Ese uvunika byoroshye? Ese ntuva amaraso byoroshye? Reka tuvuge kuri Glanzmann Thrombasthenia (GT) !

Ese wowe cyangwa umwana wawe mufata igihe kirekire kugira ngo muhagarike kuva amaraso nubwo byaba bivuye ku gukomereka gato? Cyangwa se mufite udusebe tw'ubururu gusa (ibisebe) ku mubiri wanyu wose? Hari igihe ibi bisa n'ibisanzwe, ariko hari igihe bishobora kuba ikintu cyo guhangayikishwa. Iyo ni yo ndwara tugiye kuvugaho uyu munsi, yitwa Glanzmann Thrombasthenia (GT). Ntugahangayike, tuzakomeza koroshya ibintu byose.

Glanzmann Thrombasthenia (GT) ni iki?

Muri make, Glanzmann Thrombasthenia (GT) ni indwara imara igihe kirekire (ni ukuvuga ubuzima bwose) ituma ukomereka byoroshye kandi bikagorana guhagarika kuva amaraso. Impamvu nyamukuru ni ikibazo cya platelets mu maraso yacu, uturemangingo duto dufasha kubumba amaraso.

Tekereza, niba ufite GT, bitewe n'impinduka mu miterere y'uturemangingo mu mubiri wawe, izi platelets ntizikora poroteyine y'ingenzi ikenewe kugira ngo amaraso avure. Kubera iyo mpamvu, kuvura amaraso bigenda buhoro cyane. Ingaruka ni uko uva amaraso menshi. Ingano y'uku kuva amaraso ishobora gutandukana bitewe n'umuntu ku wundi. Kuri bamwe, uku kuva amaraso guke gushobora gukemurwa mu rugo. Ku bandi , bishobora kuba bikomeye bihagije ku buryo bisaba ubuvuzi bwihutirwa .

Glanzmann Thrombasthenia (GT) ikunze kugaragara gute?

Mu by’ukuri indwara ya Glanzmann Thrombasthenia (GT) ni indwara ikunze kugaragara cyane. Nk’uko abahanga mu by’ubuvuzi babivuga, umuntu umwe gusa kuri miliyoni imwe ku isi ni we uvukana iyi ndwara.

Ariko, mu miryango imwe n'imwe aho ubu buhinduzi bw'uturemangingo buhererekanwa kuva ku gisekuru kugeza ku kindi, uyu mubare uri hejuru gato. Ni ukuvuga ko muri iyo miryango, umuntu umwe ku bantu ibihumbi magana abiri ashobora kubona iyi ndwara. Bivugwa ko umubare w'abana bavukanye iyi ndwara ya GT uri hejuru cyane mu bihugu bimwe na bimwe byo mu Burasirazuba bwo Hagati, mu ntara za Newfoundland na Labrador muri Kanada, no mu muryango w'aba Romani mu Bufaransa.

Ni ibihe bimenyetso bya Glanzmann Thrombasthenia (GT)?

Niba ufite GT, ushobora kuva amaraso menshi kurusha uko undi muntu yayava iyo bahuye n'imvune imwe. Cyangwa ushobora gutangira kuva amaraso mu buryo butunguranye kandi nta mpamvu igaragara.

Ibimenyetso by'ingenzi bya Glanzmann Thrombasthenia (GT) ni ibi bikurikira:

• Gukomeretsa byoroshye cyane: Ndetse n'igisebe gito gishobora gutera igisebe kinini.
• Ishinya riva amaraso: Ishinya ryawe rishobora kuva amaraso byoroshye iyo wogeje amenyo yawe.
• Kuva amaraso kenshi mu mazuru (epistaxis): Hari abantu bashobora kuva amaraso mu mazuru inshuro nyinshi mu cyumweru, ndetse bahagaze gusa.
• Uduheri cyangwa uduce tw'umuhengeri ku ruhu (purpura): Ibi bitandukanye n'uduheri dusanzwe kandi bishobora kuba uduheri tunini.
• Utudomo duto tw'umuhengeri, umukara, cyangwa umutuku ku ruhu (petechiae): Utu ni utuntu duto dusa n'aho twakubiswe n'agapira.
• Menorrhagia: Kuva amaraso menshi mu gihe cy'imihango ku bagore: Kuva amaraso menshi cyane kurusha uko bisanzwe.

Muri GT, kuva amaraso imbere mu mubiri ntibikunze kugaragara cyane nko kuva amaraso mu gisebe cy'uruhu (nk'igikomere) cyangwa mu rurenda (nk'imbere mu mazuru cyangwa mu kanwa).

Kuki Glanzmann Thrombasthenia (GT) ibaho? Ni izihe mpamvu?

Abana bavutse bafite Glanzmann Thrombasthenia (GT) bavukana inenge, cyangwa mutation , muri gene ikora poroteyine yitwa integrin alpha IIb / beta 3, ifasha platelets z'amaraso kuziba. Kugira ngo umwana agire GT, agomba kuragwa gene ifite inenge ivuye kuri nyina na se. Ibi byitwa autosomal recessive inheritance .

Akenshi, ababyeyi ntibazi ko ari bo batwara iyi gene ifite inenge. Ibi biterwa nuko hakenewe gene ebyiri zifite inenge kugira ngo hagaragare ibimenyetso. Uyitwara ni umuntu ufite gene imwe isanzwe n'indi ifite inenge.

Niba ababyeyi bombi ari bo banduye iyi ndwara, bafite amahirwe angana na 25% yo kubyara umwana ufite GT.

Nyuma Glanzmann Thrombasthenia (GT) itangira

Hari abantu bashobora kurwara indwara ya GT mu gihe cy'imyaka myinshi mu buzima. Ibi ni gake cyane. Nubwo bishobora kubaho, abahanga mu by'ubuvuzi baracyashyira Glanzmann Thrombasthenia (GT) mu cyiciro cy'indwara yo kuva amaraso mu muryango .

Uku ni ko GT ikura nyuma, iyo umubiri wawe ukora ubudahangarwa bw’umubiri burwanya poroteyine ya integrin alpha IIb/beta 3 yavuzwe haruguru. Indwara zimwe na zimwe, ndetse n’imiti imwe n’imwe, zishobora kubitera. Ariko ingaruka ni zimwe. Kubera ko nta poroteyine ikora ihagije, bifata igihe kirekire kugira ngo platelets zawe zipfunde.

Ni izihe ngorane zishobora guterwa na Glanzmann Thrombasthenia (GT)?

Abagore bashobora kugira ikibazo cyo kubura icyuma bitewe no kuva amaraso menshi mu gihe cy'imihango. Mu bihe bikomeye, kuva amaraso menshi mu nda (GT) bishobora gutera kuva amaraso menshi mu kaga. Ibi bishobora kuba byinshi cyane cyane mu gihe cyo kuva amaraso menshi , nko kubyara cyangwa kubagwa.

Ariko ntugahangayike, niba urwaye indwara ya GT, muganga wawe ashobora gufata ingamba zikenewe kugira ngo wirinde izi ngorane.

Wamenya ute neza ko ufite Glanzmann Thrombasthenia (GT)? (Isuzuma)

Abaganga bakoresha ibizamini bitandukanye kugira ngo bapime indwara ya GT. Ikunze gupimwa mu bwana. Nk'umubyeyi, niba umwana wawe akomereka byoroshye, akava amaraso kenshi mu mazuru, cyangwa bigafata igihe kirekire kugira ngo amaraso ahagarike, ushobora kujyana umwana wawe kwa muganga. Rimwe na rimwe, mu bihe by'ingenzi mu buzima bw'umwana, urugero:

• Iyo ukeba umwana.
• Ubwo iryinyo rya mbere ry'uruhinja rivamo.
• Iyo ari umukobwa, ni igihe ageze mu mihango ye ya mbere (gucura).

Muri iki gihe, ushobora kubona amaraso menshi cyangwa adashira. Abantu barenga 80% barwaye Glanzmann Thrombasthenia (GT) basuzumwa mbere y'imyaka 14, kandi benshi basuzumwa mbere y'imyaka 5. Abantu basuzumwa nyuma y'imyaka 5 bashobora kutamenya ko bayirwaye kugeza igihe bakomeretse cyangwa bagiriye impanuka ikomeye.

Ni ibihe bizamini biri gukorwa kuri ibi?

Abaganga bakoresha ibizamini by'amaraso kugira ngo bapime Glanzmann Thrombasthenia (GT). Ibi bizamini bishobora kugaragaza ibibazo kuri:

• Ibipimo by'amaraso yawe: Ikizamini cy'umubare wuzuye w'amaraso (CBC) gishobora gusuzuma niba umubare w'amaraso yawe ari mu buryo busanzwe. Isuzuma ry'amaraso ryo mu gice cy'imbere rishobora kandi gusuzuma niba asa n'aho adasanzwe.
• Uko amaraso yawe yapfunditse neza: Ibizamini byinshi, nko gusuzuma igihe cya Prothrombin (PT) n'ikizamini cya Partial Thromboplastin (PTT), bishobora gupima uburyo amaraso yawe yapfunditse vuba kandi neza. Ibi bishobora kandi gufasha gukuraho indwara nyinshi zikunze kugaragara zo kuva amaraso zisa n'iza GT (urugero: indwara ya von Willebrand, indwara ya Bernard-Soulier).
• Integrin alpha IIb/beta3: Ibizamini nka flow cytometry na monoclonal antibody panels bishobora kugaragaza niba ufite ikibazo cyangwa inenge muri iyi poroteyine. Bishobora kandi kumenya niba ufite antibody zirwanya iyi poroteyine.
• Ingirabuzimafatizo zawe: Ibipimo bya genetiki bishobora kwemeza niba ufite impinduka mu ngirabuzimafatizo (genes zitwa ITGA2B na ITGB3) zitera GT.

Ni ubuhe buryo bwo kuvura Glanzmann Thrombasthenia (GT)?

Niba urwaye indwara ya GT, muganga wawe azakugira inama y'uburyo wagabanya ibyago byo kuva amaraso. Bishoboka ko uzakenera gukorana n'inzobere mu by'amaraso . Uretse ibintu ushobora gukora kugira ngo wirinde kuva amaraso, uburyo bwo kuvura buzaterwa n'uburemere bw'amaraso yawe.

Ku kuva amaraso make kugeza ku rugero

Uburyo bwo kuvura bene ibi bibazo ni ubu:

• Gukanda: Muganga wawe azakwigisha uburyo bwo gushyira igitutu mu mazuru kugira ngo ugabanye kuva amaraso mu gisebe no guhagarika kuva amaraso mu mazuru. Ku mazuru afite amaraso menshi, muganga wawe ashobora gushyira ikintu nk'agatambaro cyangwa ifuro mu mazuru yawe kugira ngo ahagarike kuva amaraso mu mazuru (gupakira izuru).
• Imiti: Ushobora gukenera imiti nka fibrin sealant (ikintu nk'umuti wo mu maraso), gelatin foam, topical thrombin, n'imiti igabanya ubukana bw'amaraso kugira ngo ifashe amaraso kubumba.

Ku kuva amaraso menshi

Kuva amaraso menshi guterwa na Glanzmann Thrombasthenia (GT) bisaba ubuvuzi bwihutirwa . Ubuvuzi burimo:

• Guterwa plaquettes: Ushobora gukenera guhabwa plaquettes kugira ngo uhagarike kuva amaraso menshi, cyangwa kugira ngo wirinde gutakaza amaraso mbere yo kubagwa cyangwa kubyara. Ibi bikubiyemo kuguha plaquettes ziturutse ku muntu muzima utanga.
• Guterwa uturemangingo dutukura: Niba waramaze gutakaza amaraso menshi, ushobora no guhabwa uturemangingo dutukura kugira ngo twongere ayo maraso.
• Recombinant coagulation factor VIIa (rFVIIa): Uyu muti ushobora kuba ukenewe niba udakwiriye guterwa platelets.
• Gutera uturemangingo tw’amaraso: Gutera uturemangingo tw’amaraso ni bwo buryo bwonyine bushobora kuvura GT. Ubu bushobora kuba amahitamo ku ndwara zikomeye za GT zidashobora kugenzurwa n’ubundi buryo. Muri ibi, uturemangingo tw’amaraso twavuye ku muntu utanga uturemangingo dushyirwa mu mubiri wawe.

Ese hari ingorane ziri mu kuvura?

Yego rwose. Hagati ya 20% na 30% by'abantu bahabwa platelets bakuramo antibody zo kuzirwanya uko igihe kigenda gihita. Ibi bivuze ko platelets nshya bahabwa zitazakora. Ibyo nibibaho, uzagomba kujya mu bundi buryo bwo kuvurwa, nko guterwa inshinge za rFVIIa. Ariko uwo muti ufite n'ingaruka zimwe na zimwe. Urugero, ushobora gutuma amaraso yawe yibumba cyane.

Nubwo gutera uturemangingo tw’inyuma bishobora kuvura Glanzmann Thrombasthenia (GT), kubona uturemangingo tw’inyuma bishobora kuba ikibazo. Mu gutera uturemangingo tw’inyuma kuri GT, abatera uturemangingo akenshi baba ari abavandimwe. Nubwo habonetse uturemangingo tw’inyuma, hari ibyago by’uko umubiri wawe wakwanga uturemangingo tw’inyuma. Iyi ndwara yitwa Graft versus Host Disease (GvHD) .

Iyo umuganga wawe akubwiye uburyo bwo kuvura, azagusobanurira ingaruka n'inyungu mbere y'igihe.

Ni ubuhe buryo bw'ejo hazaza umuntu ufite GT ashobora kwitega?

Nta bantu babiri barwaye Glanzmann Thrombasthenia (GT), nubwo baba bakomoka mu muryango umwe kandi bafite imiterere imwe y’uturemangingo, bahura n’iyi ndwara mu buryo butandukanye. Uko amaraso akura n’icyo ugomba gukora kugira ngo wicunge bizaterwa n’uko indwara yawe imeze.

Inkuru nziza ni uko, iyo habayeho ubuvuzi bwiza, abantu benshi barwaye Glanzmann Thrombasthenia (GT) babaho ubuzima busanzwe kandi bamara igihe kirekire. Hari ubwo ingano y'amaraso ava ishobora kugabanuka uko bagenda basaza.

Nakora iki kugira ngo mbeho neza mfite GT? (Nigute nakwiyitaho)

Niba ufite GT, korana na muganga wawe kugira ngo ugabanye ibyago byo kuva amaraso no kubura amaraso menshi. Ugomba gukora ibi bikurikira:

• Menya neza imiti udakwiye gufata.Irinde rwose imiti igabanya amaraso (nka aspirine n'indi miti igabanya ubukana bw'amaraso) n'indi miti muganga wawe yakubwiye ko utagomba gufata.
• Wite ku buzima bwawe bw'amenyo. Koza amenyo yawe buri munsi, no kujya kwa muganga w'amenyo buri gihe bishobora kugabanya ibyago byo kuva amaraso mu ishinya.
• Wite ku mazuru yawe neza. Gukomeza kugira ubushuhe imbere mu mazuru bishobora kugabanya ibyago byo kuva amaraso mu mazuru. Ushobora gukoresha icyuma gifasha mu mazuru, ugakoresha spray yo mu mazuru, cyangwa ugasiga Vaseline mu mazuru yawe kugira ngo wirinde kuma.
• Gufata neza kuva amaraso menshi mu gihe cy'imihango. Gukoresha uburyo bwo kuboneza urubyaro bugizwe n'imisemburo bishobora gufasha kwirinda imihango myinshi. Vugana n'umuganga w'abagore kuri ibi.
• Ambara umukufi wo kumenyesha muganga. Buri gihe ujye witwaza ikarita y'indangamuntu kugira ngo ubashe kumenya ko ufite indwara yo kuva amaraso mu gihe cy'impanuka. Bishobora kurokora ubuzima bwawe.
• Gira gahunda y'icyo wakora mu gihe ugize ikibazo cyo kuva amaraso. Menya igihe cyo kuvura uri mu rugo n'igihe cyo kubonana na muganga ako kanya. Menya uwo wavugana n'aho wajya. Muri ubwo buryo, ntibizatinda kubona ubuvuzi.

Ni ryari ngomba kujya kwa muganga?

Niba wowe cyangwa umwana wawe mugaragaza ibimenyetso byo kuva amaraso byoroshye, gana muganga. Gukomeretsa no kuva amaraso mu mazuru ni ibisanzwe. Ariko niba ibi bimenyetso bikunze kugaragara, cyangwa niba kuva amaraso bitinze - gana muganga nta kabuza.

Ni ryari ugomba kujya mu Kigo cyita ku Buvuzi bw’Ingoboka (ETU) ?

Niba ufite amaraso udashobora guhagarika, harimo no kuva amaraso menshi mu gihe cy'imihango, jya mu biro byita ku barwayi ba ETU (ETU) utabanje gufata gahunda yo kubonana na muganga. Ibimenyetso ugomba kwitondera birimo:

• Kuva amaraso mu mazuru bidahagarara mu gihe cy'isaha imwe.
• Gutemba cyane mu mihango bimara amasaha abiri cyangwa arenga, bikavoma tampons ebyiri cyangwa pads ebyiri mu isaha cyangwa zirenga.

Ibibazo by'ingenzi ugomba kubaza muganga wawe

Ntiwibagirwe kubaza ibibazo nk'ibi:

• Ni ibihe bipimo bikwiye gukorwa kugira ngo hamenyekane uburwayi bwanjye?
• Ni ubuhe bwoko bw'ubuvuzi nkeneye?
• Ni izihe mpinduka mu mibereho yanjye nshobora gukora kugira ngo menye uko merewe?
• Ni amahirwe angahe ko umwana wanjye azaragwa iyi ndwara ya GT?
• Ese murangira inama yo gupima imiterere y'umubiri niba ntekereza gutwita?

Ese umubare w'imitsi itera amaraso mu maraso (platelets) usanzwe ku bantu bafite GT?

Umubare w'imitsi itera amaraso mu maraso (platelets) ubusanzwe uba usanzwe muri Glanzmann Thrombasthenia (GT).Ikibazo si umubare w'udukoko twa platelets. Ahubwo ni ikibazo cya poroteyine ifasha udukoko twa platelets gufatana, bigatuma udukoko twa platelets tudakora neza.

Ni irihe tandukaniro riri hagati ya Glanzmann Thrombasthenia (GT) na Bernard-Soulier syndrome (BSS)?

Glanzmann Thrombasthenia (GT) na Bernard-Soulier syndrome (BSS) byombi ni indwara zidasanzwe ziterwa na gene zigira ingaruka ku buryo amaraso yawe yibumba. Ariko ziterwa n'impinduka zitandukanye mu ngirabuzimafatizo. Ibibazo byihariye ku miterere y'amaraso nabyo biratandukanye. Abantu barwaye BSS bashobora kugira umubare muto wa platelets ndetse n'umubare munini udasanzwe wa platelets.

Ibintu by'ingenzi cyane byo kwibuka mu byo twaganiriyeho (Ubutumwa bwo kujyana mu rugo)

Glanzmann Thrombasthenia (GT) ni indwara y’ubuzima bwawe bwose, wowe n’umuganga wawe w’amaraso mugomba gukurikirana neza. Kuri GT, nta buryo bwo kumenya ubukana bw’amaraso yawe (cyangwa mato) buzaba bukabije. Ariko, gufata ingamba zo kugabanya amaraso yawe bishobora kugufasha kugira ubuzima bwiza. Niba GT yawe ikomeye, uburyo bwo kuvura nko guterwa platelets nabwo bushobora gufasha.

Nubwo udashobora kugenzura ko wavukanye iyi ndwara, ibuka ko ufite amahitamo yo kuyirwanya mu buryo butabangamira ubuzima bwawe. Ntugatinye gushaka inama kwa muganga no kwiyitaho. Icyo gihe ushobora kubaho neza n'iyi ndwara.