Ngaba uhlala ugula? Esi sisizathu: Masifunde nge-hypogammaglobulinemia.

Ngaba uhlala ufumana umkhuhlane, umkhuhlane, kunye nokukhwehlela? Okanye umntwana wakho omncinci uhlala efumana usulelo lwendlebe okanye iingxaki ze-sinus? Ngamanye amaxesha sicinga ukuba ezi zizifo eziqhelekileyo, kodwa ukuba ezi sulelo ziyaqhubeka zibuya, kunokubakho esinye isizathu esiyintloko. Enye imeko enokukrokrelwa ngamaxesha anjalo yi-hypogammaglobulinemia. Nangona ligama elide, masiliqonde ngokulula.

Yintoni i-hypogammaglobulinemia?

Ngamafutshane, i-hypogammaglobulinemia yimeko apho umzimba wakho unezinga eliphantsi lee-antibodies. Elinye igama lala ma-antibodies yi-immunoglobulins. Ngoku khawucinge ukuba umzimba wethu ufana nenqaba. Iintshaba eziza kule nqaba ziintsholongwane, ezifana neentsholongwane kunye neebhaktheriya. Ngoko ke amajoni asikhuselayo kwezi ntshaba yinkqubo yethu yokuzikhusela .

Le nkqubo yokuzikhusela komzimba inohlobo oluthile lweeseli ezibizwa ngokuba zii-B-cells . Zifana nee-commandos eziqeqeshwe ngokukodwa. Xa intsholongwane ingena emzimbeni, ezi B-cells zivelisa ii-antibodies ezibizwa ngokuba zii-immunoglobulins. Ezi antibodies ziyahamba zibambe intsholongwane ziyitshabalalise. Kulapho singaguli khona, okanye siphila ngokukhawuleza kwisifo.

Uza kufumanisa ngale meko kuphela xa uvavanya igazi.

Masiwahlule la magama ukuze aqondwe:

• I-Hypo: ithetha ukuba ingaphantsi kwesiqhelo.
• IiGamma globulin: Ezi zezona ntlobo zixhaphakileyo ze-immunoglobulin.
• U-Emia: kuthetha egazini.

Ngoko ke, xa inqanaba le-immunoglobulin egazini lakho lincipha, inkqubo yokuzikhusela yomzimba wakho iba buthathaka. Emva koko iintsholongwane zinokuhlasela umzimba wakho ngokulula. Ngenxa yoko , unamathuba amaninzi okufumana usulelo kwaye ufumane ezinye izifo.

Ngaba kukho iintlobo zezi?

Ewe, imeko ebizwa ngokuba yi-hypogammaglobulinemia inokuchaphazela abantwana kunye nabantu abadala. Kukho iintlobo ezimbini eziphambili:

1. I-hypogammaglobulinemia yokuqala (ezalwa nayo): Oku kubangelwa zizifo ezithile ezingaqhelekanga zemfuza. Sizibiza ngokuba ziZifo eziPhambili zeMfuza (PIDD) . Ngamafutshane, xa uzalwa, uzalwa unempazamo ethile (uguquko) kwimfuza yakho. Ezi mfuza zezo zifundisa inkqubo yomzimba yokuzikhusela indlela yokusebenza. Ngoko ke xa kukho impazamo kuloo myalelo, ukhuselo lomzimba luyancitshiswa. Ezi mpazamo zemfuza zihlala zidluliselwa kwizizukulwana ngezizukulwana.

2. I-hypogammaglobulinemia yesibini (efunyenweyo): Le yiUhlobo oluqhelekileyo. Le meko inokubangelwa ziimeko ezahlukeneyo zonyango okanye njengemiphumo emibi yamayeza athile. Oku kuthetha ukuba uzalwe usempilweni uze emva koko ube nale meko kamva ebomini.

Ingaba le meko inqabile kangakanani?

I-Hypogammaglobulinemia yenye yezona zifo ziqhelekileyo ze-primary immunodeficiency diseases (PIDDs) ezikhankanyiweyo ngaphambili. Nangona kunjalo, ii-PIDD azisiso isifo esiqhelekileyo ngokubanzi. Umzekelo, kuqikelelwa ukuba malunga nesiqingatha sesigidi sabantu e-United States bahlala ne-PIDD.

Zithini iimpawu zoku?

Uphawu oluphambili lwe-hypogammaglobulinemia kubantwana nakubantu abadala kusulelo oluhlala luhleli okanye oluhlala ixesha elide. Iimpawu zixhomekeke kuhlobo losulelo oluvelayo. Usulelo oluqhelekileyo kunye neempawu ezinxulumene ne-hypogammaglobulinemia ziquka:

• Usulelo lwendlebe: iintlungu zendlebe, ukwenzakala kwendlebe (ngamanye amaxesha amanxeba), umkhuhlane. (Khawucinge ukuba abanye abantwana banezifo zendlebe ezingapheliyo, ezibuya rhoqo nangona befumana amayeza.)
• Ukosuleleka yiSinus: Impumlo egobhozayo okanye impumlo evalekileyo, ukudinwa, umkhuhlane. (Abanye abantu bavuka kusasa beziva bevalekile kwaye bexinene intloko, akunjalo? Ukuba kuyaqhubeka, kufuneka ukhathazeke.)
• I-Bronchitis: Ukukhohlela okungapheliyo, ukuphefumla kancinci, umkhuhlane.
• I-Pneumonia: Ukuqina kwesifuba okanye intlungu, ubunzima bokuphefumla, umkhuhlane.
• Umkhuhlane wesisu: urhudo, isicaphucaphu, ukuhlanza, umkhuhlane.
• I-Meningitis: Intamo eqinileyo, intloko ebuhlungu, umkhuhlane. (Oku kuyingozi kancinci, kufuneka ukufumane ngokukhawuleza.)
• Usulelo lolusu: izilonda zesikhumba, ukudumba, ukurhawuzelela, umkhuhlane.

Kwakhona, iimpawu zinokwahluka ngokuxhomekeke kwimeko ebangela amanqanaba aphantsi e-immunoglobulin. Umzekelo, umntwana oneentlobo ezithile ze-PIDD unokuba namathuba amaninzi okuba ne-allergies. Ezinye iintlobo zinokubangela ukulibaziseka ekukhuleni nasekuphuhliseni.

Yintoni ebangela oku?

Zimbini izizathu ezinokubakho. Nokuba inkqubo yakho yomzimba ayivelisi ii-immunoglobulins ezaneleyo, okanye ezo izenzayo ziyatshatyalaliswa ngesizathu esithile. Izizathu ziyahluka ngokuxhomekeke ekubeni une-lupus eyintloko okanye yesibini.

Izizathu ze-hypogammaglobulinemia ephambili

Ezinye zezona ntlobo zixhaphakileyo ze-'PIDD' ezinokunxulunyaniswa noku zezi:

• I-Common Variable Immunodeficiency (CVID): Isiphene semfuza esithintela umzimba ekubeni wenze ii-immunoglobulins ezaneleyo. Le yeyona nto ixhaphakileyo ye-primary hypogammaglobulinemia kubantu abadala.
• I-Agammaglobulinemia edityanisiweyo ne-X (XLA): Isiphene semfuza kwi-chromosome ye-X sithintela umzimba ekubeni wenze ii-B-cells okanye ii-immunoglobulin ezaneleyo. Oku kuchaphazela kakhulu iintsana ezingamakhwenkwe (kuba zine-X chromosome enye kuphela). I-XLA yimbangela eqhelekileyo ye-hypogammaglobulinemia ebantwaneni.
• I-Transient Hypogammaglobulinemia yoBusana (THI): Oku kwenzeka xa amanqanaba e-immunoglobulin yosana eqala ukwehla malunga neenyanga ezi-3 ubudala. Iintsana ezine-THI zinokufumana usulelo, kodwa zihlala zingabonisi zimpawu. Le meko idla ngokuziphelela xa zineminyaka emi-3 ubudala.
• Ukunqongophala kwe-IgA ekhethiweyo: Oku kwenzeka xa umzimba ungazalisi uhlobo oluthile lwe-immunoglobulin ebizwa ngokuba yi-Immunoglobulin A.
• Ii-Hyper-IgM syndromes: Kule meko, ezinye iintlobo ze-immunoglobulin ziveliswa ngendlela eqhelekileyo okanye ngokugqithisileyo, kodwa ezinye aziveliswanga ngobuninzi obaneleyo.

Ukongeza koku, kukho ezinye iintlobo ze-PIDD ezibangela i-primary hypogammaglobulinemia:

• I-Ataxia-telangiectasia
• I-Autosomal recessive agammaglobulinemia (ARA)
• Isifo esilungileyo (GS)
• Ukunqongophala kwe-immunoglobulin engeyiyo i-IgG ekwahlukileyo
• Ukungakwazi komzimba okudibeneyo okunamandla (i-SCID)
• Ukungabikho kwe-antibody ethile (SAD)
• Isifo sikaWiskott-Aldrich (WAS)

Izizathu ze-hypogammaglobulinemia yesibini

Nazi izizathu zokuba le meko inokwenzeka kamva:

• Amayeza: Amanye amayeza anokubangela amanqanaba aphantsi e-immunoglobulin. Umzekelo, amayeza okunciphisa amasosha omzimba, amayeza okuthintela ukuxhuzula, ii-corticosteroids, kunye namayeza e-chemotherapy.
• Isifo seNephrotic: Kule meko, izintso zakho zikhupha iiproteni ezininzi kakhulu kumchamo wakho. Ii-immunoglobulins, ezikwayiproteni, zinokukhutshwa ngale ndlela.
• I-enteropathy elahlekelwa yiproteni: Oku kwenzeka xa iproteni eninzi kakhulu ilahleka emzimbeni wakho ngenkqubo yakho yesisu.
• Umhlaza:Iimhlaza ezichaphazela ii-B-cells zakho zinokubangela amanqanaba aphantsi e-immunoglobulin. Umzekelo, iimhlaza ezifana ne-chronic lymphocytic leukemia, i-lymphoma, kunye ne-multiple myeloma.
• I-HIV: I-HIV sisifo esidluliselwa ngesondo (STI). Inokubangela i-hypogammaglobulinemia kunye nezinye iingxaki zenkqubo yomzimba yokuzikhusela.

Zeziphi iingxaki ezinokubangela oku?

Ukuba ayinyangwa, i-hypogammaglobulinemia inokukhokelela kwiintsholongwane ezinzulu kunye neengxaki ezisongela ubomi . Ingxaki eqhelekileyo yi- bronchiectasis . Le yimeko apho imiphunga yonakala ngokusisigxina. Le meko ixhaphake kakhulu kubantu abanezifo ezixhaphakileyo zendlela yokuphefumla ephezulu.

Khawucinge ngomntu oneengxaki zemiphunga ekhwehlela rhoqo kunye ne-phlegm. Ukuba imiphunga yakhe yonakele rhoqo, inokuba yingxaki engapheliyo.

Ukongeza, i-hypogammaglobulinemia yandisa umngcipheko we:

• Izifo ezizikhusela ngokwazo: Ezi ziimeko apho inkqubo yakho yokuzikhusela komzimba ihlasela iiseli zomzimba wakho.
• Ukonakala kwamalungu angaphakathi.
• Umhlaza.
• I-Sepsis: Olu luhlobo losulelo olusasazeka emzimbeni wonke.

Uyibona njani le nto?

Oogqirha baza kwenza uvavanyo lwegazi ukulinganisa amanqanaba akho e-immunoglobulin. Ukuba amanqanaba akho aphantsi kunesiqhelo, unokufunyanwa une-hypogammaglobulinemia. Ugqirha wakho uya kuthi emva koko achaze imeko yakho njengethambileyo , ephakathi, okanye enzima .

Ukuze afumanise ukuba yintoni kanye kanye ebangela eli nqanaba liphantsi, ugqirha uza kuqwalasela oku kulandelayo:

• Iimpawu zakho: Ingakumbi nokuba ufumana usulelo rhoqo.
• Imbali yempilo yosapho lwakho: Ngaba kukho umntu kusapho lwakho one-PIDD?
• Amayeza owasebenzisayo: Ingakumbi amayeza anokubangela i-hypogammaglobulinemia njengesiphumo esingesihle.

Kusenokufuneka wenze olunye uvavanyo, olunokuquka:

• Ezinye iimvavanyo zegazi: Umzekelo, ukubalwa kwegazi okupheleleyo, uvavanyo lwee-antibody, kunye novavanyo olujonga indlela amalungu akho asebenza ngayo.
• Iinkqubo zokuthatha imifanekiso: Ukujonga umonakalo kwizicwili ngenxa yosulelo okanye iithumba (umz., i-X-ray, i-CT scan)
• Uvavanyo lwemfuza: Jonga iziphene zemfuza ezinxulumene ne-PIDD.
• Uvavanyo lwe-biopsy:Ukuthatha isampuli yethishu uze uyivavanye ukuze ubone ukuba kukho iiseli zomhlaza na.

Iphathwa njani le nto?

Unyango luxhomekeke kwisizathu se-hypogammaglobulinemia yakho kunye nokuba imbi kangakanani. Ungathunyelwa kwi-immunologist.

Ukuba imeko ilandela imeko yezonyango , oogqirha badla ngokunyanga imeko esisiseko. Umzekelo, ukuba amanqanaba akho e-immunoglobulin aphantsi ngenxa yesiphumo esingesihle seyeza olisebenzisayo, amayeza anokutshintshwa.

Ukuba une -primary hypogammaglobulinemia , kuya kufuneka ube phantsi kweliso likagqirha ubomi bakho bonke ukuze uzikhusele kwiintsholongwane ezinzulu. Iindlela zonyango zingabandakanya:

• Ukulinda ngononophelo: Ukuba amanqanaba akho aphantsi e-immunoglobulin awabeki mngcipheko omkhulu kwimpilo, ugqirha wakho unokujonga imeko yakho. Iimeko eziziphelela ngokwazo, ezifana ne-transient hypogammaglobulinemia of infant (THI), ekhankanyiweyo ngaphambili, zisenokungadingi unyango.
• Ii-antibiotics: Usenokunikwa ii-antibiotics ukunyanga usulelo olubangelwa yibhaktiriya okanye ukuthintela usulelo.
• Unyango lokubuyisela i-Immunoglobulin (IgG): Ukuba amanqanaba akho e-immunoglobulin aphantsi kakhulu, unganikwa i-immunoglobulin evela kumntu onikelayo. Oku kunikwa njengenaliti emthanjeni (IV) okanye phantsi kolusu (ngaphantsi kolusu). Abantu abane-PIDD abafuna olu nyango badla ngokufuna ukuthatha olu nyango ubomi babo bonke.
• Ukufakelwa kweeseli ze-stem: Ukuba umzimba wakho awukwazi ukwenza iiseli zomzimba ezisempilweni, ungafakelwa iiseli ze-stem ezivela kumntu onikelayo. Ezi seli ze-stem kamva ziya kukhula zibe ziiseli zegazi ezisempilweni, kuquka iiseli ze-B.

Ingaba le yimeko enzima?

I-Hypogammaglobulinemia inokuba yinto embi kakhulu. Kuxhomekeke kwisizathu kunye nokuba amanqanaba akho e-immunoglobulin aphantsi kangakanani. Ii-immunoglobulin zifana namajoni emizimbeni yethu alwa neentsholongwane eziyingozi ezifana neentsholongwane, iibhaktheriya, iiparasites, kunye nefungi. Ngoko ke xa la majoni engasekho ukuze asikhusele, sinamathuba amaninzi okufumana usulelo oluqatha nolunzima. Ukuba alunyangwa, usulelo olunzima lunokubangela umonakalo wezicubu ixesha elide kwanokufa.

Ngoko ke, le meko ayifanele ithathwe lula. Ukuba uneempawu, kubalulekile ukufuna iingcebiso zonyango ngokukhawuleza.

Kuza kwenzeka ntoni ukuba ayinyangwa?

Kubalulekile ukuba ugqirha afumanise unobangela we-hypogammaglobulinemia. Ukuba uqala unyango kwangethuba, unethuba elingcono lokuhlala usempilweni kwaye uthintele iingxaki ezinokwenzeka emva kweminyaka. Ukuba unesifo esikubeka emngciphekweni we-hypogammaglobulinemia, ugqirha wakho uya kujonga amanqanaba akho e-immunoglobulin rhoqo. Baza kucebisa isicwangciso sonyango ukunceda ukuthintela usulelo olunzima.

Ngaba oku kungathintelwa?

Asinakuyithintela imeko (ezifana neziphene zemfuza) ezibangela i-hypogammaglobulinemia. Nangona kunjalo, ugqirha wakho unokucebisa unyango olunokunceda ukuthintela iingxaki , ezinje ngezifo ezixhaphakileyo kunye nomonakalo wemiphunga.

Ndingazinyamekela njani? Ndingenza ntoni ukuthintela usulelo?

Eyona nto ibalulekileyo kukulandela imiyalelo kagqirha wakho. Ezi zinto zinokunceda ekuthinteleni usulelo:

• Hlamba izandla zakho kakuhle: Hlamba izandla zakho ngesepha namanzi ubuncinane imizuzwana engama-20. Qiniseka ukuba uhlamba izandla zakho emva kokuphuma, ngaphambi kokutya, nasemva kokusebenzisa indlu yangasese.
• Ukunxiba imaski yobuso xa kuyimfuneko ukuthintela ukusasazeka kweentsholongwane eziphuma emoyeni: Oku kubaluleke kakhulu xa usiya kwiindawo ezinabantu abaninzi nangamaxesha apho izifo zixhaphake kakhulu.
• Musa ukuhlala nabantu abagulayo nakwiindawo ezixineneyo kangangoko unako: iintsholongwane zinokusasazeka lula kwiindawo ezinjalo.
• Ukufumana zonke izitofu ngexesha: Nangona kunjalo, buza ugqirha wakho malunga nokuba ezinye izitofu eziphilayo zikulungele na, njengoko ezinye izitofu eziphilayo zisenokungafaneleki kubantu abaneenkqubo zomzimba ezibuthathaka.

Ndingayibuza ntoni ugqirha?

Xa ufumanisa ngale meko, unokuba nemibuzo emininzi. Musa ukoyika. Thetha nogqirha wakho uze ucacise oku kulandelayo:

• Yintoni ebangela ukuba amanqanaba am e-immunoglobulin ehle?
• Zeziphi iimvavanyo ekufuneka ndizithathe ukuze ndixilonge/ndijonge imeko yam?
• Loluphi unyango olucebisayo?
• Ingaba kukho naziphi na iziphumo ebezingalindelekanga kunyango? Zithini ezo?
• Zeziphi iinguqu zendlela yokuphila ekufuneka ndizenze ukuze ndithintele usulelo?
• Zeziphi iimpawu ekufuneka ndibone ugqirha ngoko nangoko? Okanye kufuneka ndiye kwigumbi longxamiseko (ETU) ?

Xa ufumanisa ukuba amanqanaba akho e-immunoglobulin aphantsi, ungaziva ubuthathaka kwaye ubuthathaka. Usenokucinga, "Kuthekani ukuba andinamajoni aneleyo okundikhusela?"

Kodwa ungakhathazeki. Ukuthetha nogqirha wakho linyathelo lokuqala lokufunda ngakumbi ngemeko yakho, ukuba yintoni ebangela oko, kunye nendlela unyango olunokunceda ngayo. Sebenzisana nogqirha wakho ukulawula imeko yakho kwaye ufumane unyango olufunayo.

Okokugqibela, izinto ekufuneka uzikhumbule

I-Hypogammaglobulinemia yimeko apho amajoni omzimba angenazo ii-antibodies (ii-immunoglobulin), ezilwa neentsholongwane. Oku kunokukhokelela kwizifo ezixhaphakileyo.

Eyona nto ibalulekileyo kukufuna ingcebiso kagqirha ukuba uhlala ufumana usulelo, ingakumbi uhlobo olufanayo losulelo, endaweni yokuluthatha njengento eqhelekileyo.

• Le meko inokubakho xa umntwana ezalwa, okanye ingavela kamva ngenxa yesinye isifo okanye amayeza.
• Ukuba ufumene unobangela kwaye ufumane unyango olufanelekileyo, unokuthintela iingxaki kwaye uphile ubomi obusempilweni.
• Kubaluleke kakhulu ukulandela iingcebiso zonyango, ukuphila ubomi obusempilweni, nokuthatha amanyathelo okuzikhusela kwiintsholongwane.

Awuwedwa, kukho oogqirha kunye nabasebenzi bezempilo abanokukunceda ujongane nale meko. Musa ukoyika ukuthetha nabo.

I- Hypogammaglobulinemia, ii-Antibodies, i-Immunoglobulin, iNkqubo yoKhuseleko loMzimba, iiNtsholongwane, i-PIDD