Dzidzɔ si nèkpɔna ne èlé ŋku ɖe viwò si wodzi yeyee ŋu la nye nusi womate ŋu aɖɔ o, alo? Gake emegbe ne èse dodokpɔ siwo wowɔna le kɔdzi le vidzĩa dzidzi vɔ megbe dometɔ aɖewo ŋu nya la, vɔvɔ̃ ɖoa wò vie eye nèdi be yeanya nu tso eŋu. Dodokpɔ mawo dometɔ ɖekae nye PKU dodokpɔ. Ðewohĩ mèse ŋkɔ sia ŋkɔ kpɔ o. Gake esia nye dodokpɔ vevi aɖe ŋutɔ. Egbea míele nu ƒom tso nɔnɔme sia si mebɔ o si woyɔna be PKU ŋu, gake ne wokpɔe dze sii kaba la, woate ŋu aɖu edzi keŋkeŋ eye vidzĩa ate ŋu anɔ agbe le lãmesẽ me.
Kpuie ko la, nukae nye PKU (Phenylketonuria)?
PKU, alo Phenylketonuria, nye domenyiŋusẽfianu ƒe dɔléle si mebɔ o . Dzilawo gbɔe wònyi. Vidzĩ si ŋu dɔléle sia le mate ŋu aɖu amino acid phenylalanine, si le protein nuɖuɖu siwo míeɖuna me nyuie, alo agbãe o.
Bu míaƒe ŋutilã be enye dɔwɔƒe gã aɖe. Nuɖuɖu si míeɖunae nye nu xoxo siwo míetsɔ de eme. Dɔwɔla sue siwo woyɔna be enzymes zãa nu xoxo siawo tsɔ wɔa nusiwo míaƒe ŋutilã hiã. Vidzĩ si ŋu PKU le la mewɔa dɔlékui tɔxɛ aɖe si woyɔna be phenylalanine hydroxylase (PAH), si gblẽa phenylalanine me o alo mewɔa kura o.
Eyata nukae dzɔna? Phenylalanine, si wokpɔna tso no me notsi me eye emegbe tso nuɖuɖu siwo vidzĩa ɖuna me la dzea ƒoƒo ɖe ʋua me le esi teƒe be woaɖee ɖa le ŋutilãa me. Ne phenylalanine si ƒoa ƒu alea dzi ɖe edzi la, eva zua aɖi na vidzĩa ƒe ahɔhɔ̃ . Ne womewɔ atike na esia o la, ate ŋu agblẽ nu le ahɔhɔ̃a ŋu eye wòahe kuxi sesẽwo abe susu ƒe dɔmawɔmawɔ nyuie kple tsitsi ƒe megbetsitsi ene vɛ.
Gake nu nyuitɔ kekeake si le afisiae nye be le dukɔ geɖe me, siwo dome Sri Lanka hã le la, kɔdziwo wɔa PKU dodokpɔ sia le vidzĩ ɖesiaɖe si wodzi ŋu. Eyata woate ŋu ade dzesi dɔlélea kaba. Esi wònye be woate ŋu adze atikewɔwɔ gɔme ne wonya de dzesii ko ta la, mɔnukpɔkpɔ sua vidzĩa si be wòatsi le mɔ si sɔ nu eye wòanɔ lãmesẽ me, si ana dzesi sesẽwo nagadze o.
Nukae nye PKU ƒe dzesiwo?
Vidzĩ si ŋu PKU le la dzena le lãmesẽ me keŋkeŋ le edziɣi. Eya ta, le gɔmedzedzea me la, ɖewohĩ tɔtrɔ aɖeke maɖe dzesi o. Ne womewɔ atike nɛ o la, dzesiawo adze dzedze gɔme tso ɣleti etɔ̃ va ɖo ade. Le ɣeyiɣi sia me la, àte ŋu ade dzesii be viwòa ƒe tsitsi tsi megbe vie.
Kaka vidzĩ naxɔ abe ƒe ɖeka ene la, ne womewɔ atike nɛ o la, dzesi siwo gbɔna ate ŋu adze ƒã.
| Dzesi si dzena | Nuɖᴐɖᴐ |
|---|---|
| Ʋeʋẽ wɔnuku aɖe | Ʋeʋẽ wɔnuku aɖe si me ʋuʋudedi le si dona tso vidzĩa ƒe gbɔgbɔ, fifia, alo aɖuɖɔ me. |
| Amadedewo trɔna | Vidzĩa ƒe ŋutigbalẽ, taɖa, kple ŋkuwo ƒe amadede le blɔ wu ne wotsɔe sɔ kple ƒomea me tɔ mamlɛawo. |
| Ŋutilãmedɔlélewo | Ŋutilãmedɔlélewo abe eczema ene. |
| Tsitsi ƒe megbetsitsi | Kpekpeme kple kɔkɔme si medzina ɖe edzi le ƒe si woxɔ nu o (Tsitsi ƒe megbetsitsi). |
| Nɔnɔme bubuwo | Dzesiwo abe ʋuʋudedi, ʋuʋudedi, kple ʋuʋudedi ene. |
| Dzesi sesẽ siwo ate ŋu ado mo ɖa ne womewɔ atike na wo o | |
| Nuwɔna ƒe kuxiwo | Woƒe ʋuʋu enuenu, nuwɔna ŋumaɖɔɖotɔe, kple agbagbadzedze be yewoawɔ nuvevi yewo ɖokui. |
| Dɔwɔwɔ fũ akpa | Dodo vevie akpa be woanɔ teƒe ɖeka. |
| Alɔ̃madɔmadɔ | Nɔnɔme siwo le abe alɔ̃dɔdɔ ene . |
Aleke fufɔfɔ kpɔa ŋusẽ ɖe vidada si ŋu PKU le dzii?
Esia nye nya vevi aɖe ŋutɔ. Ne nyɔnu si ŋu PKU le fɔ fu eye womekpɔ ŋusẽ ɖe eƒe PKU nɔnɔmea dzi le ɣeyiɣi ma me o la, ate ŋu agblẽ nu le vidzĩ si le vidzidɔa me ŋu. Phenylalanine ƒe agbɔsɔsɔ si dzi ɖe edzi le vidadaa ƒe ʋu me ate ŋu agblẽ nu le vidzĩa ŋu.
Esia nana fuɖeɖe ƒe afɔkua dzina ɖe edzi eye ate ŋu ahe kuxi vovovowo hã vɛ na vidzĩ si le dɔme.
- Dzidɔ si wodzina
- Tɔtrɔ aɖewo le mo ƒe nɔnɔme me
- Vidzidzi ƒe kpekpeme si bɔbɔ
- Ta sue aɖe (microcephaly) .
Gake mègavɔ̃ o. Ne PKU le ŋuwò, ƒo nu kple wò ɖɔkta eye nèwɔ ɖe nuɖuɖu tɔxɛ aɖe dzi le wò fufɔɣi katã la, wò hã àte ŋu adzi vi si le lãmesẽ me keŋkeŋ .
Nukatae PKU va doa mo ɖa? Nuka gbɔe wòtso?
Abe alesi míedzro eme va yi ene la, PKU nye domenyiŋusẽfianu ƒe dɔléle. Etsoa domenyiŋusẽfianu si woyɔna be `PAH` ƒe tɔtrɔ le míaƒe ŋutilã me gbɔ. `PAH` domenyiŋusẽfianu sia fiaa mɔ míaƒe ŋutilã be wòawɔ enzyme si gblẽa phenylalanine me. Ne domenyiŋusẽfianu ƒe tɔtrɔ aɖe va la, womewɔa enzyme ma o, alo nusi gblẽ le eƒe wɔwɔme ŋu.
Wonyia dɔléle sia ƒe dome le autosomal recessive pattern me. Kpuie ko la, ezɔ ale:
Hafi vidzĩ nate ŋu axɔ dɔlélea la, ele be woaxɔ domenyiŋusẽfianu si wogbugbɔ trɔ la ƒe kɔpi ɖeka tso wo dada kple wo fofo siaa gbɔ . Ne dzila ɖeka koe xɔ domenyiŋusẽfianua la, vidzĩa maxɔ dɔlélea o. Gake dzila ma ate ŋu anye amesi tsɔe. Esia fia be ɖewohĩ dzesi aɖeke manɔ wo ŋu o, gake woate ŋu atsɔ domenyiŋusẽfianua ayi na wo viwo le etsɔme.
Aleke ɖɔktawo wɔa dea dzesi PKU?
PKU nyanya va le bɔbɔe wu fifia le vidzĩ yeyewo dodokpɔ ta.
Le gaƒoƒo 24 va ɖo 72 le viwòa dzidzi megbe la, ɖɔkta alo dɔnɔdzikpɔla si le kɔdzi atsɔ aŋe sue aɖe aƒo viwòa ƒe afɔkpodzi eye wòaƒo ʋu ʋɛ aɖewo nu ƒu ɖe agbalẽvi tɔxɛ aɖe dzi. Woyɔa esia be ‘afɔkpodzi ƒoƒo dodokpɔ.’ Wozãa ʋu sia tsɔ léa ŋku ɖe phenylalanine ƒe agbɔsɔsɔ si le viwòa ƒe ʋu me ŋu.
Ne phenylalanine ƒe agbɔsɔsɔ le ʋu me sɔ gbɔ la, wogawɔa ʋu kple aɖuɖɔ dodokpɔ bubuwo tsɔ ɖoa kpe dɔlélea dzi. Ɣeaɖewoɣi la, woate ŋu awɔ domenyiŋusẽfianu ƒe dodokpɔ atsɔ ade dzesi domenyiŋusẽfianu ƒe tɔtrɔ si koŋ hea dɔlélea vɛ.
Nukawoe nye atike siwo wozãna na PKU?
Atike aɖeke meli na PKU o. Gake woate ŋu akpɔ nɔnɔmea gbɔ nyuie ŋutɔ le agbemeŋkekewo katã me . Dzesiawo ate ŋu agadze ne wodzudzɔ atikewɔwɔ. Dɔdamɔnu vevitɔe nye nuɖuɖu tɔxɛ aɖe kple ɣeaɖewoɣi atikewo zazã.
1. Nuɖuɖu tɔxɛ si me phenylalanine mesɔ gbɔ ɖo o
Esiae nye PKU dzikpɔkpɔ ƒe akpa vevitɔ kple vevitɔ kekeake .Ele be amesi ŋu PKU le nawɔ ɖe nuɖuɖu si me phenylalanine mesɔ gbɔ ɖo o dzi le eƒe agbemeŋkekewo katã me.
Esi wònye be phenylalanine nye protein ƒe akpa aɖe ta la, ele be woaɖo seɖoƒe na nuɖuɖu siwo me protein sɔ gbɔ ɖo .
- Lã, tɔmelã, koklolã
- Aziwo
- Notsi kple nyinotsibabla (yogurt, cheese) .
- Nukuwo abe lãgbalẽ kple chickpeas ene
- Soya-nuɖuɖuwo
- Nutsetsewo
Gake esi wònye be ŋutilãa hiã protein agbɔsɔsɔme aɖe hafi wòatsi ta la, ele be nàƒo nu kple nuɖuɖuŋutinunyala aɖe be wòanya protein agbɔsɔsɔme si nàte ŋu aɖu. Woawɔ nuɖuɖu ƒe ɖoɖo si me nunyiame le si sɔ na ɖevi/ame si ŋu PKU le.
Vevietɔ ŋutɔ: Ele be woaƒo asa na vividoɖeameŋunu si wowɔ kple asi si nye ‘aspartame’ keŋkeŋ. Aspartame gbãna le ŋutilãa me wòzua phenylalanine. Ate ŋu anɔ aha, nuɖuɖu, aŋe, vitamin aɖewo, kple kɔdzitsi geɖe siwo dzi woŋlɔ ɖo be ‘nuɖuɖu’ alo ‘sukli mele o’ me. Eyata ele vevie ŋutɔ be nàxlẽ nusi woŋlɔ ɖe edzi nyuie hafi aɖu nu alo ano naneke.
Ele be woadze vidzĩ siwo wodzi yeyee siwo ŋu PKU le gɔme le notsi tɔxɛ aɖe si me phenylalanine mele o zazã me enumake.
2. Atikewo zazã
Le PKU dɔnɔ aɖewo gome la, atikewo ate ŋu akpe ɖe ame ŋu tsɔ kpe ɖe nuɖuɖu ŋu. Ðɔkta ƒe aɖaŋuɖoɖo ko hafi wòle be woazã esiawo.
- Sapropterin dihydrochloride (Kuvan®): Atike sia kpena ɖe dɔnɔ aɖewo ŋu be wogbãa phenylalanine le ŋutilã me. Gake ele be nàyi edzi awɔ ɖe nuɖuɖu aɖe dzi esime nèle atike sia nom.
- Pegvaliase (Palynziq®): Esia nye atike si wokafu na dɔnɔ tsitsiwo. Enaa enzyme aɖe si gblẽa phenylalanine me la ŋutilãa.
Ðe wòanya wɔ be woanɔ agbe si sɔ kple PKUa?
Ẽ, ele nenema godoo! Togbɔ be PKU nye dɔléle si nɔa ame ƒe agbemeŋkekewo katã me hã la, ne wokpɔe kaba eye wokpɔ egbɔ nyuie la, woate ŋu aɖe ŋusẽ si wòkpɔna ɖe lãmesẽ dzi dzi akpɔtɔ.
Nuɖuɖu si me protein mesɔ gbɔ ɖo o dzi wɔwɔ le wò agbemeŋkekewo katã me ate ŋu asesẽ ɣeaɖewoɣi. Gake menye wò ɖeka koe o. Wò ɖɔkta kple nuɖuɖuŋutinunyala le klalo ɣesiaɣi be yewoakpe ɖe ŋuwò. Azɔ hã, ne ège ɖe kpekpeɖeŋunahawo me kple ame bubu siwo ŋu PKU le ate ŋu akpe ɖe ŋuwò nàgblɔ nuteƒekpɔkpɔwo eye ŋusẽ nasu asiwò.
Ðewohĩ èkpɔ ‘nuɖuɖu’ aɖewo nono ƒe aŋetuwo kple aŋekotoku siwo me nuxlɔ̃ame aɖe le be "Phenylketonurics: Phenylalanine le eme." Esia nye be woatsɔ ana amesiwo ŋu PKU le nanya be aspartame, si me phenylalanine le, le atike la me.
Ne wò alo wò ƒomea le nuteɖeamedzi me tom le wò PKU nɔnɔmea ta la, mègahe ɖe megbe le nuƒoƒo kple susumedɔléle ŋuti aɖaŋuɖola aɖe me o . Susu ƒe lãmesẽ le vevie abe alesi ŋutilã ƒe lãmesẽ le vevie ene.
Gbedasi si Wotsɔ Yi Aƒeme
- PKU nye domenyiŋusẽfianu ƒe dɔléle si woate ŋu ada eye woate ŋu aɖu edzi. Mègavɔ̃e madzemadzee o.
- Dɔlélea kpɔkpɔ kaba to vidzĩ yeyewo dodokpɔ me nye nu vevitɔ si ana woadae dzidzedzetɔe.
- Atikewɔwɔ vevitɔe nye be woawɔ ɖe nuɖuɖu tɔxɛ si me phenylalanine mesɔ gbɔ ɖo o dzi le agbe me katã.
- Ne èle nuɖuɖu kple nunono siwo woŋlɔ ɖe edzi be ‘Nuɖuɖu’ alo ‘Sukli-manɔmee’ ƒlem la, lé ŋku ɖe eŋu ɣesiaɣi be aspartame le wo me hã.
- Wɔ ɖe mɔfiame siwo wò ɖɔkta kple nuɖuɖuŋutinunyala na wò dzi pɛpɛpɛ. Nɔ kadodo me kpli wo edziedzi.
- Ne wokpɔ ɖevi alo amesi ŋu PKU le nyuie la, ate ŋu anɔ agbe si me lãmesẽ le, si sɔ, kple dzidzɔ blibo.
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