Agammaglobulinemia

Agammaglobulinemia waa cillad la iska dhaxlo oo qofku aad ugu hooseeyo heerarka difaaca jirka ee borotiinka loo yaqaan immunoglobulins. Immunoglobulins waa nooc ka mid ah antibody. Heerarka hoose ee unugyadaan jirka ah ayaa kaa dhigaya kuwo aad ugu badan tahay in aad qaaddo infekshanno.

Tani waa cillad naadir ah oo inta badan ku dhacda ragga. Waxaa sababa cillad hiddo-wade oo xannibaya koritaanka unugyada difaaca jirka ee caadiga ah, ee loo yaqaan B lymphocytes.

Sidaa darteed, jidhku wuxuu sameeyaa wax aad u yar (haddii ay jiraan) immunoglobulins. Immunoglobulins wuxuu door weyn ka ciyaaraa jawaab celinta difaaca jirka, kaasoo ka ilaaliya jirrada iyo infekshinka.

Dadka qaba cilladan waxay ku dhacdaa infekshan marar badan. Infekshannada caamka ah waxaa ka mid ah kuwa sababa bakteeriyada sida Haemophilus hargabka, pneumococci (Cudurka 'Stptoptoccus pneumoniae'), iyo staphylococci. Meelaha ugu badan ee cudurka lagu qaado waxaa ka mid ah:

• Mareenka caloosha iyo mindhicirka
• Laabatooyinka
• Sambabada
• Maqaarka
• Mareenka sare ee neef mareenka

Agammaglobulinemia waa la iska dhaxlaa, taas oo macnaheedu yahay in dadka kale ee qoyskaaga laga yaabo inay qabaan cudurka.

Calaamadaha waxaa ka mid ah dhacdooyin soo noqnoqda oo ah:

• Boronkiito (infekshinka hawo mareenka)
• Shuban joogto ah
• Conjunctivitis (infekshinka indhaha)
• Otitis media (caabuq dhegta dhexe)
• Oof-wareen (infekshinka sambabka)
• Sinusitis (infekshinka sanka)
• Caabuqyada maqaarka
• Infakshannada mareenka sare ee neef mareenka

Infekshannada waxay caadi ahaan muuqdaan 4-ta sano ee ugu horreysa nolosha.

Calaamadaha kale waxaa ka mid ah:

• Bronchiectasis (waa cudur ay kiishashka yar yar ee sanbabada ay ku waxyeeloobaan kuna weynaadaan)
• Neefta oo aan la garanayn sababteeda

Ciladda waxaa lagu xaqiijiyaa baaritaannada dhiigga ee cabbira heerarka immunoglobulins.

Tijaabooyinka waxaa ka mid ah:

• Socodka cytometry si loo cabbiro wareegga dhiigga ee B lymphocytes
• Immunoelectrophoresis - dhiig
• Qiyaasta immunoglobulins-IgG, IgA, IgM (badanaa lagu cabiro nephelometry)

Daaweyntu waxay ku lug leedahay qaadista tallaabooyin lagu yareynayo tirada iyo darnaanta infekshinka. Antibiyootikada ayaa badanaa loo baahan yahay si loo daaweeyo infekshannada bakteeriyada.

Immunoglobulins waxaa lagaa siiyaa xididka ama irbad si kor loogu qaado nidaamka difaaca jirka.

Beerista dhuuxa lafta ayaa laga fiirsan karaa.

Kheyraadkaani waxay bixin karaan macluumaad dheeri ah oo ku saabsan agammaglobulinemia:

• Aasaaska Dhimista Difaaca - primaryimmune.org
• Ururka Qaranka ee Dhibaatooyinka Fudud - rarediseases.org/rare-diseases/agammaglobulinemia
• NIH / NLM Tixraaca Hoyga Genetics - ghr.nlm.nih.gov/condition/x-linked-agammaglobulinemia

Daaweynta immunoglobulins waxay si weyn u wanaajisay caafimaadka dadka qaba cilladan.

Daaweyn la'aan, infekshannada ugu badan ee daran waa dilaa.

Dhibaatooyinka caafimaad ee ka dhalan kara waxaa ka mid ah:

• Arthritis
• Sinus joogto ah ama cudur sambabaha ah
• Canbaar
• Cilladaha malabsorption ee xiidmaha

Ballan wac wac bixiyahaaga daryeelka caafimaadka haddii:

• Adiga ama cunugaaga waxaad soo aragteen infekshanno isdaba joog ah
• Waxaad leedahay taariikh qoys oo ah agammaglobulinemia ama cillad kale oo xagga difaaca jirka ah waxaadna qorsheyneysaa inaad carruur dhasho. Weydii adeeg bixiyaha la-talinta hidda-sidaha.

La-talinta hidda-sidaha waa in loo fidiyaa waalidiinta mustaqbalka leh taariikh qoys oo ah agammaglobulinemia ama cuduro kale oo yaraada.

Bruton’s agammaglobulinemia; Agammaglobulinemia oo ku xiran X; Immunosuppression - agammaglobulinemia; Immunodepressed - agammaglobulinemia; Immunosuppressed - agammaglobulinemia

• Antibodies

Cunningham-Rundles C. Cudurada asaasiga u ah difaaca jirka. Gudaha: Goldman L, Schafer AI, eds. Daawada Goldman-Cecil. 26aad. Philadelphia, PA: Elsevier; 2020: cutubka 236.

Pai SY, Notarangelo LD. Xanuunada ku dhasha shaqada shaqada lymphocyte. Gudaha: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Mabaadi'da Aasaasiga ah iyo Tababarka. 7aad ed. Philadelphia, PA: Elsevier; 2018: cutubka 51.

Sullivan KE, Buckley RH. Cilladaha aasaasiga ah ee soosaarida antibody. Gudaha: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Buugga Buugga Caafimaadka Caruurta. 21aad ed. Philadelphia, PA: Elsevier; 2020: cutubka 150.