Agammaglobulinemia

Agammaglobulinemia ndimatenda obadwa nawo omwe munthu amakhala nawo otsika kwambiri mapuloteni oteteza chitetezo otchedwa ma immunoglobulins. Ma immunoglobulins ndi mtundu wa antibody. Kuchepetsa ma antibodies awa kumakupangitsani kuti mukhale ndi matenda.

Ichi ndi matenda osowa omwe amakhudza kwambiri amuna. Zimayambitsidwa ndi vuto la majini lomwe limalepheretsa kukula kwa maselo abwinobwino, otetezeka otchedwa B lymphocyte.

Zotsatira zake, thupi limapanga ma immunoglobulins ochepa (ngati alipo). Ma Immunoglobulins amatenga gawo lalikulu pama chitetezo amthupi, omwe amateteza kumatenda ndi matenda.

Anthu omwe ali ndi vutoli amatenga matenda mobwerezabwereza. Matenda omwe amapezeka ndi omwe amabwera chifukwa cha mabakiteriya monga Haemophilus influenzae, chibayo (Streptococcus pneumoniae), ndi staphylococci. Malo omwe matenda amapezeka ndi awa:

• Thirakiti lakumimba
• Magulu
• Mapapo
• Khungu
• Pamtunda thirakiti

Agammaglobulinemia yatengera, zomwe zikutanthauza kuti anthu ena m'banja lanu atha kukhala ndi vutoli.

Zizindikiro zimaphatikizaponso magawo a:

• Bronchitis (matenda opita pandege)
• Kutsekula m'mimba
• Conjunctivitis (matenda amaso)
• Otitis media (matenda akumakutu apakati)
• Chibayo (matenda am'mapapu)
• Sinusitis (matenda a sinus)
• Matenda a khungu
• Matenda opatsirana apamwamba

Matendawa amapezeka mzaka 4 zoyambirira za moyo.

Zizindikiro zina ndizo:

• Bronchiectasis (matenda omwe timapweya tating'onoting'ono m'mapapu timawonongeka ndikukula)
• Mphumu popanda chifukwa chodziwika

Matendawa amatsimikiziridwa ndi kuyezetsa magazi komwe kumayeza ma immunoglobulins.

Mayeso ndi awa:

• Kuyenda kwa cytometry kuyeza ma lymphocyte a B ozungulira
• Immunoelectrophoresis - seramu
• Ma immunoglobulins owerengeka - IgG, IgA, IgM (omwe nthawi zambiri amayeza ndi nephelometry)

Chithandizochi chimaphatikizapo kutenga njira zochepetsera kuchuluka kwa matenda. Maantibayotiki nthawi zambiri amafunikira kuti athetse matenda opatsirana ndi bakiteriya.

Ma immunoglobulins amaperekedwa kudzera mumitsempha kapena jakisoni kuti chitetezo chamthupi chitetezeke.

Kuika mafuta m'mafupa kungaganiziridwe.

Izi zitha kukupatsirani zambiri pa agammaglobulinemia:

• Chitetezo cha Immune Foundation - primaryimmune.org
• National Organisation for Rare Disways - rarediseases.org/rare-diseases/agammaglobulinemia
• Buku Lofotokozera la NIH / NLM Genetics - ghr.nlm.nih.gov/condition/x-linked-agammaglobulinemia

Chithandizo cha ma immunoglobulins chakweza kwambiri thanzi la iwo omwe ali ndi vutoli.

Popanda chithandizo, matenda opatsirana kwambiri amapha.

Mavuto omwe angabwere chifukwa chake ndi awa:

• Nyamakazi
• Sinus yanthawi yayitali kapena matenda am'mapapo mwanga
• Chikanga
• Matenda a m'matumbo osagwirizana ndi malabsorption

Itanani kuti mudzakumane ndi wokuthandizani ngati:

• Inu kapena mwana wanu mwakhala mukudwala matenda pafupipafupi.
• Muli ndi mbiri ya banja ya agammaglobulinemia kapena matenda ena amthupi ndipo mukukonzekera kukhala ndi ana. Funsani wopezayo za upangiri wa majini.

Upangiri wa chibadwa uyenera kuperekedwa kwa oyembekezera makolo omwe ali ndi mbiri yabanja ya agammaglobulinemia kapena mavuto ena amthupi.

Agammaglobulinemia ya Bruton; Agammaglobulinemia yolumikizidwa ndi X; Kutsekeka kwa chitetezo cha m'thupi - agammaglobulinemia; Kutetezedwa - agammaglobulinemia; Kutetezedwa ndi chitetezo chamthupi - agammaglobulinemia

• Ma antibodies

Cunningham-Rundles C. Matenda oyambira m'thupi. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. 26 wa. Philadelphia, PA: Elsevier; 2020: chap 236.

Pai SY, Notarangelo LD. Matenda obadwa nawo a lymphocyte ntchito. Mu: Hoffman R, Benz EJ, Silberstein LE, et al, olemba. Hematology: Mfundo Zoyambira ndi Zochita. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2018: chap 51.

Sullivan KE, Buckley RH. Zolakwika zoyambirira zopanga ma antibody. Mu: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 150.