Mwana wako mudiki anogara achirwara here? Zvingave XLA (X-Linked Agammaglobulinemia). Ngatikurukurei!

Kunyange zvazvo dzimwe nguva tichifunga kuti zvakajairika kuti vadiki vedu vagare vachirwara, kune vamwe vana izvi zvinogona kuva dambudziko guru. Kunyanya kana mukomana achigara achitambura nehutachiona hwemabhakitiriya, zvinogona kunge zvichikonzerwa nechirwere chemajini chisingawanzoitiki. Nhasi tichataura nezvechirwere chakadaro.

Chii chinonzi XLA (X-Linked Agammaglobulinemia)?

Saka chii chinonzi XLA (X-linked agammaglobulinemia)? Zvichitaurwa zviri nyore, chirwere chemajini . Muviri wedu une uto rakakosha remasoja ekurwisa zvirwere, uye ndiwo masoja edu ekudzivirira muviri . Rudzi rwakakosha rwesero muhurongwa uhu runonzi B-cells . Aya maB-cell ndiwo anogadzira mapuroteni anonzi maantibodies ekurwisa zvirwere kana miviri yedu yarwara. Maantibodies aya anoshanda semasoja anodzivirira nyika yedu.

Munhu ane XLA (X-Linked Agammaglobulinemia) haagadzire masero aya eB nemazvo. Kana kuti anoburitsa mashoma awo. Saka, chii chinoitika kana usingakwanise kugadzira ma antibodies? Unorwara nyore nyore uye unowanzorwara. Zvakare, matishu ari mumuviri wedu ane chekuita nesystem yekudzivirira muviri, akadai se lymph nodes , tonsils, uye adenoids, haakure zvakanaka, uye dzimwe nguva haaumbike zvachose. Dambudziko iri rinowanzoonekwa muvakomana . Tichataura nezvechikonzero cheizvi gare gare.

Dambudziko iri, rinonzi XLA (X-Linked Agammaglobulinemia), rinozivikanwawo nemamwe mazita:

• Bruton's agammaglobulinemia
• Agammaglobulinemia yekuzvarwa nayo
• Hypogammaglobulinemia

Zvisinei, zita rekuti Hypogammaglobulinemia rinoshandiswawo kune rimwe dambudziko rakafanana. Ndiro CVID (Common Variable Immunodeficiency) . CVID (Common Variable Immunodeficiency) kazhinji haisi yakanyanya kuipa seXLA (X-Linked Agammaglobulinemia), uye inonyanya kuonekwa muvanhu vakuru. Zvisinei, vana vane XLA (X-Linked Agammaglobulinemia) vanoonekwa vasati vasvitsa gore kana kuti vachiri vadiki.

Ndeupi musiyano uripo pakati peXLA (X-Linked Agammaglobulinemia) neSCID (Severe Combined Immunodeficiency)?

Zvino ungave uchishamisika kuti iyi iXLA (X-Linked Agammaglobulinemia) here kana kuti chirwere chekudzivirira muviri chakanyanya kusanganiswa chawakanzwa nezvacho chinonzi SCID (Severe Combined Immunodeficiency) . Kwete, pane musiyano mudiki pakati pezviviri izvi. MuXLA (X-Linked Agammaglobulinemia) , maB-cell atakataura nezvawo kare ndiwo anonyanya kukanganiswa. MuSCID (Severe Combined Immunodeficiency) , maT-cell anokanganiswa.Imwe mhando inokosha yemasero ekudzivirira muviri anonzi T-cells (dzimwe nguva B-cells inogonawo kukanganiswa). Ose ari maviri mamiriro ezvinhu emajini anoshayisa simba masoja emuviri uye anowanzo kukonzera zvirwere. Asi musiyano mukuru pakati pezviviri izvi ndewerudzi rwemasero anokanganiswa.

XLA (X-Linked Agammaglobulinemia) yakajairika zvakadii?

Chirwere ichi chinonzi XLA (X-Linked Agammaglobulinemia) hachiwanzoitiki . Izvi zvinoreva kuti hachisi chirwere chinowanikwa nevanhu vazhinji. Zvisinei, sezvatakambotaura, chinowanzoitika muvakomana . Maererano nehuwandu, anenge mumwe chete pavakomana mazana maviri ezviuru (200,000) vanoberekwa vaine XLA (X-Linked Agammaglobulinemia).

Ndezvipi zviratidzo zveXLA (X-Linked Agammaglobulinemia)?

Nekuti vana vane XLA (X-Linked Agammaglobulinemia) vane masoja ekudzivirira muviri asina kusimba, ma lymph nodes avo, matonsi, uye adenoids anowanzova madiki kana kuti haapo . Izvi zvinoita kuti vabatwe nezvirwere zvemabhakitiriya kakawanda kubva pazera diki. Semuenzaniso:

• Bronchitis : Uku kutapukira kwebronchi, machubhu anoenda kumapapu.
• Utachiona hwenzeve (Otitis media) : Utachiona hwenzeve yepakati.
• Sinusitis : Kutapukira kwemasinusi akakomberedza mhino.
• Pneumonia : Utachiona hwakakomba hunokanganisa mapapu.
• Zvirwere zvemudumbu : Zvinhu zvakaita sekushungurudzika kwemudumbu uye manyoka.

Asi zvakakosha kuyeuka kuti vana vane XLA (X-Linked Agammaglobulinemia) havawanzobatwa nezvirwere zvemavhairasi (semuenzaniso, Cytomegalovirus (CMV) , RSV (Respiratory Syncytial Virus) , kana hutachiona hwefungus . Vanonyanya kukanganiswa nehutachiona hwemabhakitiriya.

Chii chinokonzera XLA (X-Linked Agammaglobulinemia)?

Sezvatakambotaura, XLA (X-Linked Agammaglobulinemia) chirwere chemajini . Izvi zvinoreva kuti mwana anochigara nhaka kubva kuna amai, baba, kana vese. Tine jini rinonzi BTK gene mumuviri wedu. Jini iri rinorayira muviri wedu kugadzira masero eB. Tinoziva kuti masero eB anogadzira masoja ekudzivirira chirwere uye anorwisa zvirwere.

Saka, kana paine shanduko kana shanduko mujini iri reBTK, masero eB haagone kugadzirwa nemazvo. Zvadaro, chinoitika ndechekuti munhu asina shanduko yemajini haakwanise kurwisa zvirwere sezvaanoita. Ndosaka vachirwara nguva dzese, uye dzimwe nguva vanotoita zvirwere zvakakomba zvinogona kutyisidzira hupenyu.

Chii chinonzi 'X-linked'?

Iye zvino ngatitarisei zvinorehwa ne'X-linked'. Tose tinowana majini edu kubva kuvabereki vese. Majini aya ari pazvinhu zvinonzi machromosomes . Majini aya anoudza miviri yedu maitiro ekugadzira mapuroteni aanoda kuti ashande. Kazhinji, kunyangwe paine shanduko mune rimwe jini, rimwe jini (rinobva kune rimwe mubereki) rinenge risisina chinhu, saka muviri unogona kushanda sezvaunofanira kuita.

Zvisinei, machromosome evarume ebonde haana kufanana. Ane chromosome imwe chete yeX uye imwe yeY . Saka, kana paine shanduko mujini pane iyi chromosome yeX, hapana imwe kopi yekuitsiva. Jini reBTK ratataura nezvaro riri pane iyi chromosome yeX. Ndosaka richinzi 'X-linked'. Saka kana mukomana aine shanduko mujini reBTK paX chromosome yake, achava neXLA (X-Linked Agammaglobulinemia).

Vasikana vane ma chromosome maviri eX. Kunyangwe vaine mutation inokonzera XLA (X-Linked Agammaglobulinemia) pane imwe yema chromosome avo eX, gene reBTK pane imwe X chromosome richiri kushanda nemazvo, saka vanogona kugadzira huwandu hunodiwa hwema B-cells. Saka havabatwi nechirwere ichi, asi vanogona kuva vatakuri . Izvi zvinoreva kuti vanogona kutakura gene vasingaratidze zviratidzo.

Ndezvipi zvinhu zvinokonzera XLA (X-Linked Agammaglobulinemia)?

Kusvika pari zvino, chinhu chega chinozivikanwa chinokonzera njodzi yekubatwa neXLA (X-Linked Agammaglobulinemia) inhoroondo yemhuri yechirwere ichi . Izvi zvinoreva kuti chinogara nhaka.

Vasikana vanogona kuwana XLA (X-Linked Agammaglobulinemia) here?

Ehe, kashoma , mwana wechikadzi anogonawo kuita XLA (X-Linked Agammaglobulinemia). Asi kuti izvozvo zviitike, vabereki vese vanofanira kutakura X chromosome ine gene reBTK rakachinja. Kureva kuti, amai ndivo vanotakura uye baba vane XLA (X-Linked Agammaglobulinemia). Kazhinji, vana vechikadzi vanogona kunge vari vatakuri vekuchinja kwemajini uku. Zvadaro, kunyangwe vasina chirwere ichi, vanogona kutapurira jini iri kuvana vavo. Kana vana ivavo vari vakomana, vangangoita XLA (X-Linked Agammaglobulinemia).

Ndezvipi zvinetso zvinogona kuitika nekuda kweXLA (X-Linked Agammaglobulinemia)?

Mamwe ematambudziko anogona kuitika neXLA (X-Linked Agammaglobulinemia) ndeaya:

• Chirwere chemapapu chisingaperi : Kurwara nemapapu nguva nenguva kunogona kukanganisa mapapu nekufamba kwenguva.
• Kupararira kwehutachiona kune dzimwe nhengo dzemuviri : Semuenzaniso, hutachiona hunogona kupararira kuenda kuropa (sepsis) kana kuuropi (meningitis).
• Panewo fungidziro yekuti panogona kunge paine njodzi yakakura yekubatwa negomarara , asi tsvakiridzo iri kuitwawo pane izvi.

XLA (X-Linked Agammaglobulinemia) inoonekwa sei?

Chiremba anogona kuita bvunzo dzeropa dzinoverengeka kuti aone kana iwe kana mwana wako ane XLA (X-Linked Agammaglobulinemia). Kana bvunzo idzi dzeropa dzichiratidza kuti maB-cells enyu kana ma antibodies akaderera, chiremba wenyu achaita bvunzo dzemajini . Izvi zvinotsvaga shanduko mu BTK gene muDNA yenyu .

XLA (X-Linked Agammaglobulinemia) inorapwa sei?

Zvinosuwisa kuti hapana mushonga weXLA (X-Linked Agammaglobulinemia). Zvisinei, kune mishonga inogona kubatsira kudzivirira matambudziko akakomba. Mimwe mikuru ndeiyi:

• Kurapa kweImmunoglobulin (RIgG) : Izvi zvinosanganisira kupa ma antibodies kubva kune vanopa vane hutano netsinga (IV). Kurapa uku kunoitwa kanenge kamwe chete pamwedzi . Izvi zvinobatsira kudzora huwandu hwakaderera hwema antibodies mumuviri kusvika pamwero wakati.
• Kurapa hutachiona nekukurumidza : Kana iwe kana mwana wako muchangofungirwa kuti mune hutachiona, chiremba wenyu achatanga kurapa hutachiona hwebhakitiriya nemishonga inorwisa mabhakitiriya . Zvakakosha kutanga kurapwa nekukurumidza.
• Kudzivisa kubaiwa majekiseni mhenyu : Vanhu vane XLA (X-Linked Agammaglobulinemia) havafanirwe kugamuchira majekiseni mhenyu . Majekiseni aya anogona kukonzera kurwara kwakakomba uye anogona kutyisidzira hupenyu. Mienzaniso inosanganisira mushonga weMMR (gwirikwiti, mumps, rubella) , mushonga wehuku - varicella, uye mushonga weporiyo wemuromo . Saka, zvakakosha kutaura nachiremba wako nezveizvi uye kuziviswa zvizere.

Chii chinofanira kutarisirwa nemunhu ane XLA (X-Linked Agammaglobulinemia)?

Vanhu vane XLA (X-Linked Agammaglobulinemia) vanofanira kutora mishonga kweupenyu hwavo hwese . Izvi zvinoitirwa kuderedza njodzi yavo yekubatwa nezvirwere. Vanofanira kuchengetedza hukama hwepedyo nachiremba wavo uye kutsvaga kurapwa nekukurumidza kana paine chirwere chipi zvacho chikaitika. Iwe kana mwana wako ane XLA (X-Linked Agammaglobulinemia) munogona kupotsa mazuva mazhinji echikoro nekushanda nekuda kwekurwara kupfuura vanhu vose.

Vanhu vane XLA (X-Linked Agammaglobulinemia) vanorarama kwenguva yakareba sei?

Chinhu chakanaka kwazvo kuti, nekuvandudzwa kwenzira dzekurapa , vanhu vane XLA (X-Linked Agammaglobulinemia) munyika dzakabudirira seAmerica vari kurarama kusvika vakura . Zvisinei, munyika dzichiri kusimukira, zvichiri kuoma kuongorora nekugamuchira kurapwa. Nokudaro, kazhinji, hupenyu hwevana vane XLA (X-Linked Agammaglobulinemia) munyika dzakadaro hunogona kuderedzwa. Asi muSri Lanka, ikozvino kune mishonga yakanaka yezvirwere zvakadaro.

XLA (X-Linked Agammaglobulinemia) inogona kudzivirirwa here?

Kana uchinetseka nezveXLA (X-Linked Agammaglobulinemia), zvichireva kuti mumwe munhu mumhuri yako ane chirwere ichi, unogona kuona chiremba woongororwa majini . Izvi zvinogona kukubatsira kuziva nezvezvirwere zvemajini zvaunogona kupa mwana wako. Kana uri mutakuri wekuchinja kwemajini kunokonzera XLA (X-Linked Agammaglobulinemia), kana uine mwana, pane mukana we50% wekuti mwana iyeye achagara nhaka kuchinja uku. Kana mwana iyeye ari mukomana, anogona kuva neXLA (X-Linked Agammaglobulinemia). Kana uine XLA (X-Linked Agammaglobulinemia), vasikana vako vachava vatakuri. Mupi wezano wezvemajini kana chiremba akaraira bvunzo achakupa mamwe mazano nezveizvi.

Ndinozvitarisira sei?

Nzira yakanakisisa yekuzvitarisira neXLA (X-Linked Agammaglobulinemia) ndeyekuisa hutano hwako pakutanga . Chengeta nguva yawakarairwa nachiremba. Dzidza kuziva zviratidzo zvehutachiona. Bvunza chiremba wako zvekuita kana ukaona zviratidzo zvehutachiona.

Ndinofanira kuona chiremba rini?

Zvakanakisisa kutaura nachiremba kana paine zvinotevera:

• Kana mwana wako akarwara mushure mekugamuchira mushonga wekudzivirira chirwere (semuenzaniso mushonga weMMR, mushonga wehuku).
• Kana mwana wako achiwanzobatwa nehutachiona hwemabhakitiriya .
• Kana ukagara uchibatwa nehutachiona hwemabhakitiriya (sezvo ichi chingava chirwere chakaita seCVID, icho chinokanganisawo vanhu vakuru).

Chiremba achakwanisa kukuudza kana izvi zvichifanira kuongororwa zvakanyanya kana kuti kwete.

Mibvunzo ipi yandinofanira kubvunza chiremba wangu?

Zvingakubatsira kubvunza mibvunzo yakaita seiyi paunoona chiremba wako:

• Ndezvipi zviratidzo zvehutachiona zvandinofanira kutarisa?
• Ndinofanira kuitei kana ndichifunga kuti ini kana mwana wangu tine hutachiona?
• Ndezvipi zvingasarudzwa pakurapa?
• Kangani ini kana mwana wangu patinoda kurapwa?

Zvakajairika kuti vana vadiki vagare vachirwara. Asi kana mwana wako achigara achirwara nehutachiona, panogona kunge paine chimwe chinhu chiri kukonzera izvi. Taura nachiremba wako nezvechero dambudziko raunaro. Kuongororwa nekurapwa nekukurumidza ndiyo nzira yakanakisisa yekuwana mhedzisiro yakanaka.

Kana uchirarama neXLA (X-Linked Agammaglobulinemia) - chirwere chemajini umo muviri wako usingagadzire maB-cell nemazvo - tevera mirairo yachiremba wako nemazvo. Zvakakosha kuti ukwanise kuziva zviratidzo zvehutachiona kuitira kuti ugone kurapwa nekukurumidza.

Zvinhu zvinonyanya kukosha zvekurangarira munyaya ino

Saka hezvino zvinhu zvishoma zvaunofanira kuyeuka kubva pane zvatataura nezve XLA (X-Linked Agammaglobulinemia):

• Chii chinonzi XLA (X-Linked Agammaglobulinemia)?Chirwere chemajini chisingawanzoitiki chinonyanya kubata vakomana .
• Apa ndipo apo masero emuviri eB-cells asingakure zvakanaka , zvichikonzera kudzikira kwemasoja emuviri uye hutachiona hwemabhakitiriya hunowanzoitika .
• Zvinhu zvakaita sematoni neadenoids zvinogona kuderera kana kunyangarika.
• Kunyangwe pasina mushonga chaiwo weizvi, zvinogona kudzorwa zvakanaka nekurapwa kwehupenyu hwese (RIgG) uye kurapwa nekukurumidza kwemishonga inorwisa mabhakitiriya.
• Hazvina kunaka kupa vanhu ava majekiseni mhenyu.
• Kana mwana wako mukomana achigara achirwara zvakanyanya, zvakakosha kutsvaga rubatsiro rwechiremba nekukasika. Kuongororwa nekukurumidza uye kurapwa kunobatsira zvikuru mwana wako kurarama hupenyu hwakajairika.

Ndinovimba ruzivo urwu ruchakubatsira. Kana uine mibvunzo, usazeza kutaura nachiremba wemhuri yako!

` X-linked agammaglobulinemia, XLA, B-masero, immune system, zvirwere zvemajini, kurwara-rwara, vakomana, masoja ekudzivirira chirwere, BTK gene, RIgG therapy