Agammaglobulinemia

O le Agammaglobulinemia o se faʻamaʻi ua tuufaasolo mai i se tagata e matua maualalo lava vaega o le puipuiga o le tino puipuia o le tino e taʻu o immunoglobulins. Immunoglobulins o se ituaiga o vailaʻau. O le maualalo ole maualuga o nei vailaʻau e mafai ai ona maua oe ile faʻamaʻi pipisi.

O se faʻalavelave e seasea tupu e aʻafia ai aliʻi. E mafua mai i se faʻaletonu o le geno e poloka ai le tuputupu aʻe o sela masani o le sela ua puipuia e taʻua o le B lymphocytes.

O le iʻuga, o le tino e faʻaitiiti (pe a fai) ni immunoglobulins. Immunoglobulins faia se sao tele i le tali atu le puipuia, lea puipuia mai faʻamaʻi ma siama.

Tagata e maua ile faʻamaʻi lenei e tupu faʻasolosolo lava faʻamaʻi. O faʻamaʻi masani e aofia ai faʻamaʻi pipisi e pei o Haemophilus fulū, pneumococci (Streptococcus pneumoniae), ma staphylococci. Nofoaga masani o faʻamaʻi pipisi aofia ai:

• Alalaupapa Gastrointestinal
• Fesoʻotaʻiga
• Papa
• Paʻu
• Luga respiratory tract

Agammaglobulinemia e maua, o lona uiga o isi tagata i lou aiga ono maua i le faʻamaʻi.

Faʻailoga aofia ai vaega masani o:

• Bronchitis (faʻamaʻi pipisi o le alatele)
• Manava tatā
• Conjunctivitis (siama mata)
• Otitis media (ogatotonu taliga siama)
• Pomonia (siama o le mama)
• Sinusitis (siama siama)
• Siama o le paʻu
• Maualuga afaina auala respiratory

O faʻamaʻi pipisi e masani ona aliali mai i le muamua 4 tausaga o le olaga.

Isi faʻailoga aofia ai:

• Bronchiectasis (o se faʻamaʻi e afaina ai ma faʻalauteleina ia tamaʻi 'ea taga i mama)
• Asthma aunoa ma se lauiloa mafuaʻaga

O le faʻamaʻi ua faʻamaonia e suʻega toto e fua ai le maualuga o immunoglobulins.

O suʻega e aofia ai:

• Tafe cytometry e fua lapisi B limafositiva
• Immunoelectrophoresis - serum
• Aofaʻiga immunoglobulins - IgG, IgA, IgM (masani ona fuaina e le nephelometry)

Togafitiga aofia ai le faia o laʻasaga e faʻaititia ai le numera ma le ogaoga o faʻamaʻi. E masani ona manaʻomia vailaʻau faʻafomaʻi e togafitia ai siama siama.

Immunoglobulins e tuʻuina atu i se alatoto poʻo se tui e faʻamalosia ai le tino puipuia.

E mafai ona iloiloina le gaʻo o le ponaivi.

O nei fesoasoani e mafai ona maua ai nisi faʻamatalaga e uiga i le agammaglobulinemia:

• Puipuiga o le Puipuiga o le Puipuiga - primaryimmune.org
• National Organisation for Rare Disorder - rarediseases.org/rare-diseases/agammaglobulinemia
• NIH / NLM Genetics Fale Faʻasino Mataupu - ghr.nlm.nih.gov/condition/x-linked-agammaglobulinemia

Togafitiga ma immunoglobulins ua matua faaleleia le soifua maloloina oi latou oe maua lenei maʻi.

A aunoa ma togafitiga, sili ona ogaoga faʻamaʻi e leaga.

O faʻafitauli tau le soifua maloloina e ono aʻafia ai:

• Gugu
• Sinia faaumiumi poʻo faamaʻi mama
• Eczema
• Intan malabsorption syndromes

Valaʻau mo se taimi atofaina ma lau fomaʻi pe a fai:

• O oe poʻo lau tama na aʻafia i ni faʻamaʻi pipisi.
• E i ai sau talaʻaga aiga o le agammaglobulinemia poʻo se isi faʻaletonu o le tino ma o loʻo e fuafuaina e faia sau fanau. Fesili i le tautua e uiga i faufautua faufautua.

O le tuʻufaʻatasiga o fautuaga e tatau ona avatua i matua faʻamoemoeina ma le aiga talaʻaga o le agammaglobulinemia poʻo isi faʻaletonu o le puipuia o le tino.

Bruton’s agammaglobulinemia; X-fesoʻotaʻi agammaglobulinemia; Immunosuppression - agammaglobulinemia; Immunodepressed - agammaglobulinemia; Immunosuppressed - agammaglobulinemia

• Antibodies

Cunningham-Rundles C. Tulaga muamua faʻamaʻi e le puipuia ai lou tino. I totonu: Goldman L, Schafer AI, eds. Faʻafomaʻi Goldman-Cecil. 26th lomiga. Filatelefia, PA: Elsevier; 2020: chap 236.

Pai SY, Notarangelo LD. Faʻalavelave faʻafomaʻi o le lymphocyte function. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Faavae Autu ma Faʻataʻitaʻiga. 7th ed. Filatelefia, PA: Elsevier; 2018: chap 51.

Sullivan KE, Buckley RH. Peraimeri faʻaletonu o le gaosia o le vailaau I le: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Tusitusiga o Pediatrics. 21 ed. Filatelefia, PA: Elsevier; 2020: chap 150.