Abe dzila ene la, ɖewohĩ ètsia dzi ɖe viwò suea ƒe lãmesẽ ŋu. Ɣeaɖewoɣi la, esɔ be nàvɔ̃ vie ne èse nu tso nɔnɔme siwo mebɔ o siwo ŋu míese nu tsoe gɔ̃ hã o ŋu. Enyo, egbea míele nu ƒom tso nɔnɔme aɖe si mebɔ o si ŋu ame geɖe mese nu tsoe o, gake ele vevie be woanya nu tso eŋu. Eƒe ŋkɔe nye Homocystinuria.
Kpuie ko la, nukae nye Homocystinuria (HCY)?
Bu eŋu kpɔ, míenya be protein le nuɖuɖu siwo míeɖuna, vevietɔ lã, tɔmelã, notsi, kple azi me . Protein gã sia nye nu suesuesue siwo woyɔna be amino acids . Abe xɔtunuwo ene. Le ame si le lãmesẽ me ƒe ŋutilã me la, amino acid siawo gbãna heɖua wo, eye wozua ŋusẽ si míaƒe ŋutilã hiã.
Gake le amesi ŋu Homocystinuria (HCY) le gome la, ŋutilã mate ŋu agbã amino acid si woyɔna be methionine nyuie ahaɖui o. Woyɔa esia be lãmenugbagbeviwo ƒe dɔwɔwɔ ƒe dɔléle. Ne edzɔ alea la, methionine sia kple atike bubu si woyɔna be homocysteine, si wowɔ tso eme la dzea ƒoƒo ƒu ɖe ŋutilãa me, vevietɔ le ʋu me. Afɔku le nuƒoƒoƒu sia me. Ne womewɔ atike na esia o la, ate ŋu ahe lãmesẽkuxi sesẽwo vɛ.
Nukatae esia le dzɔdzɔm? Nuka tae wòle alea ɖo?
Enzyme tɔxɛ aɖe si le míaƒe ŋutilã me kpena ɖe ame ŋu le amino acid sia si woyɔna be methionine la gbagbã me. Bu enzyme sia be enye lãgbalẽŋudɔwɔnu. Lãgbalẽŋudɔwɔnu siawo lã nu gã si woyɔna be methionine la ɖe akpa suesuesuewo me.
Le amesi ŋu homocystinuria le gome la, womewɔa enzyme sia (scissors) le ŋutilã me o, alo ne wowɔe la, mewɔa dɔ nyuie o.
Nu vevitɔe nye be esia nye dɔléle si ƒe dome míenyi . Ema fia be wotsɔnɛ yia dzidzimewo me. Domenyiŋusẽfianu eve li siwo fiaa mɔ wò be nàwɔ enzyme sia. Ele be woanyi ɖeka ƒe dome tso dawò gbɔ eye evelia natso fofowò gbɔ. Hafi homocystinuria nadze egɔme la, ele be domenyiŋusẽfianu siwo ƒe dome nènyi tso dawò kple fofowò gbɔ siaa me gblẽ .
Dzesi kawoe míate ŋu akpɔ?
Nukutɔe la, ne èlé ŋku ɖe vidzĩ si wodzi kple Homocystinuria ŋu la, vovototo aɖeke mele eme o. Wonye vidzĩ siwo sɔ keŋkeŋ. Dzesiawo dzea egɔme dzena le eƒe agbenɔƒe ƒe ʋɛ gbãtɔwo me. Menye ɖeviwo katãe kpɔa dzesi siawo le mɔ ɖeka nu o. Dzesi siawo dometɔ geɖe ate ŋu anɔ ɖevi aɖewo ŋu, evɔ bubuwo ya maɖe ɖeke afia o.
Lé ŋku ɖe nɔnɔme siwo le kplɔ̃ si le ete dzi ŋu.
| Nɔnɔmewo ƒe hatsotso | Dzesi siwo wokpɔna zi geɖe |
|---|---|
| Ŋutilã ƒe dzedzeme | Ŋutilã kple ɖa si le ɣie ŋutɔ, akɔta ƒe nɔnɔme si mebɔ o, eƒe ŋutilã kɔkɔ eye wòle tsɛ wu ɖevi bubuwo, kple asibidɛ didi siwo le tsɛ. |
| Tsitsi | Kpekpeme ƒe dzidziɖedzi kple tsitsi blewu. |
| Ŋku ƒe nukpɔkpɔ | Nukpɔkpɔ ƒe dɔmawɔmawɔ nyuie (nukpɔkpɔ kpuie), ŋkumeɖonu ƒe ʋuʋu, kple ŋkumaʋumaʋu gɔ̃ hã. |
| Dzesi sesẽ bubuwo | Ƒuwo ƒe gbɔdzɔgbɔdzɔ (ƒuwo ƒe gbagbã), ʋu ƒe sisi (si dzia ʋusɔgbɔdɔ kple dzidɔ ƒe afɔku ɖe edzi), kple alɔ̃madɔmadɔ. |
| Tsitsi kple nuwɔna | Heheɖemegbe le ʋuʋudedi, azɔlizɔzɔ, kple nuƒoƒo me. Nusɔsrɔ̃ ƒe kuxiwo kple susu ƒe kuxiwo. Nuwɔna kple seselelãme dzi ɖuɖu ƒe kuxiwo. |
Aleke ɖɔktawo wɔna kpɔa esia?
Le dukɔ geɖe me la, wozãa vidzĩ yeyewo ƒe dodokpɔ tsɔ dea dzesi nɔnɔmewo abe Homocystinuria ene kaba. Ʋudodokpɔ sia ɖenɛ fiana ne ɖevia le afɔku me be wòaxɔ dɔlélea.
Ne nusiwo do tso eme mesɔ o la, ɖɔktaa aɖo aɖaŋu be woagawɔ ʋu kple aɖuɖɔ dodokpɔ tɔxɛ bubuwo atsɔ aɖo kpe dɔlélea dzi. Le Sri Lanka la, zi geɖe la, woɖea gbe be woawɔ dodokpɔ siawo ne dzesiawo dze eye ne ɖikekewo do mo ɖa ko hafi.
Aleke wowɔa atike nɛ? Ðe wònye nusi wòle be woavɔ̃ na?
Ao, mègatsi dzi o. Nu vevitɔe nye be woadze atikewɔwɔ gɔme ne wonya kpɔ dɔlélea ko.Ðɔkta si kpɔa lãmenugbagbeviwo ƒe dɔwɔwɔ ŋuti ɖɔkta akpe ɖe ŋuwò le esia me. Atikewɔwɔ ƒe ɖoɖoa ate ŋu ato vovo tso ɖevi ɖesiaɖe gbɔ.
Atikewɔwɔmɔnu vevi eve li:
1. Vitamin B6 ƒe atikewɔwɔ
Homocystinuria ƒomevi aɖe li si mesẽ boo o. Woate ŋu aɖu edzi ne wona vitamin B6 ƒe kpeɖeŋutɔ agbɔsɔsɔme geɖe. Wò ɖɔkta ado viwòa kpɔ be atike sia sɔ na wo hã. Vitamin sia ate ŋu akpe ɖe ɖevi aɖewo ŋu woaxe mɔ ɖe nuwɔna kple susu ƒe kuxiwo nu, eye ate ŋu akpe ɖe wo ŋu hã be woaɖe ŋku, ƒu, kple ʋu ƒe sisi ƒe kuxiwo dzi akpɔtɔ.
2. Nuɖuɖu tɔxɛ (Low-Methionine Diet) .
Ne ɖevia mewɔ nu ɖe vitamin B6 zazã ŋu o la, afɔɖeɖe si kplɔe ɖoe nye be wòadze nuɖuɖu si me methionine mesɔ gbɔ ɖo o gɔme . Zi geɖe la, nuɖuɖu sia xɔa ame ƒe agbemeŋkekewo katã. Ele vevie be nàdi kpekpeɖeŋu tso nuɖuɖuŋutinunyala si bi ɖe amino acid ƒe dɔlélewo me gbɔ.
| Nusiwo ŋu wòle be woabu ne wole nuɖuɖu si me methionine mesɔ gbɔ ɖo o dzi wɔm | |
|---|---|
| Nuɖuɖu siwo me protein sɔ gbɔ ɖo siwo wòle be woaƒo asa na keŋkeŋ |
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| Nuɖuɖu bubuwo be woaɖo seɖoƒe na alo woadzudzɔ |
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| Nusiwo nàte ŋu aɖu kple ŋuɖɔɖɔɖo | Methionine ʋɛ aɖewo koe le atikutsetsewo kple amagbewo me, eyata woate ŋu aɖu wo le agbɔsɔsɔ si dzi wokpɔna me , abe alesi wò ɖɔkta kple nuɖuɖuŋutinunyala ɖo aɖaŋui ene. |
Tsɔ kpe ɖe eŋu la, ɖɔktaa kafui be woana notsi tɔxɛ aɖe ɖevia ɖe notsi teƒe. Nunyiame bubu siwo katã hiã na ɖevia ƒe tsitsi la le notsi sia me, eye woɖea methionine la ɖa.
Azɔ hã, ɖɔktaa ate ŋu aŋlɔ atike bubu geɖe siwo woatsɔ akpe ɖe eŋu.
- Betaine kple folic acid: Esiawo ɖea homocysteine si gblẽa nu le ame ŋu si ƒoa ƒu ɖe ʋua me ƒe agbɔsɔsɔ dzi kpɔtɔna.
- Vitamin B12 kple L-cysteine: Esiawo ate ŋu agbɔdzɔ le dɔlélea ta. Wodoa B12 abe atike ene eye wonaa L-cysteine abe kpeɖeŋutɔ ene.
Be nàka ɖe edzi be dɔdamɔnua le dɔ wɔm la, ahiã be woado viwòa ƒe ʋu kple aɖuɖɔ kpɔ edziedzi. Ne viwòa le tsitsim la, ahiã be nàwɔ tɔtrɔ suesuesuewo le atikewɔwɔ ƒe ɖoɖoa ŋu.
Eyata nukae wòle be míabu tso etsɔme ŋu?
Nya nyuiae nye be ne wodze atikewɔwɔ gɔme kaba eye woyi edzi nyuie la, ɖevia ate ŋu atsi ahatsi abe alesi wòle ene . Atikewɔwɔ nyuie ate ŋu aɖe ʋusɔgbɔdɔ, dzidɔ, kple ʋu ƒe sisi ƒe afɔkua hã dzi akpɔtɔ ŋutɔ.
Ɣeaɖewoɣi la, ŋku ƒe kuxiwo ate ŋu ado mo ɖa togbɔ be wowɔ atike nɛ hã. Gake woate ŋu akpɔ wo gbɔ to amekoko alo mɔnu bubuwo dzi. Nu vevitɔe nye be nànɔ kadodo me kple wò ɖɔkta edziedzi eye nàwɔ ɖe eƒe mɔfiamewo dzi pɛpɛpɛ.
Gbedasi si Wotsɔ Yi Aƒeme
- Homocystinuria nye domenyiŋusẽfianu ƒe dɔléle si mebɔ o si me ŋutilã mete ŋu ɖua methionine, si nye amino acid si le nuɖuɖu siwo míeɖuna me o.
- Esia nye nɔnɔme si domenyiŋusẽfianu siwo gblẽ si ƒe dome wonyi tso vidadaa kple fofoa siaa gbɔ gbɔ.
- Dzesiawo (ame ƒe kɔkɔme kpui, ŋku ƒe kuxi, tsitsi ƒe megbetsitsi) dzena le ɖevia dzidzi megbe kpuie.
- Woate ŋu akpɔ nɔnɔme sia gbɔ dzidzedzetɔe ne wozã nuɖuɖu tɔxɛ si me vitamin B6 alo methionine mesɔ gbɔ ɖo o.
- Ne èkpɔ dɔlélea kaba eye nèdae le eƒe agbemeŋkekewo katã me la, ate ŋu akpe ɖe viwòa ŋu wòanɔ agbe si le lãmesẽ me, si sɔ. Ne nane tsi dzi na wò le esia ŋu la, ƒo nu kple wò ɖɔkta gaglãa.
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