Asrã kple ŋutigbalẽ ƒe ʋuʋudedi enuenua? Mina míaƒo nu tso CAPS (Cryopyrin-Associated Periodic Syndromes) ŋu .

Bu eŋu kpɔ, le susu aɖeke manɔmee la, dzoxɔxɔ, ƒunukpeƒewo le veve sem, kple ʋuʋudedi suesuesuewo nɔa wò ŋutilã katã ŋu ɣesiaɣi. Esiawo nu yina le ŋkeke eve alo etɔ̃ megbe, emegbe wogadzena ake. Ne nane si le abe esia ene dzɔ ɖe dziwò alo viwòa dzi la, ate ŋu anye dɔléle si mebɔ o si ame geɖe mese nu tsoe gɔ̃ hã o la gbɔe wòtso. Egbea míele nu ƒom tso dɔléle mawo dometɔ ɖeka ŋu, si nye CAPS.

Nukae CAPS fia?

Kpuie ko la, Cryopyrin-Associated Periodic Syndromes, alo CAPS, nye domenyiŋusẽfianu me dɔléle siwo mebɔ kura o siwo gblẽa nu le míaƒe dɔlélenutsiŋutete ŋu ƒe ƒuƒoƒo. Zi geɖe la, míaƒe dɔlélenutsiŋutete kpɔa mía ta tso dɔlékuiwo kple dɔléle bubuwo me. Gake le CAPS nɔnɔme sia me la, le dɔlélenutsiŋutete ŋutɔ ƒe gbɔdzɔgbɔdzɔ ta la, edzea mía ŋutɔwo míaƒe ŋutilã dzi dzedze gɔme. Woyɔa esia be ‘autoinflammatory disease’ le atikewɔwɔ ŋuti dzɔdzɔmeŋutinunya me. Esia nana ŋutilãa xɔa abi madzemadzee, si hea dzesiwo abe ŋutigbalẽ ƒe ʋuʋu, dzoxɔxɔ, ƒunukpeƒeveame kple vuvu, kple ŋku biã ene vɛ.

CAPS ƒomevi vevi etɔ̃e li. Na míakpɔ nusiwo wonye ɖa.

Nukae hea CAPS vɛ?

Nusi koŋ gbɔ esia tsoe nye domenyiŋusẽfianu aɖe si le míaƒe ŋutilã me ƒe tɔtrɔ. Ne míagblɔe tututu la, domenyiŋusẽfianu si woyɔna be `NLRP3` ƒe tɔtrɔ gbɔe dɔléle sia tsona.

Bu `NLRP3` domenyiŋusẽfianu sia be enye nɔnɔmetata si fiaa mɔ míaƒe ŋutilã be wòawɔ protein si woyɔna be `cryopyrin`. Zi geɖe la, `cryopyrin` protein sia kpena ɖe míaƒe ŋutilã ŋu wòwɔa nusi woyɔna be `interleukin-1 (IL-1)`, si kpena ɖe míaƒe ŋutilã ŋu wòwɔa avu kple dɔlékuiwo.

Gake esi wònye be `NLRP3` domenyiŋusẽfianu si le ame si ŋu CAPS le me trɔ ta la, `cryopyrin` protein hã mewɔa dɔ nyuie o. Enaa dzesi ŋutilãa ɣesiaɣi be wòawɔ `IL-1` fũ akpa. `IL-1` ƒe wɔwɔ fũ siae nye nusi hea abi si mehiã o vɛ le ŋutilã me eye wòhea CAPS ƒe dzesiwo vɛ.

Dɔléle sia ate ŋu adzɔ ne ɖevia nyi domenyiŋusẽfianu si trɔ la ƒe dome tso dzila ɖeka ko gbɔ, eɖanye vidadaa alo fofoa o. Ɣeaɖewoɣi la, domenyiŋusẽfianu la te ŋu trɔna le eɖokui si le vidzidɔa me, dzilawo manɔmee.

Nukawoe nye dzesiawo?

CAPS ƒe dzesi vevitɔ kple esi bɔ wue nye ʋuʋudedi si dzena abe ʋuʋudedi suesuesue siwo me ʋuʋudedi mele o ene le ŋutilã bliboa me . Esia dzena zi geɖe le vidzidzi me. Abi siawo nɔa ame aɖewo ŋu atraɖii, gake wo nu sẽna ɖe edzi le dzobibi ƒe ɣeyiɣiwo me. Dzesi bubuwo toa vovo le CAPS ƒomevi si le ŋuwò nu.

Dzesi siwo woate ŋu akpɔ le FCAS me

Zi geɖe la, dzesi siawo nɔa anyi ŋkeke 1-2.

• Asrã
• Ŋkuwo vuvu
• Vuvɔ le wɔwɔm
• Ƒunukpeƒewo ƒe vevesese
• Xeɖeɖe
• Taɖuame

Dzesi siwo wokpɔna le MWS me

Dzesi siawo vana eye wodzona. Woate ŋu anɔ anyi ŋkeke 1-3 le ɣeyiɣi ɖeka me.

• Ŋutilã ƒe ʋuʋudedi
• Asrã kple vuvɔ
• Ŋkuwo ƒe biã
• Ƒunukpeƒewo ƒe vevesese
• Taɖuame sesẽ
• Tosese ƒe dɔmawɔmawɔ nyuie (ɖevi siwo ŋu MWS le ate ŋu abu woƒe tosese katã alo eƒe akpa aɖe hafi woava ɖo tsitsime) .
• Dɔgbo ƒe vevesese

Dzesi siwo wokpɔna le NOMID me

Esiawoe nye dzesi siwo nu sẽ wu.

• Taɖuame sesẽ
• Ŋku siwo le ʋuʋum
• Ƒunukpeƒewo ƒe vevesese kple vuvu
• Ʋuʋudedi
• Nukpɔkpɔ ƒe bu
• Tosese ƒe dɔmawɔmawɔ nyuie

Aleke woawɔ anya dɔlélea?

Ne èkpɔ wò ɖɔkta kple dzesi siawo la, abia wò gbã tso wò atikewɔwɔ ŋutinya kple wò ƒomea tɔ ŋu. Emegbe, woado wò kpɔ eye ne ehiã la, woate ŋu aɖo wò ɖe dodokpɔ aɖewo, abe:

• Ʋu kple aɖuɖɔ dodokpɔ: Dodokpɔ siawo dia ʋumenugbagbevi ɣiwo kple C-reactive protein, siwo nyea dɔdzẽ ƒe dzesiwo ƒe agbɔsɔsɔ si dzi ɖe edzi, kple protein si sɔ gbɔ ɖe aɖuɖɔ me.
• Ŋutilã ƒe lãmenugbagbeviwo ɖeɖe: Woɖea wò ŋutigbalẽ ƒe akpa sue aɖe ŋutɔ hedoa eŋu le nusuekpɔmɔ̃ me.
• Domenyiŋusẽfianu dodokpɔ: Dodokpɔ sia ate ŋu aɖo kpe edzi nenye be tɔtrɔ aɖe le wò NLRP3 domenyiŋusẽfianu me. Gake ɣeaɖewoɣi la, domenyiŋusẽfianu sia ate ŋu anɔ amesiwo ŋu CAPS le ŋu le mɔ si sɔ nu.
• Ŋku kple to dodokpɔ: Wowɔa dodokpɔ siawo be woakpɔe ɖa be wò nukpɔkpɔ alo nusese gblẽ nu le ŋuwò hã.
• Akɔta ƒe dodo: Woɖea aklã me tsi sue aɖe tso aklã me eye wodoa wo kpɔna be dɔdzẽ ƒe dzesiwo le eŋu hã.
• MRI scan: Esia ate ŋu aɖe nuwo ƒe wɔwɔme siwo le ahɔhɔ̃a kple towo me ƒe foto tsitotsito akpɔe ɖa be tɔtrɔ aɖewo va hã.

Nya siwo wòle be woabia ɖɔktaa

Anyo be nàŋlɔ nyabiase aɖewo siwo le asiwò ɖe pepa dzi ne èyi ɖɔkta gbɔ. To mɔ sia dzi la, màŋlɔ naneke be o. Àte ŋu abia nya siawo tɔgbe:

• CAPS ƒomevi ka ƒomevie le asinye (alo vinyea)?
• Dzesi kawoe nye dɔléle ƒomevi si ate ŋu ahe vɛ?
• Atikewɔwɔ kawoe nèkafu? Aleke woakpe ɖe ŋunyee?
• Ðe atikewɔwɔa ƒe nugbegblẽ aɖewo lia?
• Nukae mate ŋu awɔ le aƒeme be wòaɖe dzesiawo dzi akpɔtɔ?
• Dodokpɔ bubu kawoe wòle be mawɔ hafi? Ɣekaɣie wòle be mana woawɔ wo?
• Ðe wòhiã be makpɔ domenyiŋusẽfianu ŋuti aɖaŋuɖola aɖea?

Nukawoe nye atikewɔmɔnuawo?

Ele vevie ŋutɔ be woadze dɔléle sia dada gɔme kaba, elabena ne ŋkuwo, towo, ƒuwo, alo ahɔhɔ̃a nya gblẽ ko la, esesẽna ŋutɔ be woagbugbɔ wo aɖo nɔnɔme nyuitɔ me.

Atike vevitɔe nye atike siwo xea mɔ na ŋutilãa be wòagawɔ nusi woyɔna be IL-1 wòagbɔ eme o. Esiawo ɖua dɔdzẽ dzi.

• Anakinra (Kineret) .
• Kanakinumab (Ilaris) ƒe atike .
• Rilonacept (Aɖaŋudɔwɔla) .

Tsɔ kpe ɖe esiawo ŋu la, wò ɖɔkta ate ŋu aɖo aɖaŋu bubuwo hã na wò, abe:

• Aŋekotokuwo dodo hena ƒunukpeƒeveame kple ʋuʋudedi
• Lãmesẽfefewɔwɔ
• Atikewo abe NSAID, steroid, alo methotrexate ene be woatsɔ aɖe dzoxɔxɔ kple dɔdzẽ dzi akpɔtɔ
• Tomemɔ̃ na amesiwo mesea nu nyuie o
• Amekoko ne ƒunukpeƒewo gblẽ

Aleke nàte ŋu akpɔ ɖokuiwò dzii?

Tsɔ kpe ɖe atikewɔwɔ ŋu la, tɔtrɔ suesuesuewo wɔwɔ le wò agbenɔnɔ me ate ŋu akpe ɖe ŋuwò ŋutɔ le dzesiwo dzi ɖuɖu me.

• Ƒo asa na vuvɔ: Ne dɔléle aɖe abe FCAS ene le ŋuwò la, vuvɔ ate ŋu ana wò dzesiwo nagagblẽ ɖe edzi. Eyata dze agbagba nànɔ adzɔge tso nuwo abe AC ​​xɔwo me nɔnɔ kple teƒe siwo vuvɔ le wɔwɔm ene gbɔ.
• Ðe nuteɖeamedzi dzi kpɔtɔ: Nuwo abe ŋugbledede, gbɔgbɔ deto ƒe kamedede, kple yoga ate ŋu akpe ɖe ame ŋu ŋutɔ le esia me.
• Lãmesẽgbenɔnɔ:
• Mlɔ anyi nyuie.
• Ƒo nuɖuɖu si me nunyiame le.
• Ƒo asa na atamanono, aha sesẽ, kple nuɖuɖu siwo ŋu wotrɔ asi le.