Kodi mumavulala mosavuta? Kodi simutuluka magazi mosavuta? Tiyeni tikambirane za Glanzmann Thrombasthenia (GT)!

Kodi inu kapena mwana wanu mumatenga nthawi yayitali kuti musiye kutuluka magazi ngakhale mutavulala pang'ono? Kapena mumangokhala ndi mabala abuluu (mabala) thupi lonse? Nthawi zina izi zimaoneka zachilendo, koma nthawi zina zimakhala zodetsa nkhawa. Umenewo ndi vuto lomwe tikambirane lero, lotchedwa Glanzmann Thrombasthenia (GT). Musadandaule, tidzasunga zonse mosavuta.

Kodi Glanzmann Thrombasthenia (GT) ndi chiyani?

Mwachidule, Glanzmann Thrombasthenia (GT) ndi vuto la nthawi yayitali (kutanthauza moyo wonse) lomwe limapangitsa kuti muzitha kuvulala mosavuta komanso kukhala ndi vuto loletsa kutuluka magazi. Chifukwa chachikulu ndi vuto la ma platelet m'magazi athu, maselo ang'onoang'ono omwe amathandiza magazi kuundana.

Tangoganizirani, ngati muli ndi GT, chifukwa cha kusintha kwa majini m'thupi lanu, ma platelet awa sapanga puloteni yofunika kwambiri kuti magazi aziundana. Chifukwa cha izi, magazi amaundana pang'onopang'ono. Zotsatira zake n'zakuti mumatuluka magazi ambiri. Kuchuluka kwa magazi amenewa kumatha kusiyana pakati pa munthu ndi munthu. Kwa anthu ena, izi zitha kukhala magazi ochepa omwe angathe kuthandizidwa kunyumba. Kwa ena , zitha kukhala zovuta kwambiri moti zimafunika chithandizo chadzidzidzi .

Kodi Glanzmann Thrombasthenia (GT) ndi yofala bwanji?

Matenda a Glanzmann Thrombasthenia (GT) ndi matenda osowa kwambiri. Malinga ndi akatswiri azachipatala, munthu m'modzi yekha pa anthu miliyoni padziko lonse lapansi amabadwa ndi matendawa.

Komabe, m'madera ena kumene kusiyana kwa majini kumeneku kumadutsa kuchokera ku mibadwomibadwo kupita ku mibadwomibadwo, chiwerengerochi n'chokwera pang'ono. Izi zikutanthauza kuti, m'madera otere, pafupifupi munthu m'modzi pa anthu mazana awiri aliwonse amatha kuona vutoli. Zanenedwa kuti chiwerengero cha makanda obadwa ndi vutoli la GT ndi chachikulu kwambiri m'maiko ena ku Middle East, m'zigawo za Newfoundland ndi Labrador ku Canada, komanso pakati pa anthu a ku Romani ku France.

Kodi zizindikiro za Glanzmann Thrombasthenia (GT) ndi ziti?

Ngati muli ndi GT, mungatulutse magazi ambiri kuposa ena ngati wina angatulutsenso magazi omwewo. Kapena mungayambe kutuluka magazi mwadzidzidzi popanda chifukwa chomveka.

Zizindikiro zazikulu za Glanzmann Thrombasthenia (GT) ndi izi:

• Kutupa mosavuta: Ngakhale chiphuphu chaching'ono chingayambitse kuvulala kwakukulu.
• Mkamwa umatuluka magazi: Mkamwa mwanu mumatha kutuluka magazi mosavuta mukatsuka mano anu.
• Kutuluka magazi m'mphuno pafupipafupi (epistaxis): Anthu ena amatha kutuluka magazi m'mphuno kangapo pa sabata, ngakhale atangoyimirira.
• Madontho ofiirira kapena mabala pakhungu (purpura): Izi ndi zosiyana ndi mabala wamba ndipo zimatha kukhala madontho akuluakulu.
• Madontho ang'onoang'ono ofiirira, ofiirira, kapena ofiira pakhungu (petechiae): Awa ndi madontho ang'onoang'ono omwe amawoneka ngati abayidwa ndi pini.
• Kusamba kwa msambo: Kutuluka magazi ambiri panthawi ya msambo mwa akazi: Kutuluka magazi ochuluka kwambiri kuposa masiku onse.

Mu GT, kutuluka magazi mkati mwa thupi sikofala kwambiri monga kutuluka magazi kuchokera pachilonda cha pakhungu (monga kudulidwa) kapena nembanemba ya mucous (monga mkati mwa mphuno kapena pakamwa).

N’chifukwa chiyani Glanzmann Thrombasthenia (GT) imachitika? Kodi zimayambitsa chiyani?

Makanda obadwa ndi Glanzmann Thrombasthenia (GT) amalandira chilema, kapena kusintha kwa majini , mu jini yomwe imapanga puloteni yotchedwa integrin alpha IIb/beta 3, yomwe imathandiza kuti ma platelet a magazi aziundana. Kuti mwana akhale ndi GT, ayenera kulandira jini yolakwikayo kuchokera kwa amayi ndi abambo ake. Izi zimatchedwa autosomal recessive inheritance .

Kawirikawiri, makolo sadziwa kuti ndi omwe amatenga jini lolakwikali. Izi zili choncho chifukwa majini awiri olakwika amafunika kuti asonyeze zizindikiro. Wonyamula ndi munthu amene ali ndi jini imodzi yabwinobwino ndi jini imodzi yolakwika.

Ngati makolo onse awiri ali ndi kachilombo ka GT, ali ndi mwayi wa 25% wokhala ndi mwana yemwe ali ndi kachilomboka.

Kuyamba kwa Glanzmann Thrombasthenia (GT) pambuyo pake

Anthu ena amatha kudwala matenda a shuga m'magazi akakula. Izi sizichitika kawirikawiri. Ngakhale kuti zingachitike, akatswiri azachipatala amaikabe Glanzmann Thrombasthenia (GT) ngati matenda obadwa nawo otuluka magazi .

Umu ndi momwe GT imakulira pambuyo pake, pamene thupi lanu limapanga ma antibodies motsutsana ndi puloteni ya integrin alpha IIb/beta 3 yomwe yatchulidwa pamwambapa. Matenda ena, ngakhale mankhwala ena, angayambitse izi. Koma zotsatira zake ndi zomwezo. Chifukwa chakuti puloteni yogwira ntchito siikwanira, zimatenga nthawi yayitali kuti ma platelet anu azitha kuundana.

Kodi ndi mavuto ati omwe angakhalepo chifukwa cha Glanzmann Thrombasthenia (GT)?

Azimayi amatha kukhala ndi vuto la kuchepa kwa magazi chifukwa cha kusowa kwa ayoni chifukwa cha kusamba kwambiri. Pa milandu yoopsa, GT ingayambitse kutuluka magazi kwambiri komanso koopsa (kutuluka magazi). Chiwopsezochi chimakhala chachikulu makamaka nthawi ya kutuluka magazi kwambiri , monga kubereka kapena opaleshoni.

Koma musadandaule, ngati mwapezeka ndi matenda a GT, dokotala wanu angachitepo kanthu kuti apewe mavutowa.

Kodi mungadziwe bwanji ngati muli ndi Glanzmann Thrombasthenia (GT)? (Kuzindikira)

Madokotala amagwiritsa ntchito mayeso osiyanasiyana kuti adziwe matenda a GT. Nthawi zambiri amapezeka ali mwana. Monga kholo, ngati mwana wanu amavulala mosavuta, amatuluka magazi m'mphuno pafupipafupi, kapena zimatenga nthawi yayitali kuti magazi asiye kutuluka, mungafune kupita ndi mwana wanu kwa dokotala. Nthawi zina, panthawi zofunika kwambiri pamoyo wa mwana, mwachitsanzo:

• Podula mwana.
• Dzino loyamba la mwana likatuluka.
• Ngati ndi mtsikana, ndiye kuti wafika nthawi yake yoyamba ya msambo (kusamba).

Panthawiyi, mungazindikire kutuluka magazi kwambiri kapena kosalekeza. Anthu opitilira 80% omwe ali ndi Glanzmann Thrombasthenia (GT) amapezeka asanakwanitse zaka 14, ndipo ambiri amapezeka asanakwanitse zaka 5. Anthu omwe amapezeka ndi GT ali akuluakulu sangadziwe kuti ali nayo mpaka atavulala kwambiri kapena atachita ngozi.

Ndi mayeso ati omwe akuchitidwa pa izi?

Madokotala amagwiritsa ntchito mayeso a magazi kuti adziwe matenda a Glanzmann Thrombasthenia (GT). Mayesowa angasonyeze mavuto ndi:

• Ma platelet anu: Kuyezetsa magazi konse (CBC) kungawone ngati kuchuluka kwa ma platelet anu kuli bwino. Kuyezetsa magazi m'thupi lanu kungawonenso ngati akuoneka osazolowereka.
• Kuchuluka kwa magazi anu oundana: Mayeso angapo, monga mayeso a Prothrombin Time (PT) ndi mayeso a Partial Thromboplastin Time (PTT), amatha kuyeza kuchuluka kwa magazi anu oundana mwachangu komanso moyenera. Izi zingathandizenso kuchotsa matenda ofala kwambiri otuluka magazi omwe ali ndi zizindikiro zofanana ndi za GT (monga matenda a von Willebrand, matenda a Bernard-Soulier).
• Integrin alpha IIb/beta3: Mayeso monga flow cytometry ndi monoclonal antibody panels amatha kudziwa ngati muli ndi kusowa kapena chilema mu puloteni iyi. Angathenso kuzindikira ngati muli ndi ma antibodies omwe amaukira puloteni iyi.
• Majini anu: Mayeso a majini angatsimikizire ngati muli ndi kusintha kwa majini (majini otchedwa `ITGA2B` ndi `ITGB3`) omwe amayambitsa GT.

Kodi mankhwala a Glanzmann Thrombasthenia (GT) ndi ati?

Ngati mwapezeka ndi matenda a GT, dokotala wanu adzakupatsani malangizo a momwe mungachepetsere chiopsezo chanu chotaya magazi. Mwina mungafunike kugwira ntchito ndi dokotala wa matenda a magazi . Kupatula zinthu zomwe mungachite kuti mupewe kutuluka magazi, chithandizo chidzadalira kuopsa kwa kutuluka magazi kwanu.

Kutuluka magazi pang'ono mpaka pang'ono

Mankhwala a milandu yotereyi ndi awa:

• Kupsinjika: Dokotala wanu adzakuphunzitsani momwe mungagwiritsire ntchito mphamvu kuti muchepetse kutuluka magazi kuchokera pabala ndikuletsa kutuluka magazi pang'ono m'mphuno. Pa kutuluka magazi kwambiri m'mphuno, dokotala wanu angaike chinthu chonga gauze kapena thovu m'mphuno mwanu kuti muletse kutuluka magazi (kuyika m'mphuno).
• Mankhwala: Mungafunike mankhwala monga fibrin sealant (chinachake monga guluu wa magazi), thovu la gelatin, topical thrombin, ndi mankhwala oletsa fibrinolytic kuti magazi aziundana.

Kwa kutuluka magazi kwambiri

Kutuluka magazi kwambiri chifukwa cha Glanzmann Thrombasthenia (GT) kumafuna chithandizo chadzidzidzi . Mankhwalawa akuphatikizapo:

• Kuika magazi m'magazi: Mungafunike kupatsidwa ma platelet kuti muchepetse kutuluka magazi kwambiri, kapena kuti mupewe kutaya magazi musanachite opaleshoni yaikulu kapena kubereka mwana. Izi zikutanthauza kukupatsani ma platelet kuchokera kwa wopereka wathanzi.
• Kuika maselo ofiira a magazi: Ngati mwataya kale magazi ambiri, mungapatsidwenso maselo ofiira a magazi kuti muwonjezere kuchuluka kwa magaziwo.
• Recombinant coagulation factor VIIa (rFVIIa): Mankhwalawa angafunike ngati simuli oyenera kuikidwa magazi a platelet.
• Kusamutsa maselo oyambira a m'magazi: Kusamutsa maselo oyambira ndi njira yokhayo yochiritsira matenda a GT. Iyi ingakhale njira yabwino kwa odwala matenda aakulu a GT omwe sangathe kulamulidwa ndi njira zina. Pachifukwa ichi, maselo oyambira ochokera kwa wopereka amalowetsedwa m'thupi lanu.

Kodi pali zovuta zilizonse pa chithandizochi?

Inde, ndithudi. Pakati pa 20% ndi 30% ya anthu omwe amalandira ma platelet amapanga ma antibodies motsutsana nawo pakapita nthawi. Izi zikutanthauza kuti ma platelet atsopano omwe amalandira sagwira ntchito. Ngati zimenezo zitachitika, muyenera kupita ku chithandizo china, monga jakisoni wa rFVIIa. Koma mankhwalawa alinso ndi zoopsa zina. Mwachitsanzo, angayambitse magazi anu kuundana kwambiri.

Ngakhale kuti kuyika maselo oyambira kungachiritse Glanzmann Thrombasthenia (GT), kupeza wofanana ndi thupi lanu kungakhale kovuta. Pakuika maselo oyambira a GT, opereka nthawi zambiri amakhala abale ndi alongo. Ngakhale atapezeka wofanana, pali chiopsezo chakuti thupi lanu lidzakana maselo opereka. Vutoli limatchedwa Graft versus Host Disease (GvHD) .

Dokotala wanu akamalankhula nanu za njira zochiritsira, adzakufotokozerani zoopsa ndi ubwino wake pasadakhale.

Kodi munthu amene ali ndi GT angayembekezere tsogolo lotani?

Palibe anthu awiri omwe ali ndi Glanzmann Thrombasthenia (GT), ngakhale atakhala ochokera m'banja limodzi ndipo ali ndi majini ofanana, omwe amakumana ndi matendawa mosiyana. Kuchuluka kwa magazi omwe mumatuluka komanso zomwe muyenera kuchita kuti muthane ndi vutoli kumadalira vuto lanu.

Nkhani yabwino ndi yakuti, ndi chisamaliro choyenera, anthu ambiri omwe ali ndi Glanzmann Thrombasthenia (GT) amakhala ndi moyo wabwinobwino komanso moyo wabwinobwino. Nthawi zina, kuchuluka kwa magazi kumatha kuchepa akamakalamba.

Kodi ndingakhale bwanji bwino ndi GT? (Momwe ndingadzisamalire ndekha)

Ngati muli ndi matenda a shuga, gwirani ntchito ndi dokotala wanu kuti muchepetse chiopsezo chanu chotaya magazi komanso kuchepa kwa magazi m'thupi. Muyenera kuchita izi:

• Dziwani bwino mankhwala omwe simuyenera kumwa.Pewani kugwiritsa ntchito mankhwala ochepetsa magazi (monga aspirin ndi NSAID zina) ndi mankhwala ena omwe dokotala wanu wakuuzani kuti musamwe.
• Samalani bwino thanzi la mkamwa mwanu. Kutsuka mano anu ndi floss tsiku lililonse, komanso kuonana ndi dokotala wa mano nthawi zonse kungathandize kuchepetsa chiopsezo cha kutuluka magazi m'kamwa.
• Samalani mphuno yanu bwino. Kusunga mkati mwa mphuno yanu kukhala ndi chinyezi kungathandize kuchepetsa chiopsezo cha kutuluka magazi m'mphuno. Mutha kugwiritsa ntchito chonyowetsa mpweya, kugwiritsa ntchito mankhwala opopera m'mphuno, kapena kuyika Vaseline pang'ono m'mphuno mwanu kuti isaume.
• Kuchepetsa kutuluka magazi ambiri msambo. Kugwiritsa ntchito njira zolerera za mahomoni kungathandize kupewa kusamba kwambiri. Lankhulani ndi dokotala wanu za izi.
• Valani chibangili chochenjeza dokotala. Nthawi zonse nyamulani chiphaso chanu kuti mudziwe ngati muli ndi vuto la kutuluka magazi mwadzidzidzi. Zingapulumutse moyo wanu.
• Khalani ndi dongosolo la zomwe mungachite ngati muli ndi vuto la kutuluka magazi. Dziwani nthawi yoti muchiritse matendawa kunyumba komanso nthawi yoti mukaonane ndi dokotala nthawi yomweyo. Dziwani woti mulankhule naye komanso komwe mungapite. Mwanjira imeneyi, sikudzakhala kochedwa kupeza chithandizo.

Kodi ndiyenera kupita kwa dokotala liti?

Ngati inu kapena mwana wanu mukuwonetsa zizindikiro za kutuluka magazi mosavuta, pitani kwa dokotala. Kutupa ndi kutuluka magazi m'mphuno n'kofala. Koma ngati zizindikirozi zimachitika pafupipafupi, kapena ngati kutuluka magazi kumatenga nthawi yayitali kuti kuthe - pitani kwa dokotala.

Kodi muyenera kupita ku Emergency Treatment Unit (ETU) liti?

Ngati muli ndi magazi omwe simungathe kuwaletsa, kuphatikizapo kutuluka magazi ambiri kusamba, pitani mwachindunji ku dipatimenti yothandiza anthu mwadzidzidzi (ETU) popanda kupanga nthawi yoti mukakumane ndi dokotala. Zizindikiro zoti musamale nazo ndi izi:

• Kutuluka magazi m'mphuno komwe sikutha kwa ola limodzi.
• Kusamba kwambiri kumatenga maola awiri kapena kuposerapo, kunyowetsa ma tampons awiri kapena ma pedi awiri pa ola limodzi kapena kuposerapo.

Mafunso ofunikira oti mufunse dokotala wanu

Musaiwale kufunsa mafunso ngati awa:

• Ndi mayeso ati omwe ayenera kuchitidwa kuti adziwe vuto langa?
• Kodi ndi chithandizo chamtundu wanji chomwe ndikufunika?
• Ndi kusintha kotani kwa moyo komwe ndingachite kuti ndithane ndi vuto langa?
• Kodi pali mwayi wotani woti mwana wanga adzalandira matenda a GT?
• Kodi mumalimbikitsa kuyezetsa majini ngati ndikuganiza zotenga mimba?

Kodi kuchuluka kwa ma platelet mwa anthu omwe ali ndi GT ndikwabwinobwino?

Kuchuluka kwa ma platelet nthawi zambiri kumakhala kozolowereka mu Glanzmann Thrombasthenia (GT).Vuto si kuchuluka kwa ma platelet. M'malo mwake, ndi vuto la puloteni yomwe imathandiza ma platelet kumamatirana, zomwe zimalepheretsa ma platelet kuuma bwino.

Kodi kusiyana pakati pa Glanzmann Thrombasthenia (GT) ndi Bernard-Soulier syndrome (BSS) ndi kotani?

Glanzmann Thrombasthenia (GT) ndi Bernard-Soulier syndrome (BSS) ndi matenda osowa kwambiri a majini omwe amakhudza momwe magazi anu amaundana. Koma amayamba chifukwa cha kusintha kwa majini osiyanasiyana. Mavuto enieni omwe ali ndi ma platelet m'magazi anu ndi osiyana. Anthu omwe ali ndi BSS akhoza kukhala ndi ma platelet ochepa komanso ma platelet akuluakulu.

Zinthu zofunika kwambiri zomwe tiyenera kukumbukira kuchokera pa zomwe takambirana (Uthenga Wopita Nawo Kunyumba)

Glanzmann Thrombasthenia (GT) ndi matenda omwe inu ndi dokotala wanu wa magazi muyenera kuwayang'anira mosamala. Ndi GT, palibe njira yodziwira kuchuluka kwa magazi omwe mudzatuluka (kapena ochepa) m'magazi. Komabe, kuchitapo kanthu kuti muchepetse kutuluka magazi kungakuthandizeni kukhala ndi thanzi labwino. Ngati GT yanu ndi yoopsa, mankhwala monga kuikidwa magazi m'magazi angathandizenso.

Ngakhale simungathe kulamulira kubadwa ndi vutoli, kumbukirani kuti muli ndi njira zothetsera vutoli m'njira yosasokoneza moyo wanu. Musaope kufunafuna upangiri wa dokotala ndikudzisamalira. Mukatero mutha kukhala bwino ndi vutoli.

` Glanzmann thrombasthenia, GT, kutuluka magazi, ma platelet m'magazi, matenda a majini, kuvulala, vuto la kutuluka magazi, ma platelet