I-Agammaglobulinemia

I-Agammaglobulinemia sisifo esizuzwe njengelifa apho umntu anamanqanaba asezantsi kakhulu okhusela iiproteni zomzimba ezibizwa ngokuba zii-immunoglobulins. Ii-Immunoglobulins ziluhlobo lwe-antibody. Amanqanaba asezantsi kwezi ntsholongwane akwenza ube lula ukufumana izifo.

Esi sisifo esinqabileyo esichaphazela ikakhulu amadoda. Kubangelwa sisiphene semfuza esithintela ukukhula kweeseli zomzimba eziqhelekileyo, ezivuthiweyo ezibizwa ngokuba ziiB lymphocyte.

Ngenxa yoko, umzimba wenza ii-immunoglobulin ezincinci (ukuba zikhona). Ii-Immunoglobulins zidlala indima enkulu kwimpendulo yomzimba yokuzikhusela, ekhusela kwisifo kunye nosulelo.

Abantu abanesi sifo baba nezifo rhoqo. Usulelo oluqhelekileyo lubandakanya ezo zibangelwa ziibhaktheriya ezinje Haemophilus influenzae, inyumoniya (IStreptococcus pneumoniae), kunye neStaphylococci. Iindawo eziqhelekileyo zosulelo zibandakanya:

• Iphetshana lesisu
• Amalungu
• Amaphaphu
• Ulusu
• Indawo ephezulu yokuphefumla

I-Agammaglobulinemia izuzwe njengelifa, oko kuthetha ukuba abanye abantu kusapho lwakho banokuba nale meko.

Iimpawu zibandakanya iziqendu ezixhaphakileyo ze:

• I-bronchitis (usulelo lwomoya)
• Urhudo olunganyangekiyo
• Ukudibana (usulelo lwamehlo)
• I-otitis media (usulelo lwendlebe oluphakathi)
• I-pneumonia (usulelo lwemiphunga)
• Sinusitis (usulelo lwesono)
• Usulelo lolusu
• Usulelo oluphezulu lokuphefumula

Usulelo lubonakala kwiminyaka emi-4 yokuqala yobomi.

Ezinye iimpawu zibandakanya:

• I-Bronchiectasis (isifo apho iisaka ezincinci zomoya emiphungeni zonakala kwaye zandiswe)
• Isifo sombefu ngaphandle kwesizathu esaziwayo

Ukuphazamiseka kungqinwa luvavanyo lwegazi olulinganisa amanqanaba ee-immunoglobulins.

Uvavanyo lubandakanya:

• Ukuhamba kwecytometry ukulinganisa ukujikeleza kwee-lymphocyte ze-B
• Immunoelectrophoresis - serum
• Ubungakanani be-immunoglobulins -I-IgG, i-IgA, i-IgM (ihlala ilinganiswa nge-nephelometry)

Unyango lubandakanya ukuthatha amanyathelo okunciphisa inani kunye nobukhali bosulelo. Amayeza okubulala iintsholongwane ahlala efuneka ukunyanga usulelo lwebacteria.

Ii-Immunoglobulins zinikwa ngomthambo okanye ngenaliti ukonyusa amajoni omzimba.

Ukufakelwa komongo wethambo kunokuqwalaselwa.

Ezi zixhobo zinokubonelela ngolwazi oluthe kratya kwi-agammaglobulinemia:

• Isiseko sokusweleka komzimba: primaryimmune.org
• Umbutho weSizwe woNxunguphalo oluNqabileyo-ararediseases.org/rare-diseases/agammaglobulinemia
• Isalathiso sasekhaya se-NIH / NLM yemfuzo - ghr.nlm.nih.gov/condition/x-linked-agammaglobulinemiaemia

Unyango nge-immunoglobulins luyiphucule kakhulu impilo yabo banesi sifo.

Ngaphandle konyango, ezona zifo zinzima ziyabulala.

Iingxaki zempilo ezinokubangela zibandakanya:

• Isifo samathambo
• Isifo esingapheliyo okanye isifo se-pulmonary
• Onokhwekhwe
• I-syndromes yamathumbu emathunjini

Biza ixesha lokubonana nomboneleli wakho wezempilo ukuba:

• Wena okanye umntwana wakho ufumene ukosuleleka rhoqo.
• Unembali yosapho ye-agammaglobulinemia okanye enye i-immunodeficiency disorder kwaye uceba ukuba nabantwana. Buza umboneleli malunga neengcebiso ngemfuzo.

Ingcebiso ngezofuzo kufuneka inikwe abo bafuna ukuba ngabazali abanembali yosapho ye-agammaglobulinemia okanye ezinye iingxaki zokuphazamiseka komzimba.

I-agammaglobulinemia yeBruton; I-X edityaniswe ne-agammaglobulinemia; Uxinzelelo lwe-Immunosuppression - agammaglobulinemia; Uxinzelelo lwe-immune - i-agammaglobulinemia; Uxinzelelo lwe-immune-agammaglobulinemia

• Iintsholongwane

ICunningham-Rundles C. Izifo zokuqala zokungasebenzi komzimba. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahl 236.

IPai SY, iNotarangelo LD. Ukuphazamiseka kokuzalwa komsebenzi we-lymphocyte. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 51.

USullivan KE, uBuckley RH. Iziphene eziphambili zemveliso yentsholongwane. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahluko 150.