Glucose-6-phosphate dehydrogenase le lava

O le Glucose-6-phosphate dehydrogenase (G6PD) le atoatoa o se tulaga e malepe ai sela mumu o le toto pe a aafia le tino i ni vailaʻau poʻo le mafatiaga o siama. E tuufaasolo, o lona uiga e tuufaasolo i totonu o aiga.

O le le atoatoa G6PD e tupu pe a misi se tagata pe le lava foi se enzyme e taʻua o le glucose-6-phosphate dehydrogenase. O lenei enzyme fesoasoani sela mumu toto e galue faʻalelei.

O le laititi o le G6PD e tau atu i le faʻatamaiaina o sela mumu toto. O lenei faiga e taʻua ole hemolysis. Afai o lenei gaioiga o loʻo tupu malosi, e taʻua o le hemolytic vaega. O vaega e masani ona puʻupuʻu. Aua o le tino e faʻaauau pea ona gaosia sela mumu toto, e i ai gaioiga masani.

O le faʻatamaia o le toto mumu e mafai ona faʻaosofia e faʻamaʻi pipisi, nisi o meaʻai (pei o fava pi), ma nisi vailaʻau, e aofia ai:

• Vailaau antimalarial pei o le quinine
• Aspirin (maualuga tui)
• Nonsteroidal anti-inflammatory vailaʻau (NSAIDs)
• Quinidine
• Fualaʻau Sulfa
• Antibiotics pei o quinolones, nitrofurantoin
  

O isi vailaʻau, e pei o i totonu o mothballs, mafai foi ona faʻaosofia se mea na tupu.

I le Iunaite Setete, o le G6PD le atoatoa e sili atu ona taatele i tagata uli nai lo papalagi. E tele le aʻafia o tamaloloa ile faʻamaʻi lenei nai lo fafine.

E te ono tupu i lenei tulaga pe a e:

• Aferika Amerika
• E i totonu o Sasaʻe tutotonu lelei, aemaise Kurdish poʻo Sephardic Iutaia
• O tamaloa
• Fai se talaʻaga aiga o le le lava

O se ituaiga o lenei faʻamaʻi e masani ai i papalagi o tupuʻaga Metitirani. Lenei fomu e fesoʻotaʻi foʻi ma matuitui vaega o hemolysis. Episodes e umi ma sili atu le ogaoga nai lo isi ituaiga o le faʻamaʻi.

Tagata e maua i lenei tulaga e le faʻaalia ni faʻailoga o le faʻamaʻi seʻi vagana ua aliali mai o latou sela mumu i nisi vailaʻau i meaai poʻo vailaʻau.

Faʻailoga e sili ona taatele i tamaloloa ma ono aofia ai:

• Uru pogisa
• Fiva
• Tiga i le manava
• Faʻateleina le manava ma le ate
• Le lavava
• Pallor
• Toto vave fatu
• Manava le mānava
• Lanu samasama samasama (jaundice)

E mafai ona faia se suʻega ile toto e siaki ai le maualuga ole G6PD.

O isi suʻega e ono faia e aofia ai:

• Tulaga Bilirubin
• Faitau atoa le toto
• Hemoglobin - miaga
• Haptoglobin tulaga
• LDH suʻega
• Methemoglobin faʻaititia faʻaititia
• Faitauga Retikulositula

Togafitiga ono aofia ai:

• Vailaau e togafitia ai se siama, pe a fai o iai
• Taofi ni vailaʻau e mafua ai le faʻatamaʻiaina o le toto mumu
• Toto, i nisi tulaga

I le tele o tulaga, hemolytic vaega alu ese na o latou.

I seāseā tupu, fatugao toilalo po o le oti ono tupu pe a maeʻa se hemolytic gaioiga faigata.

Valaʻau lau fomaʻi pe a fai o loʻo iai oe faʻailoga o lenei maʻi.

Valaʻau lau tautua pe a fai oe ua aʻafia ile le atoatoa G6PD ma faʻailoga e le mou pe a maeʻa togafitiga.

Tagata e le lava G6PD tatau ona matua aloese mai mea e ono faʻaosofia ai se mea. Talanoa i lau tautua e uiga i au vailaʻau.

Faʻamatalaga masani poʻo faʻataʻitaʻiga e mafai ona avanoa ia i latou o loʻo i ai se talaʻaga aiga o le tulaga.

G6PD le lava; Hemolytic anemia ona o le G6PD le lava; Anemia - hemolytic ona o le G6PD le atoatoa

• Toto sela

Gregg XT, Prchal JT. Enzymopathies mumu toto I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Faavae Autu ma Faʻataʻitaʻiga. 7th ed. Filatelefia, PA: Elsevier; 2018: mata 44.

Lissauer T, Carroll W. Haematological faʻafitauli. I le: Lissauer T, Carroll W, eds. Tusitusiga Tusitusiga o Paediatrics. 5th ed. Filatelefia, PA: Elsevier; 2018: mata 23.

Michel M. Autoimmune ma intravascular hemolytic anemias. I totonu: Goldman L, Schafer AI, eds. Faʻafomaʻi Goldman-Cecil. 26th lomiga. Filatelefia, PA: Elsevier; 2020: mata 151.