Albinism
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Albinism o se faaletonu o le gaosiaina o melanin. Melanin o se natura vailaʻau i le tino e maua ai lanu i lou lauulu, paʻu, ma iris o le mata.
Albinism tupu pe a o se tasi o le tele o faʻafitauli genes faia le tino le mafai ona gaosia pe tufatufaina melanin.
O nei faaletonu e mafai ona pasi i lalo (tofi) e ala i aiga.
O le ituaiga sili ona ogaoga o le albinism e taʻua ole albinism oculocutaneous. Tagata i lenei ituaiga o albinism i papaʻe po o le piniki laulu, paʻu, ma iris lanu. E i ai foi a latou faʻafitauli vaʻai.
O le isi ituaiga albinism, e taʻua o le ocular albinism type 1 (OA1), e naʻo mata e aafia ai. O le paʻu o le tagata ma le lanu o mata e masani ona i ai i le tulaga masani. Peitai, o le suʻega o mata o le a faʻaalia ai e leai se valivali i le pito i tua o le mata (retina).
Hermansky-Pudlak syndrome (HPS) o se ituaiga o albinism mafua mai i le suia i se tasi ituaiga. E mafai ona tupu ile faʻamaʻi toto, male mama, fatugaʻo ma faʻamaʻi ole manava.
O se tagata ma albinism ono i ai se tasi o nei faʻailoga:
- Leai se lanu ile lauulu, paʻu, poʻo iris o le mata
- Malamalama nai lo masani paʻu ma lauulu
- Faʻaputuga o le misi o le paʻu lanu
Tele ituaiga o albinism e fesoʻotaʻi ma faʻailoga nei:
- Kolose mata
- Malamalama malamalama
- Gaioiga vave a le mata
- Faʻafitauli vaʻai, poʻo le aoga tauaso
Ole suʻega ole genetic e maua ai le auala saʻo e faʻamaonia ai le albinism. O ia faʻataʻitaʻiga e aoga pe a fai e iai sau talaʻaga aiga o le albinism. E aoga foi mo nisi vaega o tagata ua lauiloa e maua i le faʻamaʻi.
E mafai foʻi ona suʻesuʻe e lau tausi soifua maloloina le faʻavae e fuafua i le aliali mai o lou paʻu, lauulu, ma ou mata. O se fomaʻi mata e taʻu o le fomaʻi mata e ono faia se electroretinogram. Ole suʻega lea e mafai ona faʻaalia ai faʻafitauli vaʻai e fesoʻotaʻi ma le albinism. O se suʻega e taʻua o se faʻaaliga vaʻaia ono mafai ona aoga tele pe a le mautinoa le faʻamaoniga.
O le faʻamoemoe o togafitiga o le faʻamamaina lea o faʻailoga.E faʻamoemoe i le ogaoga o le faʻamaʻi.
Togafitiga aofia ai le puipuia o le paʻu ma mata mai le la. Ina ia faia lenei:
- Faʻaitiitia le lamatiaga o le la i le 'aloʻese mai le la, faʻaaogaina le sunscreen, ma le ufiufi atoa i lavalava pe a faʻaalia e le la.
- Faʻaoga sunscreen ma le maualuga sun protection factor (SPF).
- Fai mata tioata (UV puipuia) e fesoasoani faʻamamaina malamalama malamalama.
O tioata e masani ona faʻatonuina e faʻasaʻo ai faʻafitauli vaʻai ma tulaga o mata. O taotoga maso maso e masani ona fautuaina e faʻasaʻo le le lelei o gaioiga o mata.
O vaega o loʻo sosoʻo e mafai ona tuʻuina mai nisi faʻamatalaga ma mea e maua:
- Faʻalapotopotoga a le Atunuʻu mo Albinism ma Hypopigmentation - www.albinism.org
- NIH / NLM Genetics Fale Faʻasino Mataupu - ghr.nlm.nih.gov/condition/ocular-albinism
Albinism e le masani ona aʻafia le olaga atoa. Peitaʻi, e mafai e le HPS ona faʻapuʻupuʻu le umi o le ola o se tagata ona o faʻamaʻi o le mama poʻo faʻafitauli tau toto.
Tagata e iai albinism ono faʻatapulaʻaina ia latou gaioiga aua latou te le mafai faʻapalepaleina le la.
O nei faʻalavelave e mafai ona tupu:
- Faʻaitiitia le vaʻai, tauaso
- Kanesa o le paʻu
Valaʻau lau tautua peʻa iai sau albinism poʻo ni faʻailoga e pei ole malamalama malamalama e mafua ai le le faʻamafanafana. Valaʻau foʻi pe a e maitauina ni suiga i paʻu atonu o se vavega o le kanesa o le paʻu.
Talu ai o le albinism e maua mai, o le faufautua o kenera e taua. Tagata e iai le talaʻaga aiga o le albinism poʻo le malamalama lanu e tatau ona mafaufau i fautuaga faʻafesagaʻi.
Albinism o le Oculocutaneous; Albinism okular
Melanin
Cheng KP. Fomaʻi faʻapitoa. I: Zitelli BJ, McIntire SC, Nowalk AJ, eds. Zitelli ma Davis 'Atlas o Pediatric Physical Diagnosis. 7th ed. Filatelefia, PA: Elsevier; 2018: chap 20.
Joyce JC. O faʻamaʻi ninii 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 672.
Paller AS, Mancini AJ. Faʻaletonu o pigmentation. I: Paller AS, Mancini AJ, eds. Hurwitz Togafitiga Pediatric Dermatology. 5th ed. Filatelefia, PA: Elsevier; 2016: mata 11.