Hemophilia B

Hemophilia B chirwere chekubuda nhaka chinokonzerwa nekushaikwa kweropa rinovhara ropa IX. Pasina chakakwana chinhu IX, ropa harigone kugwamba zvakanaka kudzora kubuda kweropa.

Paunobuda ropa, akateedzana maitiro anoitika mumuviri ayo anobatsira kuwanda kweropa fomu. Maitiro aya anonzi coagulation cascade. Inosanganisira mapuroteni akasarudzika anonzi coagulation, kana zvinogwambisa ropa. Unogona kuve nemukana wakakwirira wekubuda ropa zvakawandisa kana chimwe kana zvimwe zvezvinhu izvi zvisipo kana zvisiri kushanda sezvavanofanira.

Chinhu IX (pfumbamwe) ndechimwe chakadai coagulation chinhu. Hemophilia B mhedzisiro yemuviri usingaite zvakakwana chinhu IX. Hemophilia B inokonzereswa neyakagamuchirwa X-yakabatana yekudzokorora hunhu, iine chakaremara geni iri pane X chromosome.

Vakadzi vane makopi maviri eX chromosome. Kana iyo factor IX gene pane imwe chromosome ikasashanda, iyo geni pane imwe chromosome inogona kuita basa rekugadzira zvakakwana chinhu IX.

Vanhurume vane imwe chete X chromosome. Kana iyo factor IX gene isipo pane mukomana X chromosome, achave neHemophilia B. Nechikonzero ichi, vanhu vazhinji vane hemophilia B varume.

Kana mukadzi aine chakaremara chinokonzera IX gene, anoonekwa seanotakura. Izvi zvinoreva kuti rakakanganisika geni rinogona kupfuudzwa pasi kuvana vake. Vakomana vanozvarwa nevakadzi vakadai vane mukana we50% wekuve nehemophilia B. Vanasikana vavo vane mukana we50% wekutakura.

Vose vana vechikadzi vevanhu vane hemophilia vanotakura rakaremara jeni.

Njodzi dzinokonzera hemophilia B dzinosanganisira:

• Nhoroondo yemhuri yekubuda ropa
• Kuva murume

Kukura kwezviratidzo kunogona kusiyana. Kubuda ropa kwenguva refu ndicho chiratidzo chikuru. Inowanzoonekwa pekutanga kana mucheche achichecheudzwa. Zvimwe zvinetso zvekubuda ropa zvinowanzo kuratidza apo mucheche anotanga kukambaira nekufamba.

Mhosva nyoro dzinogona kuenda dzisingacherechedzwe kusvika gare gare muhupenyu. Zviratidzo zvinogona kutanga kuitika mushure mekuvhiyiwa kana kukuvara. Kubuda ropa mukati kunogona kuitika chero kupi.

Zviratidzo zvinogona kusanganisira:

• Ropa muzvibatanidzwa pamwe kwakabatana marwadzo uye kuzvimba
• Ropa mune weti kana chigaro
• Kutuka
• Dumbu remudumbu uye weti nzira yekubuda ropa
• Mhino dzemhino
• Kubuda ropa kwenguva yakareba kubva mukucheka, kubviswa kwezino, uye kuvhiyiwa
• Kubuda ropa kunotanga pasina chikonzero

Kana iwe uriwe wekutanga mumhuri kuve nekufungidzirwa kubuda ropa kusagadzikana, wako mupi wezvehutano anopa kuraira kwakateedzana bvunzo dzinodaidzwa kuti coagulation kudzidza. Kana iko kukanganisika kwave kuzivikanwa, vamwe vanhu mumhuri yako vachada kuyedzwa kuti vaone kusagadzikana.

Miedzo yekuongorora hemophilia B inosanganisira:

• Yakasarudzika thromboplastin nguva (PTT)
• Prothrombin nguva
• Nguva yekubuda ropa
• Chikamu cheFibrinogen
• Serum factor IX chiitiko

Kurapa kunosanganisira kutsiva chisina kuvharika. Iwe unozogashira chinhu IX chinotarisisa. Zvakawanda sei zvaunowana zvinoenderana ne:

• Kukura kwekubuda ropa
• Nzvimbo yekubuda ropa
• Kurema nekureba kwako

Kudzivirira dambudziko rekubuda ropa, vanhu vane hemophilia uye mhuri dzavo vanogona kudzidziswa kupa chinhu IX chinotarisisa kumba pazviratidzo zvekutanga zvekubuda ropa. Vanhu vane mafomu akakomba echirwere vangangoda infusions yenguva dzose, yekudzivirira.

Kana iwe uine hemophilia yakakomba, iwe ungangodawo kutora chinhu cheXX chekutarisa usati wavhiyiwa kana mamwe marudzi emabasa emazino.

Iwe unofanirwa kuwana mushonga we hepatitis B. Vanhu vane hemophilia vanowanzoita hepatitis B nekuti vanogona kugamuchira zvigadzirwa zveropa.

Vamwe vanhu vane hemophilia B vanogadzira maantibhakitiriya ekuisa IX. Ma antibodies aya anonzi inhibitors. Iyo inhibitors inorwisa chinhu IX kuitira kuti isashande. Mumamiriro ezvinhu akadaro, inogadzirwa nemurume inogwamba inonzi VIIa inogona kupihwa.

Unogona kudzikisira kushushikana kwehurwere nekujoinha hemophilia rutsigiro boka. Kugovana nevamwe vane zvakajairika zviitiko uye matambudziko zvinogona kukubatsira kuti usazonzwa kusurukirwa.

Nokurapwa, vanhu vazhinji vane hemophilia B vanokwanisa kurarama hupenyu hwakajairika.

Kana iwe uine hemophilia B, iwe unofanirwa kugara uchiongororwa pamwe nehematologist.

Zvinetso zvinogona kusanganisira:

• Matambudziko akareba-kwenguva refu, ayo anogona kuda kutsiva kwemajoini
• Kubuda ropa muuropi (intracerebral hemorrhage)
• Thrombosis nekuda kwekurapwa

Fonera mupiki wako kana:

• Zviratidzo zvekubuda ropa kusagadzikana zvinokura
• Mumwe wemhuri akaonekwa aine hemophilia B
• Kana uine hemophilia B, uye waronga kuita vana; kurairwa kwemajini kunowanikwa

Genetic counselling inogona kukurudzirwa. Kuedzwa kunogona kuziva vakadzi nevasikana vanotakura hemophilia gene.

Kuedzwa kunogona kuitwa panguva yekuzvitakura pamwana ari mudumbu raamai.

Chirwere cheKisimusi; Chinhu IX hemophilia; Kubuda ropa kusagadzikana - hemophilia B

• X-yakabatanidzwa yakawandisa majini kusakwana - mashandisiro anoita vakomana
• X-inosungirirwa yakawandisa yekuremara kusakwana - sei vasikana vanokanganiswa
• X-yakabatanidzwa yakawandisa majini kukanganisa
• Masero eropa
• Ropa rinovhara

Carcao M, Moorehead P, Lillicrap D.Hemophilia A uye B. Muna: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Misimboti uye Maitiro. 7th ed. Philadelphia, PA: Elsevier; 2018: chits. 135.

Scott JP, Mafashama VH. Hereditary clotting factor kushomeka (kubuda ropa kusagadzikana). Mune: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Chinyorwa chePediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020: chitsiko 503.