Chii chinonzi Hemophilia A?

Zvemukati

• Chii chinokonzera hemophilia A?
• Haemophilia A inosiyana sei neB naC?
• Ndiani ari panjodzi?
• Ndezvipi zviratidzo zvehemophilia A?
• Yakakomba hemophilia
• Moderm hemophilia
• Hunyoro hemophilia
• Hemophilia A inoonekwa sei?
• Ndeapi matambudziko ehemophilia A?
• Hemophilia A inorapwa sei?
• Hunyoro hemophilia A
• Yakakomba hemophilia A
• Ndeapi maonero?

Hemophilia A inonyanya kuve chirwere chekubuda ropa chinokonzerwa nepuroteni inoshaya kana isina kukwana inonzi factor VIII. Iyo inonziwo classical hemophilia kana kushomeka kwechikonzero VIII. Muzviitiko zvisingawanzoitika, haina kugarwa nhaka, asi pachinzvimbo inokonzereswa neyakajairika immune immune mukati memuviri wako.

Vanhu vane hemophilia A vanobuda ropa nekupwanya nyore, uye ropa ravo rinotora nguva yakareba kuti vagadzire. Hemophilia A chinhu chisingawanzo, chakakomba chisina mushonga, asi chinorapika.

Verenga pamusoro kuti uwane kunzwisiswa kuri nani kwechirwere ichi chekubuda ropa, kusanganisira zvikonzero, njodzi zvinhu, zviratidzo, uye zvingangoita matambudziko.

Chii chinokonzera hemophilia A?

Hemophilia A inowanzo kuve geni kusagadzikana. Izvi zvinoreva kuti zvinokonzerwa neshanduko (shanduko) kune rimwe geni. Kana shanduko iyi ikagarwa nhaka, inopfuudzwa kubva kuvabereki kuenda kuvana.

Iyo chaiyo geni shanduko inokonzera hemophilia A inotungamira mukushomeka kwechinhu chinovhara chinonzi factor VIII. Mumuviri wako unoshandisa akasiyana siyana ekugwamba zvinhu kubatsira kuumba clots pane ronda kana kukuvara.

Chembe chinhu chakaita segel-chakagadzirwa nezvinhu zvemumuviri wako zvinonzi maplatelet nefibrin. Mavhoti anobatsira kumisa kubuda kweropa kubva mukukuvara kana kutemwa uye kuritendera kupora. Pasina kukwana chinhu VIII, kubuda ropa kuchawedzerwa.

Zvishoma kazhinji, hemophilia A inoitika zvisingaite mumunhu asina nhoroondo yemhuri yekutanga yechirwere. Izvi zvinozivikanwa sekuwanikwa hemophilia A. Zvinowanzo kukonzerwa ne immune system yemunhu zvisirizvo kugadzira maantibhakosi anorwisa factor VIII. Kuwana hemophilia kunowanzoitika muvanhu vari pakati pemakore makumi matanhatu nemakumi masere ekuberekwa uye mumadzimai akazvitakura. Yakawanikwa hemophilia yave ichizivikanwa kugadzirisa, kusiyana neiyo nhaka fomu.

Haemophilia A inosiyana sei neB naC?

Kune matatu marudzi ehemophilia: A, B (inozivikanwawo seChristmas chirwere), uye C.

Hemophilia A uye B vane zviratidzo zvakafanana, asi zvinokonzerwa nekusiyana kwemajini. Hemophilia A inokonzerwa nekushomeka kwekugwamba kwechinhu VIII. Hemophilia B inoguma nekushomeka kwechinhu IX.

Kune rimwe divi, hemophilia C inokonzeresa kushomeka kwechinhu XI. Vazhinji vanhu vane mhando iyi yehemophilia havana zviratidzo uye kazhinji havana kubuda ropa muzvibatanidzwa nemhasuru.Kubuda ropa kwenguva refu kunowanzoitika mushure mekukuvara kana kuvhiyiwa. Kusiyana nehemophilia A uye B, hemophilia C inonyanya kuwanikwa muAshkenazi maJuda uye inobata vese varume nevakadzi zvakaenzana.

Chinhu VIII uye IX handicho chete chinovhara muviri muviri wako unoda kuumbika. Zvimwe zvinetso zvisingawanzo kubuda ropa zvinogona kuitika kana paine kushomeka kwezvinhu I, II, V, VII, X, XII, kana XIII. Nekudaro, kushomeka mune izvi zvimwe zvinogumbura zvinhu zvisingawanzoitika, saka hazvisi zvakawanda zvinozivikanwa nezvekukanganiswa uku.

Ese matatu marudzi ehemophilia anoonekwa sezvirwere zvisingawanzoitika, asi hemophilia A ndiyo yakajairika pane ese matatu.

Ndiani ari panjodzi?

Hemophilia ishoma - inoitika mune imwechete chete pavane zviuru zvishanu vanozvarwa. Hemophilia A inoitika zvakaenzana mumarudzi ese uye madzinza.

Inodaidzwa kuti X-yakabatana mamiriro nekuti shanduko inokonzera hemophilia A inowanikwa pane X chromosome. Vanhurume vanoona echromosomes ebonde emwana, vachipa X chromosome kuvanasikana uye Y chromosome kuvanakomana. Saka vakadzi vane XX uye varume vari XY.

Kana baba vane hemophilia A, iri pane X chromosome yake. Kufunga kuti amai havasi ivo vanotakura kana vane kusagadzikana, hapana kana mumwe wevanakomana vake achagara nhaka mamiriro, nekuti vese vanakomana vake vachave neY chromosome kubva kwaari. Nekudaro, vese vanasikana vake vanozove vatakuri nekuti vakagamuchira imwe hemophilia-yakabatwa X chromosome kubva kwaari uye isina kubatika X chromosome kubva kuna amai.

Vakadzi vari vatakuri vane 50 muzana mukana wekupfuudza shanduko kune vana vavo, nekuti imwe X chromosome inokanganiswa uye imwe haina. Kana vanakomana vake vakagara nhaka yechromosome X yakakanganiswa, vanozove nechirwere, sezvo yavo chete X chromosome ichibva kuna amai vavo. Chero vanasikana vanowana nhaka yakakanganiswa gene kubva kuna amai vavo vanozove vatakuri.

Nzira chete mukadzi yaanogona kuita hemophilia ndeyekuti baba vane hemophilia uye amai vari mutakuri kana vane chirwere zvakare. Mukadzi anoda hemophilia mutation pane ese X ma chromosomes kuratidza zviratidzo zvechimiro.

Ndezvipi zviratidzo zvehemophilia A?

Vanhu vane hemophilia A vanobuda ropa kakawanda uye kwenguva yakareba kupfuura vanhu vasina chirwere. Kubuda ropa kunogona kunge kuri kwemukati, senge mukati memapfundo kana mhasuru, kana yekunze uye inoonekwa, sekucheka. Kuomarara kweropa kunoenderana nekuti munhu ane huwandu hwei VIII muropa rake reropa. Pane matatu matanho ekuomarara:

Yakakomba hemophilia

Inenge makumi matanhatu muzana yevanhu vane hemophilia A vane zviratidzo zvakakomba. Zviratidzo zveakanyanya hemophilia zvinosanganisira:

• kubuda ropa zvichitevera kukuvara
• kubuda ropa
• dzakasimba, dzakazvimba, kana maronda majoini anokonzerwa nekubuda ropa mumajoini
• mhino dzemhino
• kubuda ropa kwakanyanya kubva pakucheka kudiki
• ropa mumurini
• ropa muchitsiko
• kukuvara kukuru
• maronda anobuda ropa

Moderm hemophilia

Vanosvika zvikamu gumi neshanu kubva muzana zvevanhu vane hemophilia A vane kesi ine mwero. Zviratidzo zve hemophilia A zvine mwero zvakafanana neakaomarara hemophilia A, asi zvishoma zvakanyanya uye zvinoitika kashoma. Zviratidzo zvinosanganisira:

• kubuda ropa kwenguva refu mushure mekukuvara
• kubuda ropa pasina chikonzero chaicho
• kukuvadza zviri nyore
• kuomarara kwemajoini kana kurwadziwa

Hunyoro hemophilia

Inenge makumi maviri neshanu muzana yehemophilia A zviitiko zvinoonekwa sezvinyoro. Kazhinji kuongororwa hakuitwi kusvikira mushure mekukuvara kwakakomba kana kuvhiyiwa. Zviratidzo zvinosanganisira:

• kubuda ropa kwenguva refu mushure mekukuvara zvakanyanya, kushungurudzika, kana kuvhiyiwa, senge kubviswa kwezino
• kupwanya kuri nyore uye kubuda ropa
• kubuda ropa zvisina kujairika

Hemophilia A inoonekwa sei?

Chiremba anoita ongororo nekuyera chiyero chechinhu VIII chiitiko mune sampuli yeropa rako.

Kana paine nhoroondo yemhuri yehemophilia, kana amai vanozivikanwa mutakuri, kuongororwa kuongororwa kunogona kuitwa panguva yekuzvitakura. Izvi zvinonzi prenatal diagnostic.

Ndeapi matambudziko ehemophilia A?

Kudzokorora uye kubuda ropa zvakanyanya kunogona kutungamira kumatambudziko, kunyanya kana kukaenda kusarapwa. Izvi zvinosanganisira:

• zvakanyanya anemia
• kubatana kukuvara
• kubuda ropa kwakadzika mukati
• neurological zviratidzo kubva pakubuda ropa mukati meuropi
• immune immune kune yekugwamba chinhu kurapwa

Kugamuchira infusions yeropa rakapihwa kunowedzerawo njodzi yako yezvirwere, zvakadai sehepatitis. Nekudaro, mazuva ano ropa rakapihwa rinoyedzwa kwazvo risati rawedzerwa.

Hemophilia A inorapwa sei?

Iko hakuna mushonga wehemophilia A uye avo vane chirwere vanoda kurapwa kwehupenyu hwese. Zvinokurudzirwa kuti vanhu vagamuchire kurapwa kune yakasarudzika hemophilia kurapwa nzvimbo (HTC) pazvinogoneka. Pamusoro pekurapwa, maHTC anopa zviwanikwa nerutsigiro.

Kurapa kunosanganisira kutsiva chinoshaikwa chekugwamba kuburikidza nekuwedzerwa. Chinhu VIII chinogona kuwanikwa kubva kune zvipo zveropa, asi izvozvi zvinowanzo gadzirwa zvakagadzirwa murabhorari. Izvi zvinonzi zvinodzokorodza chinhu VIII.

Kuwanda kwekurapwa kunoenderana nekukura kwechirwere.

Hunyoro hemophilia A

Ivo vane zvinyoro mafomu ehemophilia A vangangoda kutsiva kurapwa mushure mekubuda ropa episode. Izvi zvinonzi episodic kana pane-inoda kurapwa. Kukanganiswa kwehormone inozivikanwa se desmopressin (DDAVP) kunogona kubatsira kukurudzira muviri kuburitsa yakawanda inogwamba yekumisa chikamu chekubuda ropa. Mishonga inozivikanwa se fibrin sealants inogona zvakare kuiswa kune iyo ronda saiti kubatsira kusimudzira kupora.

Yakakomba hemophilia A

Vanhu vane hemophilia yakanyanya A vanogona kugamuchira nguva nenguva infusions yechinhu VIII kubatsira kudzivirira kubuda kweropa zvikamu uye matambudziko. Izvi zvinonzi prophylactic therapy. Varwere ava vanogona zvakare kudzidziswa kupa infusions kumba. Mhosva dzakakomba dzinogona kuda kurapwa kwemuviri kudzikamisa marwadzo anokonzerwa nekubuda ropa mumajoini. Muzviitiko zvakakomba, kuvhiya kunodiwa.

Ndeapi maonero?

Maonero acho anoenderana nekuti munhu arapwa zvakakodzera here kana kuti kwete. Vanhu vazhinji vane hemophilia A vanofa vasati vakura kana vakasagamuchira tarisiro yakakwana. Nekudaro, nekurapwa kwakakodzera, tarisiro yepedyo-yakajairwa tarisiro inofanotaurwa.