Agammaglobulinemia

ʻO ka Agammaglobulinemia kahi maʻi hoʻoilina i loaʻa i kahi kanaka nā haʻahaʻa haʻahaʻa loa o nā pale pale pale pale i kapa ʻia immunoglobulins. ʻO Immunoglobulins kahi ʻano antibody. ʻO nā pae haʻahaʻa o kēia mau mea ʻino e loaʻa pinepine ai i nā maʻi.

He maʻi laha ʻole kēia e hoʻopili nui i nā kāne. Hoʻokumu ʻia ia e ka ʻāpana kīnā ʻole e ālai i ka ulu ʻana o nā ʻea pale pale makua i kapa ʻia ʻo lymphocytes B.

ʻO ka hopena, hana liʻiliʻi ke kino (inā kekahi) immunoglobulins. He kuleana nui nā Immunoglobulins i ka pane pale ʻana, ka mea e pale ai i nā maʻi a me nā maʻi.

Hoʻonui pinepine ka poʻe me kēia maʻi i nā maʻi. Loaʻa nā maʻi āpau i nā mea ma muli o nā bacteria e like me Haemophilus influenzae, pneumococci (ʻO Streptococcus pneumoniae), a staphylococci. ʻO nā pūnaewele maʻamau o ka maʻi pū kekahi:

• ʻAha Gastrointestinal
• Hui pū
• Lungs
• ʻIli
• ʻAha hanu luna

Hoʻoilina ʻo Agammaglobulinemia, ʻo ia ka manaʻo i loaʻa i nā poʻe ʻē aʻe i kou ʻohana.

ʻO nā hōʻailona me nā ʻāpana pinepine o:

• Bronchitis (maʻi lele)
• Pohā ʻōpū
• Conjunctivitis (maka maʻi)
• Otitis media (ma waena o ka pepeiao maʻiʻaʻai)
• Ponia (maʻi hū)
• Sinusitis (sinus maʻiʻaʻai)
• Nā maʻi ʻili
• ʻO nā maʻi maʻi hanu luna

ʻIke pinepine nā maʻi i nā makahiki 4 mua o ke ola.

ʻO nā ʻōuli ʻē aʻe e like me:

• ʻO Bronchiectasis (kahi maʻi kahi e ʻino ai a hoʻonui ʻia ai nā ʻeke ea liʻiliʻi i nā akemāmā)
• Asthma me ke kumu ʻole i ʻike ʻia

Hōʻoia ʻia ka maʻi e nā hōʻike koko e ana i nā pae o immunoglobulins.

Loaʻa i nā hoʻāʻo:

• ʻO ka cytometry kahe e ana i nā lymphocytes B e hoʻopolo nei
• Immunoelectrophoresis - serum
• Nā immunoglobulins nui - IgG, IgA, IgM (ana pinepine ʻia e nephelometry)

Hoʻopili ka mālama ʻana i nā hana e hōʻemi ai i ka helu a me ke koʻikoʻi o nā maʻi. Pono pinepine nā lāʻau antibiotic e mālama i nā maʻi bacteria.

Hāʻawi ʻia nā Immunoglobulins ma o ke aa a i ʻole ke kuʻi ʻana e hoʻonui i ka ʻōnaehana pale.

E noʻonoʻo ʻia kahi hoʻoneʻe iwi iwi.

Hiki i kēia mau kumuwaiwai ke hāʻawi i ka ʻike hou aku e pili ana i ka agammaglobulinemia:

• ʻO ka Immune Deficit Foundation - primaryimmune.org
• National Organization for Rare Disorder - rarediseases.org/rare-diseases/agammaglobulinemia
• NIH / NLM Genetics Home Reference - ghr.nlm.nih.gov/condition/x-linked-agammaglobulinemia

ʻO ka mālama ʻana me nā immunoglobulins i hoʻomaikaʻi maikaʻi i ke olakino o ka poʻe i loaʻa i kēia maʻi.

Me ka mālama ʻole ʻia, make nui nā maʻi ʻino loa.

ʻO nā pilikia olakino e hopena ʻia paha:

• Pākuʻi
• Sinus maʻi a maʻi pulmonary paha
• Eczema
• Nā syndrome malabsorption ʻōpū

Kāhea i kahi manawa me kāu kahu mālama ola inā:

• Ua ʻike ʻoe a ʻo kāu keiki paha i nā maʻi maʻi pinepine.
• Loaʻa iā ʻoe ka moʻolelo ʻohana o agammaglobulinemia a i ʻole kekahi maʻi ʻino e aʻe a ke hoʻolālā nei ʻoe i nā keiki. E nīnau i ka mea nāna e hāʻawi e pili ana i ka ʻōlelo aʻo kūpuna.

Pono e hāʻawi ʻia i nā ʻōlelo aʻo kūmole i nā mākua e hiki mai ana me ka moʻolelo ʻohana o agammaglobulinemia a i ʻole nā ​​maʻi maʻi ʻino.

ʻO ka agammaglobulinemia a Bruton; Agammaglobulinemia pili X; Immunosuppression - agammaglobulinemia; Immunodepressed - agammaglobulinemia; Immunosuppressed - agammaglobulinemia

• Nā Antibodies

ʻO Cunningham-Rundles C. Nā maʻi pale maʻi ʻole. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 236.

Pai SY, Notarangelo LD. Nā maʻi kūlohelohe o ka hana lymphocyte. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 51.

Sullivan KE, ʻO Buckley RH. Nā kīnā mua o ka hana antibody. I: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. ʻO Puka Textbook of Pediatrics ʻo Nelson. 21st ed. Piladelapia, PA: Elsevier; 2020: chap 150.