I-Agammaglobulinemia

I-Agammaglobulinemia yisifo esizuzwe njengefa lapho umuntu enamazinga aphansi kakhulu amaprotheni omzimba wokuzivikela abizwa ngama-immunoglobulin. Ama-immunoglobulins awuhlobo lwe-antibody. Amazinga aphansi alawa masosha omzimba enza ukuthi ube sethubeni lokuthola izifo.

Lesi yisifo esingajwayelekile esithinta kakhulu abesilisa. Kubangelwa ukukhubazeka kwezakhi zofuzo okuvimba ukukhula kwamaseli omzimba avamile, avuthiwe abizwa ngama-B lymphocyte.

Ngenxa yalokho, umzimba wenza ama-immunoglobulin amancane (uma akhona) amancane kakhulu. Ama-immunoglobulin adlala indima enkulu ekuphenduleni komzimba, okuvikela izifo nokutheleleka.

Abantu abanalesi sifo baba nezifo kaninginingi. Ukutheleleka okuvamile kufaka phakathi okungenxa yebhaktheriya efana Haemophilus influenzae, i-pneumococci (I-Streptococcus pneumoniae), kanye ne-staphylococci. Izindawo ezivamile zokutheleleka zifaka:

• Ipheshana lesisu
• Amalunga
• Amaphaphu
• Isikhumba
• Umgudu wokuphefumula ongaphezulu

I-Agammaglobulinemia izuzwe njengefa, okusho ukuthi abanye abantu emndenini wakho bangaba nalesi simo.

Izimpawu zifaka phakathi iziqephu ezijwayelekile ze:

• I-bronchitis (ukutheleleka komoya)
• Uhudo olungamahlalakhona
• I-Conjunctivitis (ukutheleleka kweso)
• I-Otitis media (ukutheleleka kwendlebe okuphakathi)
• I-pneumonia (ukutheleleka kwamaphaphu)
• Sinusitis (isifo sesono)
• Ukutheleleka kwesikhumba
• Izifo ezingenhla zokuphefumula

Izifo zivame ukuvela eminyakeni emi-4 yokuqala yokuphila.

Ezinye izimpawu zifaka:

• I-Bronchiectasis (isifo lapho amasaka amancane omoya emaphashini elimala futhi akhuliswa)
• Isifuba somoya ngaphandle kwesizathu esaziwayo

Lesi sifo siqinisekiswa ukuhlolwa kwegazi okulinganisa amazinga ama-immunoglobulin.

Izivivinyo zifaka:

• Ukugeleza kwe-cytometry ukukala ukujikeleza kwama-lymphocyte B
• I-Immunoelectrophoresis - i-serum
• Ama-immunoglobulin amaningi - i-IgG, i-IgA, i-IgM (imvamisa ilinganiswa nge-nephelometry)

Ukwelashwa kubandakanya ukuthatha izinyathelo zokwehlisa inani nokuqina kwezifo. Imithi elwa namagciwane ivame ukudingeka ukwelapha ukutheleleka ngamagciwane.

Ama-immunoglobulin anikezwa ngomthambo noma ngomjovo ukukhulisa amasosha omzimba.

Kungase kubhekwe ukufakelwa umnkantsha.

Lezi zinsizakusebenza zinganikeza imininingwane eminingi nge-agammaglobulinemia:

• Isisekelo sokushoda komzimba - primaryimmune.org
• Inhlangano Kazwelonke Yezinkinga Ezingajwayelekile - rarediseases.org/rare-diseases/agammaglobulinemia
• Isethenjwa Sasekhaya se-NIH / NLM - ghr.nlm.nih.gov/condition/x-linked-agammaglobulinemia

Ukwelashwa ngama-immunoglobulin kuyithuthukise kakhulu impilo yalabo abanalesi sifo.

Ngaphandle kokwelashwa, izifo ezinamandla kakhulu ziyabulala.

Izinkinga zempilo ezingaba nomphumela zifaka:

• Isifo samathambo
• Isinus engalapheki noma isifo samaphaphu
• I-Eczema
• Ama-syndromes wamathumbu emathunjini

Shayela i-aphoyintimenti nomhlinzeki wakho wezokunakekelwa kwempilo uma:

• Wena noma ingane yakho nihlangabezane nokutheleleka okuvamile.
• Unomlando womndeni we-agammaglobulinemia noma enye i-immunodeficiency disorder futhi uhlela ukuba nezingane. Buza umhlinzeki mayelana nokwelulekwa ngezakhi zofuzo.

Ukwelulekwa ngofuzo kufanele kunikezwe labo abazoba ngabazali abanomlando womndeni we-agammaglobulinemia noma okunye ukuphazamiseka komzimba.

I-agammaglobulinemia kaBruton; I-agammaglobulinemia exhunywe ku-X; Ukucindezelwa komzimba - i-agammaglobulinemia; Ukucindezelwa okungalawuleki - i-agammaglobulinemia; Ukucindezelwa komzimba - i-agammaglobulinemia

• Amasosha omzimba

ICunningham-Rundles C. Izifo eziyinhloko zokuzivikela emzimbeni. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 236.

I-Pai SY, i-Notarangelo LD. Ukuphazamiseka kokuzalwa komsebenzi we-lymphocyte. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ama-eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 51.

USullivan KE, uBuckley RH. Ukukhubazeka okuyisisekelo kokukhiqizwa kwama-antibody. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 150.