Ðe nèle teƒe wɔnukuwo kpɔm le wò ŋutigbalẽ ŋua? Ðe wòate ŋu atso (Incontinentia Pigmenti) gbɔa?

Èse nɔnɔme sia si woyɔna be `(Incontinentia Pigmenti)` alo `(IP)` kpɔa? Ðewohĩ ne èse ŋkɔ sia la, aɖi nya sesẽ vie eye wòawɔ nuku. Gake esia nye nyati vevi aɖe si ŋu wòle be míaƒo nu tsoe, elabena enye domenyiŋusẽfianu ƒe nɔnɔme si mebɔ o si ate ŋu adze ɖevi aɖewo dzi, vevietɔ nyɔnuviwo. Eyata egbea míaƒo nu tso nu geɖewo ŋu le nya bɔbɔewo me, abe nusi `(Incontinentia Pigmenti)` nye, nusiwo nye eƒe dzesiwo, kple alesi wowɔa atike nɛ ene.

Nukae nye `(Incontinentia Pigmenti)`? Mina míase egɔme bɔbɔe!

Kpuie ko la, Incontinentia Pigmenti nye domenyiŋusẽfianu ƒe dɔléle si do ƒome kple X si xɔ aƒe ɖe eme. Àte ŋu anɔ eŋu bum be, "Oo, ɖɔkta, nyemese esia gɔme o." Enyo, mina míawɔe wòanɔ bɔbɔe wu. Enye dɔléle si toa domenyiŋusẽfianuwo dzi, si fia be etso dzila gbɔ yia vi gbɔ. Enɔa dɔlélewo ƒe ƒuƒoƒo aɖe si woyɔna be ahɔhɔ̃mekawo ƒe dɔlélewo me. Esia fia be dɔléle siawo ƒomevi ate ŋu akpɔ ŋusẽ ɖe míaƒe lãmekawo ƒe dɔwɔwɔ dzi (si nye ahɔhɔ̃ kple aklã), ŋutigbalẽ, ŋkuwo, aɖuwo, kple ƒunukpeƒewo .

Le `(IP)` gome la, tɔtrɔ aɖewo (nutations) le míaƒe domenyiŋusẽfianuwo me nana be teƒeteƒewo kple teƒeteƒewo dzena vivivi le ŋutigbalẽa ŋu. Esiae nye dɔléle sia ƒe dzesi vevitɔ kple esi bɔ wu. Woate ŋu akpɔ teƒe siawo le ŋutigbalẽa ŋu le vidzidzi me, eye ɣeaɖewoɣi la, woate ŋu adze le vidzidzi megbe kwasiɖa ʋɛ aɖewo gɔ̃ hã.

Teƒe ƒomevi enee le ŋutigbalẽa ŋu!

Ŋutilã ƒe teƒe siawo ate ŋu adze ɖekaɖeka, le akpa ene me. Ɣeaɖewoɣi la, afɔɖeɖe siawo ate ŋu ava ɖe ɖoɖo nu, alo akpa ɖeka ate ŋu adze ɖe wo nɔewo yome enumake. Bu eŋu abe ɣeyiɣiawo ƒe tɔtrɔ ene, gake ɣeaɖewoɣi la, ɣeyiɣi ɖeka wu enu hafi esi kplɔe ɖo dzea egɔme.

1. Vesicular stage: Esia nye esime ʋuʋudedi suesuesue siwo biã, siwo me tsi yɔ fũu la dzena le ŋutigbalẽa ŋu. Woɖi ʋuʋudedi siwo dona le koklozi ƒe dɔléle me. Woate ŋu adze abe fliwo ene le ŋutilã bliboa me, vevietɔ le abɔwo kple afɔwo dzi.

2. Verrucal stage: Le ɣeyiɣi aɖe megbe la , teƒe siwo le abe ʋuʋudedi ene, siwo kɔ, siwo kɔ ɖe dzi la dzea dzedze gɔme le afisi ʋuʋudediawo nɔ. Esiawo te ŋu nɔa anyi ɣleti geɖe ɣeaɖewoɣi.

3. Hyperpigmentation stage: Eyome, teƒe siwo le ɣie, siwo ƒe amadede nye blɔ-blɔ la dzena le ŋutigbalẽa ŋu. Esiawo dzena le ŋutilã kple abɔ kple afɔwo dzi madzemadzee, eye woɖia kpe xɔasi siwo wotsɔ kpe xɔasiwo wɔe alo fli siwo le ƒutsotsoewo me . Teƒe siawo nye susu siwo ta woto ŋkɔ `(Incontinentia Pigmenti)` vɛ la dometɔ ɖeka. "Pigmenti" gɔmee nye amadede. Akpa sia ate ŋu anɔ anyi ƒe geɖe.

4. Hypopigmentation stage: Mlɔeba la, teƒe siwo do viviti la ava xɔ ɖe teƒe siwo le ɣie, siwo le abe abi ene ɣeaɖewoɣi . Zi geɖe la, nusiawo te ŋu nɔa anyi le woƒe agbemeŋkekewo katã me.

Nu vevitɔe nye be menye amesiamee akpɔ ɣeyiɣi ene siawo katã le ɖoɖo sia nu o. Ðewohĩ ɣeyiɣi ɖeka koe ame aɖewo akpɔ nyuie, evɔ ame bubuwo ya ate ŋu akpɔ wo dometɔ geɖe zi ɖeka.

Zi geɖe la, domenyiŋusẽfianu si woyɔna be NEMO (NF-kappaB essential modulator) ƒe tɔtrɔ gbɔe IP tsona. NEMO domenyiŋusẽfianu sia nye domenyiŋusẽfianu vevi aɖe si kpena ɖe lãmenugbagbeviwo ƒe dɔwɔwɔ ŋu le míaƒe ŋutilã me eye wòkpena ɖe lãmenugbagbeviwo ƒe ku si ŋu wowɔ ɖoɖo ɖo hã ŋu. Nublanuitɔe la, womekpɔ atike aɖeke koŋ na IP haɖe o.

Nukae nye kuxi siwo ate ŋu ado tso `(Incontinentia Pigmenti)` me?

Menye ŋutigbalẽ ƒe dɔléle aɖe koe IP nye o. Ɣeaɖewoɣi la, ate ŋu ahe lãmesẽkuxi bubuwo hã vɛ. Ele vevie ŋutɔ be míanya nu tso ema hã ŋu.

Ahɔhɔ̃mekawo ƒe kuxiwo

• Ahɔhɔ̃ ƒe dɔmawɔmawɔ nyuie.
• Do suesuesue siwo le nu ɣia me le ahɔhɔ̃a titina.
• Lãmetsiŋusẽ ƒe lãmenugbagbevi siwo le ahɔhɔ̃mekawo me ƒe tsɔtsrɔ̃.

Le susu siawo ta la, ɖevi siwo ŋu `(IP)` le la dometɔ siwo ade 20% ate ŋu axɔ nɔnɔme siawo:

• Mɔ̃ ƒe dɔwɔwɔ ƒe ŋutete siwo tsi megbe (e.g., ʋuʋudedi si tsi megbe, azɔlizɔzɔ si tsi megbe).
• Susu ƒe kuxiwo (abe nusɔsrɔ̃ ƒe kuxiwo ene).
• Lãmetsiwo ƒe gbɔdzɔgbɔdzɔ.
• Fit (alɔ̃madɔmadɔ) ƒe amesinɔnɔ.

Nukpɔkpɔ ƒe kuxiwo

Ðevi aɖewo siwo ŋu `(IP)` le hã ate ŋu akpɔ kuxiwo. Le kpɔɖeŋu me:

• Ŋku siwo wotso (strabismus).
• Ŋku ƒe ʋuʋu.
• Le nɔnɔme sesẽ aɖewo me la, nukpɔkpɔ ate ŋu abu keŋkeŋ .

Aɖuŋutinunya ƒe kuxiwo

Woateŋu akpɔ aɖu ƒe kuxiwo hã kple `(IP)`.

• Aɖu aɖewo bu.
• Aɖuawo nye ``peg-shaped``.
• Aɖuɖɔtoe si tsi megbe.

Esia tae wòle vevie ŋutɔ be nàdi aɖaŋuɖoɖo tso aɖudɔdala gbɔ.

Nukae nye `(Incontinentia Pigmenti Achromians)`?

Nɔnɔme bubu si ɖi esia vie enye `(incontinentia pigmenti achromians)`. Le esia me la, ŋutigbalẽa ƒe amadede nɔa keklẽm, si ƒe amadede nye blɔ (si ƒe amadede mesɔ gbɔ o) kple flitete . To vovo na teƒe siwo do viviti le `(IP)` me la, teƒe siwo ƒe amadede le keklẽm koe le nɔnɔme sia me. Ke hã, abe `(IP)` ene la, nɔnɔme sia `(incontinentia pigmenti achromians)` ate ŋu ado ƒome kple kuxi siwo le lãmekawo me hã.

Aleke wowɔa atike na `(Incontinentia Pigmenti)`?

Abe alesi míegblɔe va yi ene la, atike tɔxɛ aɖeke meli na `(IP)` haɖe o. Gake atikewɔmɔnu vovovowo li woatsɔ aɖu dzesiwo kple kuxi siwo dɔléle sia hena vɛ dzi. Ame ɖesiaɖe ƒe dzesiwoe nɔa te ɖe atikewɔwɔa dzi.

• Ŋutilã ƒe teƒeteƒewo: Zi geɖe la, ŋutigbalẽ ƒe ʋuʋudedi siawo, vevietɔ ʋuʋudedi kple ʋuʋudedi siwo dzena le woƒe gɔmedzedze la, nu yina vivivi atike aɖeke mawɔmawɔe, le ƒewuiwo me alo le tsitsime o.Teƒe doviviti mawo (hyperpigmentation) kple teƒe siwo me kɔ (hypopigmentation) ate ŋu anɔ anyi ɣeyiɣi didi, ɣeaɖewoɣi le woƒe agbemeŋkekewo katã me. Gake zi geɖe la, womehea vevesese aɖeke vɛ o.
• Lãmetsiŋusẽ ƒe dzesiwo: Ne lãmekawo ƒe ʋuʋu, lãmekawo ƒe gbɔdzɔgbɔdzɔ, alo lãmekawo ƒe ʋuʋu si mesɔ o le ŋuwò la, ahɔhɔ̃ŋutinunyala ate ŋu aŋlɔ atikewo kple atikewɔmɔ̃ aɖewo na wò .
• Nukpɔkpɔ ƒe kuxiwo: Ne mèkpɔa nu nyuie o la, wò ɖɔkta ate ŋu aɖo aɖaŋu na wò be nàzã ŋkumeɖobolo, atike, alo le nɔnɔme sesẽwo me la, amekoko .
• Aɖukuxiwo: Aɖudɔŋutinunyalawo ate ŋu ada aɖukuxiwo. Woate ŋu awɔ nuwo abe aɖuwɔwɔe kple aɖuwɔwɔe ene.

Aleke agbea anɔ kple nɔnɔme si nye `(Incontinentia Pigmenti)`? (Nyagblɔɖi) .

Esia nye kuxi na ame geɖe. Menye amesiame si ŋu IP le lae xɔa kuxi sesẽwo o. Ame geɖe, vevietɔ amesiwo mekpɔa kuxi sesẽwo le vidzĩme alo ɖevimenɔɣi o, ate ŋu anɔ agbe si sɔ eye wòanɔ lãmesẽ me. Zi geɖe la, amadede ƒe teƒe siwo dzena le ŋutigbalẽa ŋu la nu yina le ɣeyiɣi aɖe megbe, eye ɣeaɖewoɣi la, wobuna keŋkeŋ.

Ke hã, ahɔhɔ̃mekawo kple ŋutilã me kuxiwo ate ŋu ayi edzi anɔ fu ɖem na ame aɖewo. Eyata ne IP le wò alo viwò ŋu la, anyo wu be nàƒo nu kple wò ɖɔkta alo domenyiŋusẽfianu ŋuti aɖaŋuɖola aɖe be yeanya lãmesẽnyawo gbɔ kpɔkpɔ ƒe ɖoɖo nyuitɔ kekeake na wò.

Fifia numekulawo le domenyiŋusẽfianu ƒe kadodo ŋuti numekuku wɔm be yewoanya afisi domenyiŋusẽfianu siwo do ƒome kple ahɔhɔ̃mekawo ƒe dɔlélewo abe IP ene le. Wole nu srɔ̃m tso alesi domenyiŋusẽfianu ƒe tɔtrɔ siawo kpɔa ŋusẽ ɖe ahɔhɔ̃ kple lãmekawo ƒe tsitsi kple dɔwɔwɔ dzii hã ŋu. Numekuku siawo tɔgbe akpe ɖe mía ŋu míase nu geɖe tso domenyiŋusẽfianu ƒe dɔlélewo ŋu eye ate ŋu aʋu mɔ na atikewɔwɔ le etsɔme.

Nu vevitɔ kekeake siwo dzi wòle be woaɖo ŋkui (Take-Home Message) .

• Incontinentia Pigmenti nye domenyiŋusẽfianu ƒe dɔléle si mebɔ o, si gblẽa nu le ŋutigbalẽ ŋu koŋ, gake ate ŋu agblẽ nu le lãmekawo, ŋkuwo, kple aɖuwo hã ŋu.
• Ŋutilã ƒe teƒeteƒewo ate ŋu adze le akpa ene me. Zi geɖe la, nusiawo nu yina le ɣeyiɣi aɖe megbe.
• Menye amesiamee xɔa kuxi sesẽwo o. Ame akpa gãtɔ ate ŋu anɔ agbe si sɔ.
• Dzesiawoe nɔa te ɖe atikewɔwɔa dzi. Àte ŋu adi kpekpeɖeŋu tso ahɔhɔ̃ŋutinunya, ŋkudɔ, kple aɖudɔdada ŋuti nunyala bibiwo gbɔ.
• Ne wò ŋutɔ alo viwòa tsi dzi ɖe nɔnɔme sia ŋu la, di atikewɔlawo ƒe aɖaŋuɖoɖo enumake. Nu vevitɔe nye be màgatsi dzodzodzoe o, nàxɔ nyatakaka siwo sɔ, eye nàdi atike si hiã.

Mele mɔ kpɔm be nyatakaka sia akpe ɖe ŋuwò. Ne nya aɖe le asiwò la, mègakpe ŋu abia ɖɔkta o. Mí katã míele afisia be míakpe ɖe ŋuwò.

` Incontinentia Pigmenti, IP, ŋutigbalẽdɔlélewo, domenyiŋusẽfianu ƒe dɔlélewo, NEMO domenyiŋusẽfianu, ŋutigbalẽ ƒe teƒeteƒewo, ahɔhɔ̃mekawo ƒe dɔlélewo, ɖeviwo ƒe dɔlélewo