Ðe amoniaka sɔ gbɔ ɖe wò ʋu mea? Mina míasrɔ̃ nu tso afɔku ƒe nɔnɔme si me hyperammonemia le ŋu!

Nu geɖewo le edzi yim le míaƒe ŋutilã me, alo? Ɣeaɖewoɣi la, ne nuwɔna siawo to vovo vie la, kuxi gãwo ate ŋu ado mo ɖa. Le kpɔɖeŋu me, hyperammonemia nye dɔléle sesẽ aɖe si dzɔna ne gbeɖuɖɔ si woyɔna be ammonia dzi ɖe edzi le míaƒe ʋu me. Ele vevie ŋutɔ be nànya esia elabena ate ŋu akpɔ ŋusẽ gã aɖe ɖe wò agbe dzi ne mèlé be nɛ nyuie o.

Nukae Nye Hyperammonemia?

Kpuie ko la, hyperammonemia nye ne amoniaka si le wò ʋu me sɔ gbɔ le mɔ si mesɔ o nu. Ammonia, alo NH3, nye gbeɖuɖɔ si dona le míaƒe dɔgbowo me ne míeɖu protein siwo míeɖuna. Zi geɖe la, amoniaka sia yia míaƒe aklã me eye wowɔa dɔ le afima. Míeyɔa nuwɔna sia be urea ƒe tsatsam . To tsatsam sia me la, amonia trɔna zua nusi megblẽa nu boo o si woyɔna be urea eye wòdona le aɖuɖɔ me. Gake ne nane gblẽ le urea ƒe tsatsam sia ŋu, alo ne aklã mete ŋu trɔ asi le amonia sia ŋu nyuie o la, amonia dzea ƒoƒo ɖe ʋua me gɔme. Amonia ƒe dzidziɖedzi sia le ʋu me nye aɖi ŋutɔ na míaƒe Central Nervous System (CNS) , si nye ahɔhɔ̃ kple aklã. Esia tae hyperammonemia nye dɔléle si hiã be woakpɔ egbɔ enumake eye ate ŋu ade ame ƒe agbe afɔku me gɔ̃ hã .

Amekae ate ŋu atu esia ɖo wu?

Nɔnɔme sia si woyɔna be hyperammonemia ate ŋu adze vidzĩ yeyewo, ɖevi suewo, kple ame tsitsiwo dzi.

• Le ame tsitsiwo dome la, amesiwo ŋu dɔléle sia le dometɔ siwo ade 90% nye amesiwo ŋu aklãdɔléle le .
• Vidzĩ siwo wodzi yeyee ate ŋu axɔ esia elabena wodzia wo kple urea ƒe tsatsam ƒe gbɔdzɔgbɔdzɔ (congenital urea cycle disorder) . Esiawo nye domenyiŋusẽfianu ƒe nɔnɔmewo.
• Azɔ hã, aklã ƒe dɔmawɔmawɔ nyuie kple lãmenugbagbeviwo ƒe dɔwɔwɔ ƒe dɔléle siwo ƒe dome wonyi ate ŋu ahe hyperammonemia vɛ na ɖevi suewo.

Esesẽ be míagblɔ alesi tututu esia bɔe, elabena ate ŋu atso nu vovovowo gbɔ. Gake numekulawo bu akɔnta be urea ƒe tsatsam ƒe dɔléle, si nye nusiwo gbɔ wòtsona dometɔ ɖeka, gblẽa nu le vidzĩ 250,000 ɖesiaɖe si wodzi le United States dometɔ ɖeka kple vidzĩ 440,000 ɖesiaɖe si wodzi le dukɔwo dome ŋu.

Nukawoe hea hyperammonemia vɛ?

Susu geɖewo ate ŋu anɔ esia ta. Susu siawo ate ŋu atrɔ ne ame tsi. Gake le goawo katã me la, nu vevi eve siwo gbɔ wòtsonae nye aklã me kuxiwo kple urea cycle disorders (UCDs) .

Le aklã ƒe kuxiwo ta

Míaƒe aklãe nye ŋutinu vevitɔ si trɔa amonia wòzua urea. Eyata ne aklãa gblẽ alo dɔléle le eŋu la, mate ŋu atrɔ asi le amonia ŋu nyuie o. Esiae nye esime amonia ƒoa ƒu ɖe ʋua me. Dɔléle siwo do ƒome kple aklã siwo ate ŋu ahe hyperammonemia vɛ dometɔ aɖewoe nye esi:

• Aklãdɔ : Ne aklãa gblẽ la , ŋutete si le esi be wòatrɔ asi le amonia ŋu la mebɔ o. Esia bɔ ɖe aklãdɔ sesẽwo me wu, gake le amesiwo ŋu aklãdɔléle si li ke le gɔ̃ hã gome la, amonia ƒe agbɔsɔsɔ ate ŋu adzi ɖe edzi kpata ɣeaɖewoɣi, le kpɔɖeŋu me, ne nane dzɔ le dɔgboa alo dɔgbo me ( ʋu si dona le dɔgboa me ) alo ne elektrikŋusẽ ƒe dadasɔ masɔmasɔ . Esia koŋ gbɔe amonia ƒe agbɔsɔsɔ si sɔ gbɔ ɖe ame tsitsiwo kple ɖeviwo me tsona.
• Hepatic encephalopathy : Esia dzɔna ne aklãa gblẽ eye mete ŋu trɔa asi le ammonia ŋu nyuie o. Amonia ƒoa ƒu ɖe ʋua me eye wòzɔa mɔ yia ahɔhɔ̃a me. Esia ate ŋu ana ame natɔtɔ, eƒe susu natɔtɔ , eye wòana ame natsi alɔ̃ me gɔ̃ hã. Ate ŋu awu ame ɣeaɖewoɣi.
• Aklã ƒe ʋuʋu: Aɖuɖɔtoe nye aklã ƒe lãkusi si le lãmesẽ me ɖɔliɖɔli kple abi ƒe lãkusi. Esia ate ŋu ana aklãa nagawɔ dɔ nyuie o eye ate ŋu ana ame naxɔ hyperammonemia. Cirrhosis nye aklãdɔléle si nɔa anyi didi.
• Aklã ƒe dɔmawɔmawɔ nyuie: Esiae nye esime aklãa dzudzɔ dɔwɔwɔ zi ɖeka. Dɔléle kple nɔnɔme vovovowo ate ŋu ahee vɛ. Le kpɔɖeŋu me, aklã ƒe abi si atike aɖewo, abe acetaminophen , ene hena vɛ le atikewo ta la nye aklã ƒe dɔmawɔmawɔ nyuie ƒe dɔlélewo katã ƒe 50% le United States. Ewɔa 64% le hyperammonemia ƒe dɔlélewo me le ɖeviwo me.
• Ʋu ƒe sisi yi wò aklã me dzi ɖe kpɔtɔ: Ne ʋu mekɔ yi wò aklã me nyuie o la, ŋutilãa mate ŋu aɖo amonia ɖe aklã be woawɔ dɔ tso eŋu o. Ɣemaɣi gɔ̃ hã la, amonia ƒoa ƒu ɖe ʋua me.
• Reye ƒe dɔlélea:Esia nye nɔnɔme si mebɔ kura o. Egblẽa nu le ʋu, ahɔhɔ̃, kple aklã ŋu. Zi geɖe la, enaa amoniaka ƒe agbɔsɔsɔ dzina ɖe ʋu me eye sukli ƒe agbɔsɔsɔ dzi ɖena kpɔtɔna le ʋu me. Edzea ɖevi suewo kple ƒewuivi siwo le hayahayam tso dɔlékuiwo abe koklozi alo dzamezã/dzamezã ene me wu eye wono aspirin ɖe dzesi mawo ta. Womenya nusi tututu gbɔ Reye ƒe dɔlélea tso o. Gake le afɔku sia ta la, mele be woana aspirin ɖevi suewo kple ƒewuiviwo o negbe ɖe ɖɔkta aɖe kafui koŋ hafi.

Le Urea ƒe Tsitretsitsi ƒe Dɔlélewo (UCDs) ta .

Abe alesi míeƒo nu tso eŋu va yi ene la, urea ƒe tsatsam nye mɔnu si dzi woto trɔa amonia si me aɖi le wòzua urea eye wòɖenɛ doa goe le aɖuɖɔ me. Afɔɖeɖe geɖe le tsatsam sia ŋu, eye wo dometɔ ɖesiaɖe hiã enzyme ƒomevi vovovo. Enzyme siawoe nye:

• N-acetyl-glutamate-nuwɔwɔ (NAGS) .
• Dɔlékuiwutike si woyɔna be carbamoyl phosphate synthetase (CPS) .
• Ornithine transcarbamylase (OTC) ƒe dɔlékuiwutike .
• Argininosuccinate si wotsɔ wɔa dɔe (AS) .
• Argininosuccinic acid lyase (ASL) si nye aɖi si woyɔna be .
• Arginase (ARG1) ƒe dɔlékuiwo .

Ne enzyme siawo dometɔ ɖeka mehiã o la, urea ƒe tsatsam mewɔa dɔ nyuie o. Amonia ƒoa ƒu ɖe ʋua me. Woyɔa enzyme ƒe anyimanɔmanɔ sia ƒomevi be urea cycle disorder (UCD). UCD ate ŋu ana hyperammonemia, si ate ŋu anye esi nu sẽ alo esi nɔa anyi didi. UCD-wo nye nɔnɔme siwo wodzina. Zi geɖe la, vidzĩ yeye siwo ƒe urea cycle enzyme mehiã kura o la xɔa hyperammonemia sesẽ le gaƒoƒo 24 va ɖo 72 me tso esime wodzi wo. Eɖanye enzyme la ƒe anyimanɔmanɔ le sue alo eƒe akpa aɖe o, ammonia ƒe agbɔsɔsɔ ate ŋu adzɔ le agbeme ɣeyiɣi ɖesiaɖe me, le dɔléle, nuteɖeamedzi, alo nuteɖeamedzi bubuwo me. UCD siawo nyea 23% le acute hyperammonemia ƒe dɔlélewo me le ɖevi sue siwo le dɔ lém vevie me.

Susu bubuwo tae

To vovo na esia la, susu bubu geɖewo ate ŋu anɔ anyi:

• Ayiku (ayiku) ƒe dɔmawɔmawɔ nyuie: Ne ayikuwo mete ŋu ɖe urea do goe nyuie o le ayiku ƒe dɔmawɔmawɔ nyuie ta la, ate ŋu ana amoniaka naƒo ƒu ɖe ʋua me.
• Ʋumedɔléle aɖewo: Ʋumenugbagbeviɣidɔ abe multiple myeloma kple acute leukemia ene hã ate ŋu ahe hyperammonemia vɛ.
• Dɔlékui aɖewo: nugbagbevi siwo wɔa urease abe Proteus mirabilis , Escherichia coli (E. coli) kple KlebsiellaDɔlékuiwo ƒe dɔlékuiwo abe streptococci ene, vevietɔ le ɖevi siwo dzi wodzia aɖuɖɔtoe me dɔmawɔmawɔ nyuie le kple ame tsitsi siwo ƒe aɖuɖɔtoe me dɔlékuiwo (UTIs) ta la, ate ŋu ahe hyperammonemia sesẽ vɛ.

Nukae nye Hyperammonemia ƒe dzesiwo?

Dzesiawo ate ŋu ato vovo ŋutɔ le alesi dɔlélea nu fa alo nu sẽe, kpakple ƒe si wòxɔ nu.

Dzesi fafɛ siwo bɔ ɖe ɖeviwo kple ame tsitsiwo me

• Aɖuɖɔtoe kple ʋuʋudedi
• Dɔgbo ƒe vevesese
• Dzikudodo enuenu
• Taɖuame (taɖuame) .
• Dadasɔ, nuwɔwɔ ɖekae, kple nuƒoƒo ƒe sesẽ (ataxia) .
• Nuwɔna ƒe tɔtrɔ

Dzesi fafɛ bubu siwo ɖeviwo koe dzena

• Do kpo nu le dzidzedzekpɔkpɔ me
• Lãmetsiwo ƒe gbɔdzɔgbɔdzɔ (hypotonia) .
• Ahɔhɔ̃mekawo ƒe tsitsi ƒe megbetsitsi

Dzesi sesẽwo le ɖeviwo kple ame tsitsiwo ŋu

Esiawo nye dzesi siwo me afɔku le ŋutɔ. Ne esiawo dometɔ aɖe le ŋuwò la, ele be nàdi atikewɔlawo ƒe aɖaŋuɖoɖo enumake.

• Tɔtɔ kple susu ƒe tɔtɔ
• Seselelãme ƒe tɔtrɔ
• Alɔ̃dɔdɔ fũ akpa
• Tɔtrɔ siwo vaa ame ƒe susu me
• Alɔ̃madɔmadɔ
• Yagbɔgbɔ kabakaba (Yagbɔgbɔ fũu akpa) .
• Coma

Nuxlɔ̃ame: Ne dzesi siawo le ŋuwò alo ame aɖe si te ɖe ŋuwò la, taflatse ƒo ka na 911 enumake, alo yi kɔdzi si te ɖe ŋuwò ƒe dɔwɔƒe si kpɔa nudzɔdzɔ kpatawo gbɔ. Amoniaka ƒe agbɔsɔsɔ si dzi ɖe edzi le ʋu me ate ŋu ade ame ƒe agbe afɔku me eye wòhiã be woakpɔ egbɔ enumake!

Dzesi siwo dzena le vidzĩ siwo wodzi yeyee ŋu

Ne hyperammonemia gblẽ nu le vidzĩ si wodzi yeyee ŋu la, zi geɖe la, dzesi siwo gbɔna dzena le gaƒoƒo 24 va ɖo 72 me tso esime wodzii:

• Dzikudodo enuenu, avifafa enuenu
• Ʋuʋudedi
• Kuviawɔwɔ
• Alɔ̃madɔmadɔ
• Gbeƒãɖeɖe ne wole gbɔgbɔm
• Yagbɔgbɔ kabakaba (Yagbɔgbɔ fũu akpa) .

Vevietɔ: Ne viwòa ɖe dzesi siawo fia la, yɔ ɖɔkta enumake ne ègakpɔtɔ le kɔdzi. Ne èle aƒeme la, ƒo ka na 911 enumake, alo kplɔ wo yi kɔdzi si te ɖe ŋuwò. Esia menye nusi woahe ɖe megbe o.

Aleke ɖɔktawo dea dzesi esiae?

Zi geɖe la, ɖɔktawo susuna be amoniaka sɔ gbɔ ɖe ʋua me to amoniaka ƒe agbɔsɔsɔ si le ʋua me dodokpɔ me.

• Le vidzĩ siwo xɔ vaseɖe ɣleti ɖeka gome la, ne amonia ƒe agbɔsɔsɔ le ʋua me wu micromol 80/lita (micromol 80/L).
• Le ɖevi tsitsiwo gome la, ne ewu 55 micromol/lita (55 micromol/L).
• Le ame tsitsiwo gome la, ne ewu micromol 30/lita (30 micromol/L).

Dzidzenu sia (micromol/L) nye mɔnu si dzi woato adzidze amoniaka ƒe agbɔsɔsɔ le ʋua me.

Dodokpɔ kawoe wowɔna tsɔ dea dzesi esia?

Dodokpɔ vevitɔe nye ʋudodokpɔ si me wodzidzea amonia ƒe agbɔsɔsɔ le wò ʋu me. Ne wò amonia sɔ gbɔ la, wò ɖɔkta ate ŋu awɔ dodokpɔ bubuwo atsɔ adi nusi gbɔ wòtso. Le kpɔɖeŋu me, ʋudodokpɔ be woatsɔ akpɔ wò aklã ƒe dɔwɔwɔ kple ayiku ƒe dɔwɔwɔ , kpakple aɖuɖɔtoe me dzodzro . Esi wònye be hyperammonemia ate ŋu agblẽ nu le lãmekawo ƒe dɔwɔwɔ ŋu eye wòade ame ƒe agbe afɔku me ta la, wò ɖɔkta ate ŋu awɔ nɔnɔmetatawo dodokpɔwo hã, abe CT (computed tomography) scan alo MRI (magnetic resonance imaging) scan ene, atsɔ akpɔe ɖa be kuxi siwo gblẽa nu le ahɔhɔ̃a ŋu hã.

Nukawoe nye atike siwo wozãna na hyperammonemia?

Atikewɔwɔ nɔ te ɖe nusi gbɔ wòtso dzi. Le kpɔɖeŋu me, le ame tsitsi gome la, aklãdɔ alo aklãdɔléle gbɔe wòtsona, alo le vidzĩ yeye gome la, urea cycle disorder (UCD) gbɔe wòtsona. Le acute hyperammonemia me la, taɖodzinu vevitɔe nye be woaɖe ammonia ƒe agbɔsɔsɔ si le ʋu me dzi akpɔtɔ eye woaɖu kuxi tɔxɛwo abe ahɔhɔ̃mekawo ƒe ʋuʋu kple nyaƒoɖeamenu si dzina ɖe edzi le ahɔhɔ̃a ŋu (intracranial hypertension) dzi.

• Ne amoniaka ƒe ʋusɔgbɔdɔ le vidzĩ si wodzi yeyee ŋu la, ɖɔktawo adzudzɔ protein nana wo (elabena amonia dona ne protein ɖuɖu) eye woana sukli, si nye suklitsi, wo boŋ be wòana nuɖuɖumeŋusẽ wo. Woazã mɔnu aɖe si woyɔna be hemodialysis hã . Esia bia be woakɔ vidzĩa ƒe ʋu ŋu eye woaɖe amoniaka ɖa to mɔ̃ si wotsɔna tsia ʋu me kple nusi wotsɔna tsia ʋu me (abe ayiku si wowɔ ene) dzi.
• Wonaa protein ɖeɖeɖa kple sukli hã amesiwo ŋu urea cycle enzyme deficiency (UCD) ƒe akpa aɖe le siwo xɔ hyperammonemia le nuteɖeamedzi ta, abe nuteɖeamedzi ƒe hyperammonemia ene, hã. Ne woƒe amonia ƒe agbɔsɔsɔ dzi meɖe kpɔtɔ le gaƒoƒo ʋɛ aɖewo me tso esime wowɔ atike na wo zi gbãtɔ o ko hafi wowɔa ʋumenugbagbeviɣidɔ.
• Aklã ƒe tagbɔdɔ dadae nye be woaɖe amoniaka ƒe wɔwɔme dzi akpɔtɔ le dɔgboa me. Atike gbãtɔ si wozãnae nye atike siwo woano siwo me lactulose kple lactitol le . Sukli siawo ɖea amoniaka ƒe wɔwɔme kple eƒe xɔxlɔ̃ le dɔgboa me dzi kpɔtɔna.

Ðɔktawo ate ŋu akafu nuɖuɖu kpeɖeŋutɔ abe esiawo ene be wòakpe ɖe amesiwo ŋu urea ƒe tsatsam ƒe dɔléle le ŋu be woaɖe woƒe dzesiwo ƒe dzɔdzɔ zi gbɔ zi geɖe dzi akpɔtɔ:

• L-carnitine si woyɔna be L-carnitine
• L-ornithine-L-aspartate ƒe lãgbalẽ
• Arginine si woyɔna be arginine

Ðe mɔ aɖewo li siwo dzi míato aƒo asa na nɔnɔme sia?

Esi wònye be nu geɖe li hea hyperammonemia vɛ ta la, menye nɔnɔmeawo katãe woate ŋu axe mɔ na o. Gake àte ŋu aɖe afɔ aɖewo atsɔ axe mɔ ɖe aklãdɔ ƒomevi aɖewo nu, vevietɔ esiwo do ƒome kple wò nuɖuɖu kple agbenɔnɔ. Ne aklãdɔ le afɔku me la, wò ɖɔkta ate ŋu aɖo aɖaŋu na wò be nàwɔ tɔtrɔ siawo le agbenɔnɔ me:

• Enyo be nàƒo asa na ahanono alo aɖo seɖoƒe nɛ.
• Ƒo asa na nuɖuɖu kple nunono siwo me ami siwo woyɔna be trans fats alo blitsi si me fructose sɔ gbɔ ɖo le.
• Be nàƒo asa na aklã ƒe gbegblẽ la, kpɔ nyuie ŋutɔ ne èle atike siwo woŋlɔ na wò kple esiwo womeƒlena o zãm.
• Kamedede edziedzi .
• Lã dzĩ ɖuɖu dzi ɖeɖe kpɔtɔ.

Be nàxe mɔ ɖe Reye ƒe dɔléle, si nye dɔléle si hea hyperammonemia vɛ na ɖeviwo kple sɔhɛ tsitsiwo nu la, mègana aspirin ɖeviwo ɖe dɔlékui ƒe dɔléle (abe dzamezã alo koklozi ene) ƒe dzesiwo ta o negbe ɖe ɖɔkta kafui tẽ hafi. Urea cycle defects (UCDs) mebɔ o, gake domenyiŋusẽfianu ƒe dodokpɔwo li be woakpɔe ɖa be domenyiŋusẽfianu siwo hea wo vɛ la le asiwò hã. Ðo dze kple wò ɖɔkta tso esia ŋu.

Nukae nye nɔnɔme sia ƒe ŋgɔdonya?

Mɔkpɔkpɔ na hyperammonemia, si nye ŋutete si le ame si be wòahaya la nɔ te ɖe nu geɖe dzi:

• Aleke gbegbe wò amoniaka ƒe agbɔsɔsɔme sɔ gbɔe.
• Ɣeyiɣi didi kae wodo amoniaka ƒe agbɔsɔsɔ ɖe dzi.
• Nukae hea hyperammonemia vɛ?

Le goawo katã me la, mɔkpɔkpɔ si li be woakpɔ hyperammonemia la medea ame dzi o. Ne womewɔ atike nɛ o alo wodae nɛ megbe la, ahɔhɔ̃a ƒe vuvu si womate ŋu atrɔ o do ƒome kple ame gbogbo aɖewo siwo kuna. Nublanuitɔe la, aleke kee woɖatsɔ hyperammonemia sesẽ da vidzĩ yeyee kaba alo le adãtɔe o, kakaɖedzi aɖeke meli be emetsonua anyo o. Le United States la, ƒe 11 ƒe agbetsitsi ƒe agbɔsɔsɔme na amesiwo ŋu urea cycle defects (UCDs) siwo xɔ hyperammonemia le woƒe agbemeŋkekewo ƒe gɔmedzedze la ade 35%. Le amesiwo xɔ hyperammonemia le woƒe tsitsime gome la, agbetsitsi ƒe agbɔsɔsɔme anɔ abe 87% ene. Woka nya ta be ƒe ɖeka kple ƒe etɔ̃ ƒe agbetsitsi ƒe agbɔsɔsɔme si dɔnɔ siwo ŋu aklã me tagbɔdɔ sesẽ le le hyperammonemia ta la nye 42% kple 23%.

Kuxi kawoe ate ŋu ado tso hyperammonemia me?

Ne womekpɔ dɔléle sia gbɔ kaba o la, amoniaka ƒe agbɔsɔsɔ si dzi ɖe edzi ate ŋu agblẽ nu le lãmekawo ƒe dɔwɔwɔ ŋu eye wòahe kuxi siwo ate ŋu ade ame ƒe agbe afɔku me vɛ. Kuxi mawo dometɔ aɖewoe nye:

• Ahɔhɔ̃ me ʋuʋu
• Nyaƒoɖeamenu si dzina ɖe edzi ƒo xlã ahɔhɔ̃a (Intracranial hypertension) .
• Brain herniation – Esia tsoa ahɔhɔ̃a ƒe vuvu kple nyaƒoɖeamenu si dzina ɖe edzi ƒo xlã ahɔhɔ̃a gbɔ zi geɖe.
• Coma
• Ku

Ne hyperammonemia si nu mesẽ o si nɔa anyi didi le ame aɖe ŋu ɣeyiɣi didi la, ate ŋu axɔ susu ƒe nuwɔametɔnyenye kple nuwɔna kple tagbɔdɔléle ƒe kuxi siwo nɔa anyi ɖaa.

Ɣekaɣie wòle be míayi ɖɔkta gbɔ?

Ne hyperammonemia ƒe dzesiwo le ŋuwò alo ame aɖe si ŋu nètsɔ ɖe le la, ƒo ka na 911 enumake alo yi kɔdzi si te ɖe ŋuwò. Esia menye nusi woahe ɖe megbe o. Ne aklãdɔ alo urea cycle enzyme ƒe akpa aɖe ƒe anyimanɔmanɔ le ŋuwò la, ele vevie ŋutɔ be nàyi ɖɔkta gbɔ edziedzi be nàkpɔ wò nɔnɔmea gbɔ ahalé ŋku ɖe wò amonia ƒe agbɔsɔsɔ ŋu.

Hyperammonemia nye dɔléle sesẽ aɖe si ate ŋu atso nu geɖe gbɔ. Ne dzesi siawo le wò, viwò, alo viwòa ŋu la, di kpekpeɖeŋu enumake. Ne aklãdɔ le ŋuwò, alo ne urea cycle disorders (UCD) le wò ƒomea me la, ƒo nu kple wò ɖɔkta tso afɔku si le eme be nàxɔ hyperammonemia ŋu. Wole afima be yewoakpe ɖe ŋuwò.

Mlɔeba la, ɖo ŋku esia dzi.

Enyo, eyata nu vevi siwo dzi wòle be nàɖo ŋkui tso nusiwo ŋu míeƒo nu tsoe me la dometɔ aɖewoe nye esi:

• Hyperammonemia nye amoniaka si sɔ gbɔ ɖe ʋua me si me afɔku le. Esia gblẽa nu le míaƒe ahɔhɔ̃ kple lãmekawo ŋu ŋutɔ.
• Susu geɖewo ate ŋu anɔ esia ta. Aklã me kuxiwo kple urea cycle disorders (UCDs) ye nye nusiwo koŋ gbɔ wòtsona.
• Dzesiawo toa vovo le alesi nɔnɔmea nu sẽe kple ƒe si wòxɔ nu. Woate ŋu atso dzesi siwo mesẽ o abe ʋuʋudedi, ʋuʋudedi, kple taɖuame ene dzi va ɖo dzesi siwo nu sẽ wu abe tɔtɔ, alɔ̃madɔmadɔ, kple alɔ̃madɔmadɔ ene dzi. Ele be vidzĩ yeyewo hã natsi dzi vevie ɖe woƒe dzesiwo ŋu.
• Ne dzesi sesẽwo le ŋuwò la, ele be nàdi atikewɔlawo ƒe aɖaŋuɖoɖo kaba. Esia ate ŋu anye agbe alo ku ƒe nɔnɔme.
• Atikewɔwɔ nɔ te ɖe nusi gbɔ wòtso dzi. Atikewɔwɔ kaba ate ŋu aɖe amoniaka ƒe agbɔsɔsɔ dzi akpɔtɔ eye wòaɖu kuxiwo dzi.
• Togbɔ be womate ŋu axe mɔ ɖe nusiwo gbɔ wòtsona dometɔ aɖewo nu o hã la, woate ŋu aɖe afɔku aɖewo dzi akpɔtɔ ne èlé agbenɔnɔ nyui me ɖe asi eye nèlé be na wò aklã .
• Eɖanye aklãdɔ le ŋuwò alo UCD le wò ƒomea me o, ele vevie be nàdo wò kpɔ edziedzi.

Mègaŋlɔ be o, wòe nya wò lãmesẽ nyuie wu. Ne èse le ɖokuiwò me be nane to vovo alo esi mebɔ o la, mègavɔ̃ be yeaƒo nu kple ɖɔkta o. Hyperammonemia, ammonia, aklã, urea ƒe tsatsam, ahɔhɔ̃, dzesiwo, atikewɔwɔ