Hemophilia chirwere chinotapukira chemajini chinotadzisa ropa kugwamba, zvichikonzera kubuda kweropa kwenguva refu kana kukuvara uye dzimwe nguva pasina kukuvara. Inokonzerwa nekuchinja kwemajini kunokonzera kushayikwa kana kusavapo kwekuvhara mapuroteni anodzivirira kuvhara kubva pakuumbwa kwakasimba zvakakwana kumisa kubuda ropa.

Hemophilia inoonekwa sechirwere chevakomana uye inobata 1 mu5000 kuberekwa kwevakomana. Zvisinei, vasikana vanogona kutakura genetic mutation uye kukudziridza chimiro chiduku chechirwere. Muhuwandu hwevanhu, 1 muvanhu gumi nevaviri vane hemophilia. (12) Kune marudzi maviri makuru echirwere: hemophilia A uye B. Kuwanda kwehemophilia A yakakwirira kudarika yehemophilia B (000/1 varume vachipikisana ne1 / 6). (000).

Hemophilia yakanga ichiri chirwere chinopedza simba uye chinouraya makumi emakore mashoma apfuura kubva paudiki uye kuyaruka. Nhasi, kurapa kunoshanda asi kunoganhurira kunoita kuti zvikwanise kudzikamisa kubuda ropa uye kuderedza kukuvadzwa kwemuviri nekuremara muvanhu vane hemophilia.

mwana. Kubuda ropa kwenguva refu kunoitika zvichitevera kukuvara kana kukuvara kudiki. Vanogona kunge varipo (saka vanopindira mukusavapo kwekutambudzika) mumhando dzakakomba dzechirwere. Kubuda ropa kunogona kuva mukati kana kunze. Ziva kuti kubuda ropa mumunhu ane hemophilia hakuna kunyanya kusimba, asi kuti nguva yaro yakareba. Kubuda ropa mumhasuru (mavanga) uye mumajoini (hemarthrosis), kunyanya muzviziso zvetsoka, mabvi uye muhudyu, nekufamba kwenguva kunogona kukonzera kuoma uye kuremara, izvo zvinogona kukonzera kuoma mitezo.

Chirwere ichi chinotonyanya kuomarara kana huwandu hwezvinhu zvinosangana muropa zvadzikira (1):

• Chimiro chakakomba: kungoerekana uye kazhinji kubuda ropa (50% yematambudziko);
• Chimiro chiri pakati nepakati: kubuda ropa kurefu kusingaenzaniswi zvichitevera kukuvara kudiki uye kubuda kweropa kusingawanzoitiki (10 kusvika 20% yezviitiko);
• Chimiro chidiki: kubuda ropa kurefu zvisingaite asi kusavapo kwekubuda kweropa (30 kusvika 40% yezviitiko).

Ropa rine mapuroteni, anonzi clotting factor, iyo inobvumira kuvhara kweropa uye nokudaro kumisa kubuda ropa. Kuchinja kwemajini kunodzivirira kugadzirwa kwemapuroteni aya. Kana zviratidzo zvine chekuita nehemophilia A uye B zvakanyanya kufanana, mhando mbiri dzechirwere ichi zvakadaro dzine mabviro akasiyana: hemophilia A inokonzerwa nekuchinja kweiyo F8 gene (Xq28) encoding iyo coagulation factor VIII uye l hemophilia B nekushanduka. muF9 gene (Xq27) inokodha iyo coagulation factor IX.

Hemophilia inokonzerwa nemajini ari pa X chromosome. Icho chirwere chemajini chinozivikanwa se "X-yakabatana recessive nhaka". Izvi zvinoreva kuti murume anorwara achapfuudza geni rakashandurwa chete kuvanasikana vake, vanogona kuripfuudza, nengozi ye50%, kuvanasikana vavo nevakomana. Nekuda kweizvozvo, chirwere ichi chinongobata varume chete, asi vakadzi vatakuri. Inenge 70% yehemophilia ine nhoroondo yemhuri. (1) (3)

Mishonga yava kuita kuti zvikwanisike kudzivirira nekumisa kubuda ropa. Zvinosanganisira intravenously kutungamirira antihemophilic factor: factor VIII yehemophiliacs A uye factor IX yehemophiliacs B. Iyi mishonga inorwisa hemophilic inogadzirwa kubva kune zvigadzirwa zvakatorwa kubva muropa (yeplasma yakabva), kana kuti inogadzirwa neuinjiniya. genetics (recombinants). Anoitwa nemajekiseni enguva dzose uye akarongeka kudzivirira kubuda ropa, kana kutevera chiitiko chekubuda ropa. Physiotherapy inobvumira vanhu vane hemophilia kuchengetedza kuchinjika kwemhasuru uye kufamba kwemajoini akatambura kubuda ropa kakawanda.