Ðe nède dzesii be viwò suea ƒe tsitsi tsi megbe, alo tɔtrɔ aɖewo va le woƒe dzedzeme alo ƒunukpeƒewo nyitsɔ laa? Ɣeaɖewoɣi la, nusi le megbe na nu mawo ate ŋu anye nɔnɔme si mebɔ o si ŋu míese nu geɖe tsoe o. Egbea míele nu ƒom tso domenyiŋusẽfianu ƒe nɔnɔme aɖe si woyɔna be Hunter Syndrome ŋu. Mègavɔ̃ ne èse nya sia o, elabena nu vevitɔ kekeakee nye be nànɔ ŋudzɔ.

Nukae nye Hunter Syndrome? Mina míase egɔme bɔbɔe ŋutɔ!

Kpuie ko la, Hunter Syndrome nye domenyiŋusẽfianu ƒe dɔléle si mebɔ o. Esia nye nɔnɔme si me sukli ƒe molecule sesẽ aɖewo siwo le viwò ƒe ŋutilã me (siwo woyɔna be ``Glycosaminoglycans'' alo ``GAGs'') megbãna nyuie o eye womeɖua wo nyuie o. Abe alesi gbeɖuɖɔ siwo le míaƒe aƒewo me ƒoa ƒu ne míetsɔe ƒu gbe nyuie o ene la, sukli molecule siawo hã ƒoa ƒu ɖe ŋutilã ƒe lãmenugbagbeviwo me, vevietɔ le akpa siwo woyɔna be ``Lysosomes'' me. Le ɣeyiɣi aɖe megbe la, nuƒoƒoƒu sia tea nu gblẽm le ŋutinu kple lãkusi vovovowo ŋu le ŋutilãa me. Nusiwo gblẽ le ɖevia ŋu alea ate ŋu akpɔ ŋusẽ ɖe ɖevia ƒe ŋutilã kple susu ƒe tsitsi dzi.

Ðɔktawo ma Hunter Syndrome ɖe hatsotso vevi eve me:

1. Severe type: Esia nye ƒomevi si nu sẽ wu si yia ŋgɔ kabakaba wu. Le go sia me la, ekpɔa ŋusẽ ɖe ɖevia ƒe susuŋutetewo hã dzi. Zi geɖe la, ne ɖevia xɔ tso ƒe 6 va ɖo ƒe 8 la, edzea egɔme sesẽna nɛ be wòawɔ gbesiagbedɔ veviwo. Ame siwo ŋu Hunter Syndrome le la dometɔ siwo ade 60% ye ŋu dɔléle sia ƒomevi sesẽ.

2. Mild type: Dzesiwo dona blewu vie. Ðewohĩ makpɔ ŋusẽ ɖe nunya ƒe ŋutetewo dzi vevie o.

Hunter Syndrome nye dɔlélewo ƒe hatsotso gã aɖe si woyɔna be ``Mucopolysaccharidoses.`` Eyata wogayɔnɛ be ``Mucopolysaccharidosis type II`` alo ``MPS II``.

Aleke gbegbee Hunter Syndrome bɔe?

Le nyateƒe me la, esia nye nɔnɔme si mebɔ kura o. Azɔ hã, ŋutsuviwo koŋue wògblẽa nu le. Le akɔntabubu nu la, ŋutsuvi siwo ade ɖeka le 100,000 va ɖo 170,000 ɖesiaɖe me koe dɔléle sia le. Gake nyɔnuviwo ate ŋu anye domenyiŋusẽfianu ƒe tɔtrɔ si hea dɔléle sia vɛ la tsɔlawo. Esia fia be ne dzesi aɖeke mele wo ŋu o hã la, woate ŋu atsɔ domenyiŋusẽfianua ayi na wo viwo.

Nukae nye ɖevi si ŋu Hunter Syndrome le ƒe dzesiwo?

Zi geɖe la, dzesi siawo dzea egɔme dzena le ɖevi si xɔ tso ƒe 2 va ɖo ƒe 4. Dzesiawo ate ŋu ato vovo tso ame ɖeka gbɔ yi bubu gbɔ, eye woƒe sesẽme hã ate ŋu ato vovo. Mina míalé ŋku ɖe dzesi vevi siwo woate ŋu akpɔ ŋu:

• Ƒunukpeƒewo ƒe sesẽ, sesẽ be woabɔbɔ: Ate ŋu ase le eɖokui me abe ɖe ƒunukpeƒeawo "tsi" ene.
• Mo ƒe dzedzeme ƒe ƒoƒo: Teƒe siwo le abe ŋɔti, nuyi, kple aɖe ene ate ŋu aƒo eye wòadze abe ɖe wòle ƒuƒlu vie ene.
• Aɖuwo dodo tsi megbe alo dometsotso gãwo le aɖuawo dome.
• Ta lolo wu alesi wòle dzɔdzɔmee, akɔta keke wu, eye kɔ hã le kpuie.
• Tosese (tosese) si dzina ɖe edzi vivivi le ɣeyiɣi aɖe megbe.
• Tsitsi ƒe megbetsitsi: Kɔkɔme ate ŋu aɖiɖi, vevietɔ le ƒe 5 xɔxɔ vɔ megbe.
• Aɖuɖɔtoe kple aklã si lolo.
• Aŋɔ ɣi siwo le ŋutigbalẽa ŋu ƒe dzedzeme.

Anyo wu be màgatsi dzodzodzoe ne èkpɔ dzesi siawo dometɔ ɖeka alo eve o, gake ne viwòa yi edzi le dzesi siawo dometɔ siwo wu ɖeka kpɔm la, nunya anɔ eme be nàdi atikewɔlawo ƒe aɖaŋuɖoɖo.

Nukatae Hunter Syndrome dzɔna? Nuka gbɔe wòtso?

Susu vevitɔ si tae nye domenyiŋusẽfianu ƒe tɔtrɔ le `IDS` domenyiŋusẽfianu me. `IDS` domenyiŋusẽfianu sia kpɔa ŋusẽ ɖe enzyme si woyɔna be `(Iduronate 2-sulfatase)` alo `(I2S)` ƒe wɔwɔ dzi le míaƒe ŋutilã me. Dɔ si `(I2S)` enzyme sia wɔna enye be wòagbã sukli ƒe molecule sesẽ siwo woyɔna be `(Glycosaminoglycans)` alo `(GAGs)` siwo ŋu míeƒo nu tsoe va yi.

Eyata, le amesi ŋu Hunter syndrome `(MPS II)` le gome la, `(I2S)` enzyme sia mewɔa le ŋutilã me o, alo wowɔae le agbɔsɔsɔ sue aɖe ko me. Esi enzyme sia mele afima o ta la, sukli molecule mawo `(GAGs)` ƒoa ƒu ɖe akpa siwo woyɔna be `(Lysosomes)` me le lãmenugbagbeviwo me. `(Lysosomes)` nye teƒe siwo le lãmenugbagbeviwo me siwo gblẽa molecule siwo mehiã o eye wogbugbɔa wo zãna. Esi `(GAGs)` ƒoa ƒu alea ta la, `(MPS II)` dɔlélea le dɔlélewo ƒe ƒuƒoƒo si woyɔna be `(Lysosomal storage disorder)` me. Nuƒoƒoƒu siawo tae ŋutilãa ƒe ŋutinu kple lãkusi vovovowo gblẽna.

Amekawoe le afɔku gã me be woaxɔ esia?

Ne dɔléle sia le ame aɖe si le ƒomea me, si nye ame aɖe si do ƒome kplii le nugbagbewo me, la, afɔku si li be ame bubuwo naxɔe la lolo wu.

Abe alesi míegblɔe va yi ene la, ŋutsuviwoe nyi dɔléle sia ƒe dome wu. Esia le alea elabena dɔlélea do ƒome kple X domenyiŋusẽfianu. Ènyaa, nyɔnuviwo nyia X domenyiŋusẽfianu eve dome, ŋutsuviwo nyia X domenyiŋusẽfianu ɖeka kple Y domenyiŋusẽfianu ɖeka dome. Eyata ne nyɔnuvi nyi X domenyiŋusẽfianu si me domenyiŋusẽfianu sia si gblẽ le gɔ̃ hã la, X domenyiŋusẽfianu bubu si le lãmesẽ me ate ŋu ana enzyme si hiã. Eyata ɖewohĩ womaɖe dzesiwo afia o eye woanye amesi tsɔa dɔlélea. Gake ne ŋutsuvi aɖe nyi X domenyiŋusẽfianu si me domenyiŋusẽfianu si gblẽ ma le la ƒe dome la, axɔ dɔlélea elabena X domenyiŋusẽfianu bubu mele esi o.

Nukawoe nye kuxi siwo ate ŋu ado tso Hunter Syndrome me?

Le alesi dɔléle sia nu sẽe nu la, kuxi vovovowo ate ŋu ado mo ɖa. Ðɔktawo zãa atikewo kple ɣeaɖewoɣi amekoko gɔ̃ hã tsɔ ɖua kuxi siawo dzi. Na míakpɔ nusiwo nye kuxi siawo ɖa:

• Yagbɔgbɔ sesẽ: Yagbɔgbɔ sesẽ ate ŋu atso lãmekawo ƒe ƒoƒo kple yaƒoƒomɔ̃wo ƒe xexe ta.
• Dzidɔ (`(Dzidɔ)`).
• Nusiwo mesɔ o le ƒunukpeƒewo kple ƒuwo me.
• Ahɔhɔ̃a ƒe dɔwɔwɔ dzi ɖena kpɔtɔna vivivi.
• Carpal tunnel syndrome (`(Abɔta ƒe mɔ̃ ƒe dɔléle)`) .: Nɔnɔme si tsoa lãmekawo ƒe tete ɖe asibidɛ ƒe akpa si.
• Hernias (`(Hernias)`).
• Nɔnɔmewo abe asrãdɔ (`(Seizures)`).
• Nuwɔna ƒe kuxiwo.

Kuxi siawo medzɔna le mɔ ɖeka nu na amesiame o. Woate ŋu ato vovo le ɖevia ƒe nɔnɔme nu. Eyata ele vevie be nàƒo nu kple ɖɔkta edziedzi eye nànya ɖevia ƒe nɔnɔmea.

Aleke nàwɔ anya ne Hunter Syndrome le ŋuwò?

Viwòa ƒe ɖɔkta awɔ dodokpɔ geɖe atsɔ aɖo kpe nɔnɔme sia dzi.

• Aɖuɖɔ dodokpɔ: Esia léa ŋku ɖe sukli molecule siwo le aɖuɖɔ me (si woyɔna be GAGs) sɔ gbɔ le mɔ si mesɔ o nu ŋu.
• Ʋudodokpɔ: Esia ateŋu anya nenye be enzyme `(I2S)` ƒe dɔwɔwɔ le ʋu me le sue alo mele eme o. Esia hã nye dɔlélea ƒe dzesi vevi aɖe.
• Domenyiŋusẽfianu dodokpɔ: Esiae dea dzesi nenye be tɔtrɔ aɖe le IDS domenyiŋusẽfianu tɔxɛa me.

Nukawoe nye Hunter Syndrome ƒe atikewo?

Wodaa Hunter Syndrome le ɖevia ƒe dzesiwo nu. Esia bia be eŋutinunyala bibiwo ƒe ƒuƒoƒo aɖe nado alɔe. Ame siwo bi ɖe dɔ vovovowo me la wɔa dɔ aduadu tsɔ kpɔa ɖevia ƒe nɔnɔmea gbɔ. Taɖodzinu vevi siwo le atikewɔwɔ ŋue nye be woaɖe dɔlélea ƒe ŋgɔyiyi dzi akpɔtɔ, woade dzesi kuxi siwo ate ŋu ado mo ɖa le dɔlélea ta ahada wo kaba, eye woana ɖevia ƒe agbenɔnɔ nanyo ɖe edzi.

Atike nyuitɔ kekeake si li fifia be woaɖo taɖodzinu siawo gbɔe nye enzyme replacement therapy (`(Enzyme replacement therapy)`). Le esia me la, wotsɔa enzyme si amegbetɔ wɔ (`(Idursulfase (Elaprase®))` ɖɔlia `(I2S)` enzyme si bu la. Zi geɖe la, wodoa atike sia ɖe ʋuka me zi ɖeka le kwasiɖa me.

Tsɔ kpe ɖe eŋu la, wole numekuku wɔm tso domenyiŋusẽfianu ƒe dɔdamɔnu (alo domenyiŋusẽfianu ƒe tɔtrɔ) ŋu fifia. Esia ate ŋu ahe mɔkpɔkpɔ gã aɖe vɛ na dɔnɔ siwo ŋu Hunter Syndrome le le etsɔme. Ke hã, wogale emetsonuwo lalam kokoko.

Ðe mɔ aɖe li si dzi woato axe mɔ ɖe esia nua?

Esi wònye domenyiŋusẽfianu ƒe nɔnɔme sia nye ta la, nublanuitɔe la, womate ŋu axe mɔ ɖe enu o. Gake ele vevie ŋutɔ be ɖevi si ŋu Hunter Syndrome le dzilawo naƒo nu kple domenyiŋusẽfianu ŋuti aɖaŋuɖola hafi adzi vi bubu. Eŋutinunyala bibi sia ate ŋu akpe ɖe dzilawo ŋu woase afɔku si le dɔlélea tsɔtsɔ na ɖevi bubu me la gɔme.

Nukae nye etsɔme na amesi ŋu Hunter Syndrome le?

Womeke ɖe esia ƒe atike blibo ŋu haɖe o.Dɔlélea ƒe dɔléle sesẽwo ate ŋu ade ame ƒe agbe afɔku me. Ðevi mawo ƒe agbenɔƒe le mama dedie nu tso ƒe 10 va ɖo ƒe 20. Gake amesiwo ŋu dɔlélea mele bɔbɔe o ate ŋu anɔ agbe didi wu sã, vaseɖe tsitsime.

Le ame geɖe gome la, atikewɔwɔ abe atikewo, kamedede, kple amekoko ene ate ŋu akpe ɖe wo ŋu woakpɔ dɔlélea ƒe kuxiwo gbɔ eye woƒe agbenɔnɔ nanyo ɖe edzi.

Ðe vinyea agate ŋu awɔ dɔ nyuie akea?

Ðevi siwo ŋu Hunter Syndrome le ate ŋu asesẽ na gbesiagbedɔwo kple woƒe ʋuʋu ne woƒe dzesiwo le gbegblẽm ɖe edzi vivivi. Ðewohĩ ahiã be woatrɔ asi le dɔwɔna aɖewo ŋu. Viwòa ƒe ɖɔkta aƒo nu tso dɔwɔna kple atikewɔwɔ siwo ate ŋu akpe ɖe ŋuwò nànɔ te ɖe dzesiawo nu ŋu na wò.

Ɣekaɣie wòhiã be nàyi ɖɔkta gbɔ?

Ne viwòa dze Hunter Syndrome ƒe dzesiwo ɖeɖefia gɔme, alo ne ède dzesii be eƒe tsitsi tsi megbe la, te ɖe viwòa ƒe ɖɔkta ŋu enumake. Ne wodze atikewɔwɔ gɔme kaba ate ŋu akpe ɖe ame ŋu be woagagblẽ nu le ŋutinuwo kple lãkusiwo ŋu tegbee o.

Nukae wòle be nàbia ɖɔktaa?

Ne ènya nya be Hunter Syndrome le viwòa ŋu ko la, àte ŋu abia nya siawo abe esiawo ene ɖɔktaa:

• Aleke gbegbee Hunter Syndrome nu sẽe?
• Nukae anye vinye ƒe ɣeyiɣi kpui kple ɣeyiɣi didi ƒe nyagblɔɖi?
• Aleke dɔléle sia akpɔ ŋusẽ ɖe vinyea ƒe agbenɔnɔ dzii?
• Nukawoe nye atikewɔmɔnu siwo woate ŋu azã?

Bia nya siawo eye nàɖe ɖikeke ɖesiaɖe si anɔ mewò la ɖa. Elabena zi alesi nènya nu geɖee la, zi nenemae nàte ŋu akpe ɖe viwòa ŋu nyuie wu.

Vovototo kae le Hunter kple Hurler ƒe dɔlélea dome?

Hunter Syndrome kple Hurler Syndrome nye dɔléle eve le dɔlélewo ƒe ƒuƒoƒo si woyɔna be ``Lysosomal storage disorders'', ``Mucopolysaccharidoses'' me.

Hurler ƒe dɔléleae nye dɔléle si nu sẽ wu si nye Mucopolysaccharidosis type I (MPS I). Le MPS I me la, enzyme si nye alpha-L-iduronidase mele eme o. Hurler ƒe dɔlélea nu sẽ wu Hunter ƒe dɔlélea.

Gbedasi si woatsɔ ayi aƒemee

Mese alesi wòate ŋu asesẽ be nànya be dɔléle aɖe abe Hunter Syndrome ene le viwò ŋu gɔme. Ate ŋu agbã dzi na wò, vevietɔ ne èse nu tso viwò ƒe agbenɔƒe didi ŋu nya. Le ɣeyiɣi sesẽ sia me la, ɖo ŋku edzi be menye wò ɖeka koe o.

Nu vevitɔe nye be nàwɔ dɔ kple viwòa ƒe ɖɔktawo be nàsrɔ̃ nu geɖe tso dɔlélea kple edada ŋu alesi nàte ŋui. Azɔ hã, ƒo xlã wò kple ame siwo kpena ɖe ŋuwò, abe xɔ̃wòwo kple ƒowòmetɔwo ene. Woƒe kpekpeɖeŋu kple akɔfafa anye ŋusẽtsoƒe gã aɖe le ɣeyiɣi sia me. Ðo ŋku edzi be le kuxi ɖesiaɖe me la, mɔkpɔkpɔ li.

` Hunter Syndrome, Hunter Syndrome, MPS II, Domenyiŋusẽfianu ƒe Dɔlélewo, Enzymes, Ðeviwo ƒe Dɔlélewo, Dzesiwo, Atikewɔwɔ