Hemophilia A

Hemophilia A chirwere chekubuda nhaka chinokonzerwa nekushaikwa kweropa rinovhara ropa VIII. Pasina kukwana chinhu VIII, 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 kugwamba, zvinhu. Unogona kuve nemukana wakakwirira wekubuda ropa zvakawandisa kana chimwe kana zvimwe zvezvinhu izvi zvisipo kana zvisiri kushanda sezvavanofanira.

Chinhu VIII (sere) chimwe chakadaro coagulation chinhu. Hemophilia A mhedzisiro yemuviri usingaite zvakakwana chinhu VIII.

Hemophilia A inokonzereswa neyakagamuchirwa X-yakabatana yekudzokorora hunhu, iine chakaremara gene iri pane X chromosome. Vakadzi vane makopi maviri eX chromosome. Saka kana chinhu VIII geni pane imwe chromosome ikasashanda, iyo geni pane imwe chromosome inogona kuita basa rekugadzira zvakakwana chinhu VIII.

Vanhurume vane imwe chete X chromosome. Kana iyo factor VIII geni ikashaikwa pane X yechromosome yemukomana, achave aine hemophilia A. Nechikonzero ichi, vanhu vazhinji vane hemophilia A varume.

Kana mukadzi aine chakaremara chinokonzera VIII gene, anoonekwa seanotakura. Izvi zvinoreva kuti rakakanganisika geni rinogona kupfuudzwa pasi kuvana vake. Vakomana vanozvarwa nevakadzi vakadai vane mukana we50% wekuve nehemophilia A. Vanasikana vavo vane mukana we50% wekutakura. Vose vana vechikadzi vevanhu vane hemophilia vanotakura rakaremara jeni. Njodzi dzinokonzera hemophilia A dzinosanganisira:

• Nhoroondo yemhuri yekubuda ropa
• Kuva murume

Kukura kwezviratidzo kunosiyana. 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.

Kuyedza kuongorora hemophilia A kunosanganisira:

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

Kurapa kunosanganisira kutsiva chisina kuvharika. Iwe unogashira chinhu VIII chinotarisisa. Zvakawanda sei zvaunowana zvinoenderana ne:

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

Hunyoro hemophilia inogona kurapwa ne desmopressin (DDAVP). Mushonga uyu unobatsira muviri kusunungura chinhu VIII icho chakachengetwa mukati memukati memidzi yeropa.

Kudzivirira dambudziko rekubuda ropa, vanhu vane hemophilia nemhuri dzavo vanogona kudzidziswa kupa chinhu VIII chinotarisisa kumba pazviratidzo zvekutanga zvekubuda ropa. Vanhu vane mafomu akakomba echirwere vangangoda kurapwa kwekudzivirira.

DDAVP kana factor VIII concentrate inogona zvakare kudikanwa usati wave nekuburitsa mazino kana kuvhiya.

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

Vamwe vanhu vane hemophilia A vanogadzira antibodies kune factor VIII. Ma antibodies aya anonzi inhibitors. Iyo inhibitors inorwisa chinhu VIII 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 A vanokwanisa kurarama hupenyu hwakajairika.

Kana iwe uine hemophilia A, iwe unofanirwa kugara uchiongororwa nahematologist.

Zvinetso zvinogona kusanganisira:

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

Fonera mupiki wako kana:

• Zviratidzo zvechirwere chekubuda ropa zvinokura
• Mumwe wemhuri akawanikwa aine hemophilia A
• Une hemophilia A uye waronga kuita vana; kurairwa kwemajini kunowanikwa

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

Kuedzwa kunogona kuitwa panguva yekuzvitakura pamwana mudumbu raamai.

Factor VIII kushomeka; Classic hemophilia; Kubuda ropa kusagadzikana - hemophilia A

• 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.