosome yake. Kungoganiza kuti mayi siwonyamula kapena ali ndi vutoli, palibe mwana wake wamwamuna amene adzalandire vutoli, chifukwa ana ake onse azikhala ndi chromosome Y kuchokera kwa iye. Komabe, ana ake onse aakazi adzakhala onyamula chifukwa adalandira chromosome X imodzi yokhudzidwa ndi hemophilia kuchokera kwa iye komanso X chromosome ya X yomwe sinakhudzidwe ndi mayi.

Amayi omwe amanyamula ali ndi mwayi wa 50% wopatsira ana awo kusintha, chifukwa X imodzi ya chromosome imakhudzidwa ndipo inayo sichoncho. Ngati ana ake adzalandira chromosome X yokhudzidwayo, adzakhala ndi matendawa, chifukwa X yawo yokhayo yotchedwa chromosome imachokera kwa amayi awo. Mwana wamkazi aliyense yemwe adzalandire jini lomwe lakhudzidwa ndi amayi ake adzakhala onyamula.

Njira yokhayo yomwe mayi angakhalire ndi hemophilia ndi ngati bambo ali ndi hemophilia ndipo mayi ali ndi chonyamulira kapena ali ndi matendawa. Mkazi amafuna kusintha kwa hemophilia pama chromosomes onse awiri a X kuti awonetse zisonyezo.

Kodi zizindikiro za hemophilia A ndi ziti?

Anthu omwe ali ndi hemophilia A amatuluka magazi nthawi zambiri komanso kwa nthawi yayitali kuposa anthu omwe alibe matendawa. Kutuluka magazi kumatha kukhala kwamkati, monga mkati mwa mafupa kapena minofu, kapena kunja ndikuwoneka, monga pakucheka. Kuchuluka kwa magazi kumatengera kuchuluka kwa VIII kwa munthu m'mwazi wake wamagazi. Pali magawo atatu azovuta:

Matenda otupa magazi kwambiri

Pafupifupi 60 peresenti ya anthu omwe ali ndi hemophilia A ali ndi zizindikiro zoyipa. Zizindikiro za hemophilia yayikulu ndi monga:

• kutuluka magazi kutsatira kuvulala
• kutuluka mwadzidzidzi
• zolimba, zotupa, kapena zopweteka zophatikizana zomwe zimayamba chifukwa chamagazi m'magazi
• mwazi wa m'mphuno
• Kutaya magazi kwambiri kuchokera pakucheka pang'ono
• magazi mkodzo
• magazi mu chopondapo
• mikwingwirima yayikulu
• nkhama zotuluka magazi

Chiwombankhanga chochepa

Pafupifupi 15 peresenti ya anthu omwe ali ndi hemophilia A ali ndi vuto lochepa. Zizindikiro za kuchepa kwa haemophilia A ndizofanana ndi hemophilia A woopsa, koma sizowopsa ndipo sizimachitika kawirikawiri. Zizindikiro zake ndi izi:

• Kutaya magazi nthawi yayitali pambuyo povulala
• kutuluka mwadzidzidzi popanda chifukwa chomveka
• kuvulaza mosavuta
• kuuma molumikizana kapena kupweteka

Haemophilia wofatsa

Pafupifupi 25 peresenti ya hemophilia A milandu imawonedwa ngati yofatsa. Nthawi zambiri matenda samapangidwa pokhapokha atavulala kwambiri kapena kuchitidwa opaleshoni. Zizindikiro zake ndi izi:

• Kutaya magazi kwa nthawi yayitali pambuyo povulala kwambiri, kupsinjika, kapena kuchitidwa opaleshoni, monga kuchotsa mano
• kuvulaza kosavuta komanso kutuluka magazi
• magazi osazolowereka

Kodi hemophilia A imapezeka bwanji?

Dokotala amapima matenda ake poyeza kuchuluka kwa chinthu VIII pamagazi anu.

Ngati pali mbiri ya banja ya hemophilia, kapena mayi ndi wonyamula wodziwika, kuyezetsa matenda kumatha kuchitika panthawi yapakati. Izi zimatchedwa kuti matenda a prenatal.

Kodi zovuta za hemophilia A ndi ziti?

Kutuluka magazi mobwerezabwereza komanso mopitirira muyeso kumatha kubweretsa zovuta, makamaka ngati sizichiritsidwa. Izi zikuphatikiza:

• kuchepa kwa magazi m'thupi
• kuwonongeka molumikizana
• Kutaya magazi mkati kwambiri
• Zizindikiro zamitsempha zotuluka magazi mkati mwaubongo
• chitetezo chamthupi pakachiza ka clotting factor

Kulandila magazi operekedwa kumawonjezeranso chiopsezo chanu chotenga matenda, monga matenda a chiwindi. Komabe, masiku ano magazi operekedwa amayesedwa mokwanira asanapatsidwe magazi.

Kodi hemophilia A imachiritsidwa bwanji?

Palibe mankhwala a hemophilia A ndipo iwo omwe ali ndi matendawa amafunikira chithandizo chamoyo wonse. Ndikulimbikitsidwa kuti anthu azilandira chithandizo kuchipatala chapadera cha hemophilia (HTC) ngati zingatheke. Kuphatikiza pa chithandizo, ma HTC amapereka zothandizira ndi chithandizo.

Chithandizochi chimaphatikizapo kuchotsa m'malo mwa magazi omwe asoweka chifukwa chama magazi. Factor VIII itha kupezeka kuchokera pakupereka magazi, koma pano nthawi zambiri imapangidwa mwaluso ku labu. Izi zimatchedwa zophatikizanso chinthu VIII.

Kuchuluka kwa chithandizo chimadalira kuopsa kwa matendawa:

Haemophilia wofatsa A

Omwe ali ndi mitundu yochepa ya haemophilia A angafunikire kuthandizidwanso pambuyo poti magazi ayamba kutuluka. Izi zimatchedwa chithandizo chazing'ono kapena chofunikira. Kulowetsedwa kwa mahomoni omwe amadziwika kuti desmopressin (DDAVP) atha kuthandiza kulimbitsa thupi kuti litulutse chinthu china chotsekereza kuti asiye kuyambika kwa magazi. Mankhwala omwe amadziwika kuti fibrin sealants amathanso kugwiritsidwa ntchito pamalo amabala kuti athandizire kuchiritsa.

Matenda otupa magazi kwambiri A

Anthu omwe ali ndi hemophilia A wowopsa amatha kulandira infusions nthawi ndi nthawi ya factor VIII yothandizira kupewa magawo amwazi komanso zovuta. Izi zimatchedwa mankhwala othandizira. Odwalawa amathanso kuphunzitsidwa kuti azilowetsa kunyumba. Milandu yayikulu imafunikira chithandizo chakuthupi kuti muchepetse kupweteka komwe kumadza chifukwa chakutuluka magazi m'malo am'magazi. Pazovuta kwambiri, opaleshoni imafunika.

Kodi malingaliro ake ndi otani?

Maganizo ake amatengera ngati wina alandila chithandizo choyenera kapena ayi. Anthu ambiri omwe ali ndi hemophilia A amamwalira asanakule ngati salandira chisamaliro chokwanira. Komabe, ndi chithandizo choyenera, chiyembekezo chokhala ndi moyo pafupifupi zaka zambiri chimanenedweratu.