Agammaglobulinemia

Agammaglobulinemia chirwere chinogarwa nhaka umo munhu ane mashoma pasi ekudzivirira immune immune mapuroteni anonzi immunoglobulins. Immunoglobulins rudzi rweantibody. Kuderera kwema antibodies aya kunoita kuti iwe uwane mikana yekutapukirwa.

Ichi chirwere chisingawanzo chinonyanya kubata varume. Iyo inokonzerwa nehurema hwemajini hunotadzisa kukura kweyakajairika, maseru ekukura emuviri anonzi B lymphocyte.

Nekuda kweizvozvo, muviri unogadzira mashoma (kana aripo) maemonoglobulin. Immunoglobulins inoita chinzvimbo chikuru mumhinduro dzemuviri, dzinodzivirira pakurwara uye hutachiona.

Vanhu vane chirwere ichi vanowana hutachiona kakawanda. Zvirwere zvakajairika zvinosanganisira izvo zvinokonzerwa nehutachiona senge Haemophilus influenzae, pneumococci (Streptococcus pneumoniae), uye staphylococci. Nzvimbo dzinozivikanwa dzehutachiona dzinosanganisira:

• Nzira yemudumbu
• Mapfundo
• Mapapu
• Ganda
• Yekumusoro kufema turakiti

Agammaglobulinemia inogarwa nhaka, zvinoreva kuti vamwe vanhu mumhuri yako vanogona kuve nemamiriro acho.

Zviratidzo zvinosanganisira zvinowanzoitika zvikamu zve:

• Bronchitis (hutachiona hwemhepo)
• Chirwere chisingaperi
• Conjunctivitis (hutachiona hwemaziso)
• Otitis media (yepakati nzeve hutachiona)
• Pneumonia (chirwere chemapapu)
• Sinusitis (sinus chirwere)
• Zvirwere zveganda
• Epamusoro mweya wekufema

Zvirwere zvinowanzoonekwa mumakore ekutanga mana ehupenyu.

Zvimwe zviratidzo zvinosanganisira:

• Bronchiectasis (chirwere umo mweya masaga mudiki mumapapu anokuvara uye kuwedzerwa)
• Chirwere pasina chikonzero chinozivikanwa

Iko kusagadzikana kunosimbiswa nekuongororwa kweropa kunoyera huwandu hwema immunoglobulins.

Miedzo inosanganisira:

• Kuyerera cytometry kuyera kutenderera B lymphocyte
• Immunoelectrophoresis - serum
• Yakawanda immunoglobulins - IgG, IgA, IgM (kazhinji inoyerwa nephelometry)

Kurapa kunosanganisira kutora matanho ekuderedza huwandu uye kuomarara kwezvirwere. Mishonga inorwisa mabhakitiriya inowanzo dikanwa kurapa hutachiona.

Immunoglobulins inopihwa kuburikidza netsinga kana jekiseni kusimudzira immune system.

Kuiswa kwemongo wemapfupa kunogona kutariswa.

Izvi zviwanikwa zvinogona kupa rumwe ruzivo nezve agammaglobulinemia:

• Immune Deficiency Foundation - primaryimmune.org
• National Sangano reRare Dambudziko - rarediseases.org/rare-diseases/agammaglobulinemia
• NIH / NLM Genetics Kumba Referensi - ghr.nlm.nih.gov/condition/x-linked-agammaglobulinemiaemia

Kurapa nema immunoglobulins kwakavandudza zvakanyanya hutano hweavo vane chirwere ichi.

Pasina kurapwa, hutachiona hwakanyanya hwakanyanya hunouraya.

Matambudziko ehutano anogona kuzoguma anosanganisira:

• Arthritis
• Chirwere chisingaperi kana chirwere chemapapu
• Eczema
• Intestinal malabsorption syndromes

Dana kuti usangane nemupi wako wezvehutano kana:

• Iwe kana mwana wako akasangana nezvirwere zvinowanzoitika.
• Iwe une nhoroondo yemhuri yeagammaglobulinemia kana imwe immunodeficiency disorder uye urikuronga kuva nevana. Bvunza mupi wezvekupa mazano nezvechibereko.

Genetic counselling inofanirwa kupihwa kune vangangozove vabereki vane nhoroondo yemhuri yeagammaglobulinemia kana mamwe matambudziko ekudzivirira muviri.

Bruton's agammaglobulinemia; X-yakabatana agammaglobulinemia; Immunosuppression - agammaglobulinemia; Kudzvinyirirwa - agammaglobulinemia; Immunosuppressed - agammaglobulinemia

• Antibodies

Cunningham-Rundles C. Zvirwere zvekutanga zvekudzivirira muviri. Mune: Goldman L, Schafer AI, eds. Goldman-Cecil Mushonga. 26th ed. Philadelphia, PA: Elsevier; 2020: chitsiko 236.

Pai SY, Notarangelo LD. Congenital kusagadzikana kweiyo lymphocyte basa. Muna: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Misimboti uye Maitiro. 7th ed. Philadelphia, PA: Elsevier; 2018: chits. 51.

Sullivan KE, Buckley RH. Kukanganisa kwekutanga kwekugadzira antibody. Mune: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Chinyorwa chePediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020: chitsiko 150.