Agammaglobulinemia

Agammaglobulinemia ke lefu le futsitsoeng leo ho lona motho a nang le maemo a tlase haholo a liprotheine tsa sesole sa 'mele tse bitsoang immunoglobulins. Li-immunoglobulin ke mofuta oa antibody. Maemo a tlase a lithibela-mafu tsena a etsa hore ho be bonolo ho fumana tšoaetso.

Ena ke lefu le sa tloaelehang le amang haholo banna. E bakoa ke bofokoli ba liphatsa tsa lefutso bo sitisang kholo ea lisele tse itšireletsang mafung tse tloaelehileng tse bitsoang B lymphocyte.

Ka lebaka leo, 'mele o etsa li-immunoglobulin tse nyane haholo (haeba li teng). Li-immunoglobulin li bapala karolo e kholo karabong ea 'mele ea ho itšireletsa mafung, e sirelletsang mafu le tšoaetso.

Batho ba nang le bothata bona ba ba le tšoaetso khafetsa. Ts'oaetso e tloaelehileng e kenyelletsa tse bakoang ke baktheria joalo ka Haemophilus influenzae, pneumococci (Streptococcus pneumoniae), le staphylococci. Libaka tse tloaelehileng tsa tšoaetso li kenyelletsa:

• Karolo ea meno
• Manonyeletso
• Matšoafo
• Letlalo
• Karolo e kaholimo ea phefumoloho

Agammaglobulinemia e futsitsoe, ho bolelang hore batho ba bang lelapeng la hau ba ka ba le boemo bona.

Matšoao a kenyelletsa liketsahalo tsa khafetsa tsa:

• Bronchitis (tšoaetso ea sefofane)
• Letšollo le sa foleng
• Conjunctivitis (tšoaetso ea mahlo)
• Otitis media (tšoaetso ea tsebe e bohareng)
• Pneumonia (tšoaetso ea matšoafo)
• Sinusitis (tšoaetso ea sinus)
• Tšoaetso ea letlalo
• Matšoao a kaholimo a phefumoloho

Hangata tšoaetso e hlaha lilemong tse 4 tsa pele tsa bophelo.

Matšoao a mang a kenyelletsa:

• Bronchiectasis (lefu leo ​​ho lona mekotla e menyenyane ea moea matšoafong e senyehang le ho holisoa)
• Pherekano ntle le lebaka le tsejoang

Boloetse bona bo netefatsoa ke liteko tsa mali tse metha litekanyo tsa li-immunoglobulin.

Liteko li kenyelletsa:

• Phallo ea cytometry ho lekanya li-lymphocyte tsa B tse potolohang
• Immunoelectrophoresis - serum
• Li-immunoglobulin tse ngata - IgG, IgA, IgM (hangata e lekanyetsoa ke nephelometry)

Kalafo e kenyelletsa ho nka mehato ea ho fokotsa palo le ho tiea ha tšoaetso. Hangata lithibela-mafu lia hlokahala ho phekola tšoaetso ea baktheria.

Li-immunoglobulin li fanoa ka methapo kapa ka ente ho matlafatsa sesole sa 'mele.

Ho kanna ha nahanoa ka phetoho ea moko oa masapo.

Lisebelisoa tsena li ka fana ka tlhaiso-leseling e batsi ka agammaglobulinemia

• Motheo oa Khaello ea Immune - primaryimmune.org
• Mokhatlo oa Naha oa Mathata a Rare - rarediseases.org/rare-diseases/agammaglobulinemia
• Referense ea Lehae la NIH / NLM - genh.nlm.nih.gov/condition/x-linked-agammaglobulinemia

Kalafo e nang le li-immunoglobulin e ntlafalitse bophelo ba ba nang le bothata bona haholo.

Ntle le kalafo, tšoaetso e matla haholo ea bolaea.

Mathata a bophelo bo botle a ka hlahang a kenyelletsa:

• Ramatiki
• Sinus e sa foleng kapa lefu la pulmonary
• Eczema
• Matšoao a mala a mala

Bitsa kopano le mofani oa tlhokomelo ea bophelo haeba:

• Uena kapa ngoana oa hau le kile la tšoaetsoa hangata.
• U na le nalane ea lelapa ea agammaglobulinemia kapa bothata bo bong ba ho itšireletsa mafung 'me u rera ho ba le bana. Botsa mofani ka tlhabollo ea lefutso.

Keletso ea lefutso e lokela ho fuoa bao e tla ba batsoali ba nang le nalane ea lelapa ea agammaglobulinemia kapa mathata a mang a ho itšireletsa mafung.

Agammaglobulinemia ea Bruton; Agammaglobulinemia e amanang le X; Khatello ea li-immune - agammaglobulinemia; Khatello ea boits'oaro - agammaglobulinemia; Ho itšireletsa mafung - agammaglobulinemia

• Masole a mmele

Cunningham-Rundles C. Maloetse a mantlha a ho itšireletsa mafung. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 236.

SI ea Pai, Notarangelo LD. Mathata a tlhaho ea mosebetsi oa lymphocyte. Ka: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Melao-motheo le Tloaelo ea Motheo. La 7th. Philadelphia, PA: Elsevier; 2018: khaolo ea 51.

Sullivan KE, Buckley RH. Liphoso tsa mantlha tsa tlhahiso ea antibody. Ka: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, bahlophisi. Buka ea Nelson ea Pediatrics. La 21 Ed. Philadelphia, PA: Elsevier; 2020: khaolo ea 150.