Ðe protein siwo le ŋutilãa me tɔtɔa? Míele nu ƒom tso amyloidosis sia ŋu!

Ðe ɖeɖi te ŋuwò, ɖeɖi te ŋuwò, eye ɖewohĩ èle lolo dzi ɖem le mɔ si womate ŋu asusui nu hã le ŋkeke siawo mea? Ɣeaɖewoɣi la, tɔtrɔ suesuesue siwo vaa míaƒe ŋutilã me ate ŋu ahe nu gãwo vɛ. Egbea míele nu ƒom tso dɔléle aɖe si dzɔna ne protein ƒomevi aɖe si le míaƒe ŋutilã me ƒe tagbɔ gblẽ vie, eƒe nɔnɔme mesɔ o, eye wòge ɖe míaƒe ŋutinu veviwo me ŋu. Esiae nye nusi míeyɔna be amyloidosis . Mègatsi dzi o, míaƒo nu tso esia ŋu bɔbɔe eye míase egɔme.

Nuka tututue nye amyloidosis?

Kpuie ko la, amyloidosis nye nɔnɔme si me amyloid protein (siwo nye protein siwo mesɔ o ŋutɔŋutɔ) siwo le míaƒe ŋutilã me la ƒoa ƒu nyuie, ƒoa ƒu ɖekae, eye wowɔa ƒuƒoƒo suesuesuewo, abe ka siwo woyɔna be fibrils ene, siwo nɔa ŋutinu veviwo abe míaƒe dzi, ayiku, kple aklã ene me. Ne wowɔe, eye ne womewɔ atike na wo o la, protein fibril siawo siwo mesɔ o ate ŋu agblẽ nu le míaƒe ŋutinuwo ŋu vevie.

Ɣeaɖewoɣi la, protein siawo siwo mesɔ o ate ŋu aƒo ƒu ɖe ŋutinu aɖe ƒe akpa sue aɖe ko. Woyɔa esia be localized amyloidosis . Zi geɖe la, esia ate ŋu agblẽ nu le míaƒe ŋutigbalẽ, tsinyegoe ƒe akpa sue aɖe, alo gbɔgbɔtsixe ŋu. Gake zi geɖe la, ka siawo nɔa ŋutinu blibo aɖe me alo nɔa ŋutinu vovovowo me le ŋutilã bliboa me. Woyɔa esia be systemic amyloidosis .

Nya nyui aɖe li. Ðɔktawo ate ŋu atɔ te protein siwo mesɔ o siwo hea amyloidosis vɛ la wɔwɔ. Le amyloidosis ƒomevi aɖewo me la, mía ŋutɔwo ƒe dɔlélenutsiŋutete ate ŋu akpe ɖe mía ŋu míakɔ protein siawo ŋu. Esia ate ŋu aɖe wò dzesiwo dzi akpɔtɔ eye wòana ŋutinu siwo ŋu dɔlélea le ƒe lãmesẽ nanyo ɖe edzi.

Ðe amyloidosis ƒomevi aɖewo lia?

Ẽ, ɖɔktawo nɔa te ɖe protein si ƒomevi wòdze la dzi daa dɔléle sia ɖe hatsotsowo me. Ƒomevi vevi siwo wokpɔe nye:

• AL amyloidosis : Esia nye ne wò ŋutilã wɔa protein siwo le kɔsɔkɔsɔ si me kɔ (siwo nye dɔlékuiwutikewo ƒe akpa vevi aɖe) ƒe agbɔsɔsɔ si mesɔ o. Zi geɖe la, ka siawo ƒoa ƒu ɖe dzia (dzitodzito me amyloidosis) kple ayiku me. AL amyloidosis ate ŋu akpɔ ŋusẽ ɖe wò lãmekawo, ŋutigbalẽ, kple nuɖuɖumeŋusẽwo hã dzi. Esiae nye amyloidosis ƒomevi si bɔ wu.
• AA amyloidosis : Le esia me la, serum A protein kakɛwo ƒoa ƒu ɖe wò ŋutinuwo me. Esia ate ŋu adzɔ ne dɔdzẽ ƒe dɔléle aɖe si nɔa ŋuwò ɣeyiɣi didi, si fia dɔléle si naa dɔdzẽ le ŋutilã me (le kpɔɖeŋu me, ƒunukpeƒetetedɔ, dɔgbo ƒe dɔléle si naa dɔdzẽ, alo dɔlékui siwo nɔa anyi didi). Esia ate ŋu agblẽ nu le wò ayiku, nuɖuɖumeŋusẽ, alo dzi ŋu.
• ATTR amyloidosis: Le nɔnɔme sia me la, wò aklã wɔa transthyretin protein ƒe agbɔsɔsɔ si mesɔ o . Transthyretin protein sia si mesɔ o la gena ɖe dzia me eye ɣeaɖewoɣi la, egena ɖe lãmekawo me. Wonyia ƒomevi aɖewo ƒe dome (ƒome ƒe amyloidosis), eye bubuwo hã va nɔa tsitsim le eƒe agbe me ne ame le tsitsim (ATTR si nye gbemelã).

Aleke gbegbee dɔléle sia bɔe?

Le nyateƒe me la , amyloidosis nye dɔléle si mebɔ kura o . Le kpɔɖeŋu me, le United States gɔ̃ hã la, ɖɔktawo bu akɔnta be ame yeye 1,275 va ɖo 3,200 koe wokpɔna le AL amyloidosis ŋu ƒe sia ƒe. Eyata, menye dɔléle si bɔ o.

Nukae nye dɔléle sia ƒe dzesiwo?

Amyloidosis ƒe dzesiwo ate ŋu ato vovo tso ame ɖeka gbɔ yi bubu gbɔ, le protein si mesɔ o ƒe ƒomevi kple afisi kaawo le nu.

Bu eŋu kpɔ, ne protein siawo de dzia me (`(cardiac amyloidosis)`), ekema dzesi siwo dona tso emee nye gbɔdzɔgbɔdzɔ, gbɔgbɔ sesẽ, alɔ̃madɔmadɔ (esia ate ŋu anye dzi ƒe tsotso si mesɔ o ƒe dzesi) alo dzi ƒe dɔmawɔmawɔ nyuie si me afɔwo vuvu.

Azɔ hã, ne protein siawo de ayiku me (ayiku me amyloidosis) la, dzesiwo abe afɔ kple afɔwo ƒe vuvu kple kuɖɔwo kpɔkpɔ le aɖuɖɔ me ate ŋu ado mo ɖa.

Dzesi geɖe li siwo bɔ siwo woate ŋu akpɔ le amyloidosis me:

• Sese le eɖokui me be ɖeɖi te ye ŋu ale gbegbe.
• Lolo dzi ɖeɖe kpɔtɔ susu aɖeke manɔmee.
• Gbɔdzɔgbɔdzɔ le abɔwo me le goawo katã me alo woƒe lãme ʋuʋu, ŋutete manɔamesi be woalé nane nyuie o.
• Seselelãme be asiwo le ʋuʋum.
• Ŋutilã ƒe tɔtrɔ: Esia fia nuwo abe ŋutigbalẽa ƒe ƒoƒo bɔbɔe kple teƒe siwo le dzo dam (purpura) siwo ƒo xlã ŋkuwo ene.

Nukatae amyloidosis sia dzɔna?

Amyloidosis dzɔna ne protein siwo le míaƒe ŋutilã me megabla nyuie o eye woléna ɖe nu ŋu. Woƒoa ƒu ɖekae, wowɔa ƒuƒoƒo, alo ka, eye wotsia ŋutinuwo kple lãkusiwo me. Ðɔktawo yɔa esia be “protein ƒe babla si mesɔ o ƒe dɔléle.” Protein siwo wòle be woanɔ ɖoɖo nu, le kɔsɔkɔsɔ didiwo me, va xaxana eye ŋutilãa mete ŋu gbãa wo nyuie o, si hea kuxiwo vɛ.

Nusiwo ate ŋu ahe esia vɛ enye:

• Domenyiŋusẽfianu ƒe tɔtrɔ (nutations) : Àte ŋu anyi domenyiŋusẽfianu ƒe tɔtrɔ (mutations) si naa amyloid protein siwo mesɔ o la dome. Alo, domenyiŋusẽfianu ƒe tɔtrɔ siawo ate ŋu adzɔ le ame ƒe agbenɔɣi, le susu siwo womenya o ta.
• Atikewɔwɔ me nɔnɔme bubu siwo gbɔ wòtso: Ɣeaɖewoɣi la, atikewɔwɔ ƒe nɔnɔme bubu aɖe ate ŋu ahe amyloidosis vɛ. Esiae nye nusi dzɔna le AA amyloidosis me. Esia bɔ ɖe amesiwo ŋu dɔlékuiwo le ɣeyiɣi didi alo dɔdzẽ ƒe nɔnɔme siwo nɔa anyi didi abe ƒunukpeƒetetedɔ ene me wu.

Amekae ate ŋu axɔ nusia wu? (Afɔku Siwo Le Ame Ŋu) .

Afɔku geɖe li siwo ate ŋu akpɔ ŋusẽ ɖe amyloidosis ƒe dzɔdzɔ dzi:

• Ƒe : Ame siwo xɔ ƒe 60 alo esi wu nenema mee wokpɔa dɔléle sia le zi geɖe .
• Ŋutsu alo nyɔnu nyenye : Ŋutsuwo te ŋu xɔa dɔléle sia wu.
• Ameƒomevinyenye : Wokpɔe be ameyibɔ siwo le United States la nyi domenyiŋusẽfianu ƒe tɔtrɔ si hea domenyiŋusẽfianu si nye `ATTR amyloidosis` vɛ la dome wu. (Nyatakaka ʋɛ aɖewo koe li tso esia ŋu le míaƒe dukɔa me, gake zi geɖe la, wogblɔna be ameƒomevi aɖewo te ŋu xɔa ameƒomevi aɖewo bɔbɔe wu.)
• Atikewɔwɔ me nɔnɔme bubuwo : Ne dɔdzẽ ƒe dɔléle si nɔa anyi didi le ame ŋu la, edzia afɔku si le AA amyloidosis xɔxɔ me ɖe edzi. Azɔ hã, amesiwo ŋu multiple myeloma, si nye ʋumenugbagbeviɣidɔ ƒomevi aɖe le, ƒe 12% va ɖo 15% xɔa AL amyloidosis.
• Dialysis ɣeyiɣi didi : Ame siwo ŋu ayikudɔléle si nɔa anyi didi le siwo le dialysis dzi ɣeyiɣi didi aɖe hã ate ŋu axɔ amyloidosis ƒomevi aɖewo.
• Amyloidosis le ƒomea me : Amyloidosis ƒomevi aɖewo ate ŋu azɔ le ƒomewo me. Esia fia be woate ŋu anyi wo dome.

Kuxi kawoe esia ate ŋu ahe vɛ?

Amyloid siawo siwo le lãme na ame ate ŋu axe mɔ na ŋutinu siwo ŋu dɔlélea le be woagawɔ dɔ nyuie o. Kuxi siwo ate ŋu ado mo ɖa dometɔ aɖewoe nye:

• Dzi ƒe dɔwɔwɔ dzi ɖe kpɔtɔ eye mlɔeba dzi ƒe dɔmawɔmawɔ nyuie.
• Ayikudɔ kple ayiku ƒe dɔmawɔmawɔ nyuie mlɔeba .
• Ahɔhɔ̃mekawo ƒe dɔléle (si nana ame ƒe lãme ʋuʋuna eye wògbɔdzɔna).

Gake ɖo ŋku edzi be ne èdi atike na atikewɔlawo ƒe ƒuƒoƒo si sɔ la, àte ŋu aɖe kuxi siawo ƒe mɔnukpɔkpɔ dzi akpɔtɔ.

Aleke wowɔna dea dzesi dɔléle sia pɛpɛpɛ? (Dɔléle ƒe dzesi) .

Ðɔktawo wɔa lãmenugbagbeviwo ƒe dɔwɔwɔ be woatsɔ ade dzesi protein si hea amyloidosis vɛ. Esia bia be woaɖe lãkusi sue aɖe tso ŋutinu si ŋu dɔlélea le me alo tso teƒe si ka siawo ƒoa ƒu ɖo zi geɖe (le kpɔɖeŋu me, le ami si le wò ƒodo ƒe ŋutigbalẽ te alo wò ƒumeŋuɖui me).

Tsɔ kpe ɖe eŋu la, dodokpɔ bubuwo li nàte ŋu awɔ:

• Ʋu kple aɖuɖɔ dodokpɔ : Dodokpɔ siawo ate ŋu akpɔe ɖa be protein ƒe agbɔsɔsɔ si mesɔ o le wò ʋu alo aɖuɖɔ me hã. Woate ŋu azã wo hã atsɔ akpɔ alesi ŋutinu siwo ŋu dɔlélea le le dɔ wɔm.
• Nɔnɔmetatawo dodokpɔ: Dodokpɔ siawo ate ŋu akpe ɖe ɖɔktawo ŋu woakpɔ nusiwo gblẽ le ŋutinu siwo ŋu dɔlélea le ŋu. Le afisi kaawo le nu la, ahiã be woawɔ echocardiogram , si léa ŋku ɖe dzia ŋu, MRI , si ɖea ŋutinuwo kple lãkusiwo ƒe nɔnɔmetata tsitotsito fiana, alo dodokpɔ bubuwo.
• Domenyiŋusẽfianu dodokpɔ : Ne wò ɖɔkta susui be amyloidosis le ŋuwò le domenyiŋusẽfianu ƒe tɔtrɔ ta la, woate ŋu awɔ dodokpɔ sia.

Ðe dɔlélea ƒe akpa aɖewo lia?

Ẽ, ɖɔktawo zãa amyloidosis staging tsɔ nyaa afisi dɔlélea kaka ɖo kple alesi gbegbe wòsẽe. Dɔléle ƒomevi vovovowo ƒe ɖoɖo vovovowo li. Be wò ɖɔkta nate ŋu aɖo dɔlélea ɖe ɖoɖo nu la, ate ŋu abu nu siwo gbɔna ŋu:

• Afisi wògblẽa nu le ŋutinuwo ŋui.
• Ʋudodokpɔwo ɖea protein si mesɔ o ƒe agbɔsɔsɔ fiana ne wotsɔe sɔ kple amyloid protein si sɔ.
• Wò dzesiwo kple woƒe sesẽme.

Ele vevie ŋutɔ be nàƒo nu kple wò ɖɔkta tso afisi wò amyloidosis le kple alesi wòakpɔ ŋusẽ ɖe wò ŋgɔdonya dzii ŋu.

Dɔdamɔnu kawoe li?

Ðɔktawo kpɔa amyloidosis gbɔ to dɔléle si gbɔ wòtso la dada me. Atikewɔwɔ ate ŋu ana amyloidosis ƒe ŋgɔyiyi nagbɔdzɔ eye wòaxe mɔ na ka yeyewo ƒe dzɔdzɔ. Atikewo hã ate ŋu akpe ɖe ŋuwò nàɖe wò dzesiwo dzi akpɔtɔ. Le AL amyloidosis me la, ne atikewɔwɔ xe mɔ na amyloid yeyewo ƒe dzɔdzɔ la, wò dɔlélenutsiŋutete ate ŋu akɔ esiwo le afima xoxo la ɖa. Wogale numekuku wɔm be woadi mɔ siwo dzi woato ana amyloid ɖeɖeɖa le wò ŋutinuwo me nanyo ɖe edzi.

Atike siwo wozãna na amyloidosis dometɔ aɖewoe nye:

• Atikewɔwɔ : Atikewɔwɔ sia gblẽa ʋumenugbagbevi dzĩ siwo mesɔ o siwo wɔa protein siwo le kɔsɔkɔsɔ si me kɔ siwo gblẽ siwo hea AL amyloidosis vɛ la dome. Ame geɖe noa steroid kpe ɖe atike siwo wozãna tsɔ daa dɔlékuiwoe ŋu.
• Atikewɔwɔ si woɖo taɖodzinu na : Atikewɔwɔ sia tɔa ŋku protein, domenyiŋusẽfianu, alo lãkusi tɔxɛ aɖewo siwo hea amyloidosis vɛ. Elɔ atike siwo xea mɔ na proteinwo be woagabla nyuie o ɖe eme.
• Ŋutinuwo dodo na ame : Ne ayiku, aklã, alo dzi gblẽ ale gbegbe be megate ŋu wɔa dɔ nyuie o la, ɖɔktawo ate ŋu aɖo aɖaŋu be woado ayiku na ame bubu. Esia ate ŋu anye atike na amyloidosis ƒomevi siwo ƒe dome wonyi.

Nuka ŋue wòle be amesi ŋu amyloidosis le natsi dzi ɖo?

Wò ɖɔkta ate ŋu ada wò dzesiwo, akpɔ dɔlélea ƒe kaka dzi, eye le go aɖewo me la, akpe ɖe ŋuwò gɔ̃ hã be dɔlélea natrɔ. Gake ne womeda amyloidosis ƒomevi aɖewo o la, ate ŋu agblẽ nu le ŋutinuwo ŋu wòade ame ƒe agbe afɔku me. Esia tae wòle vevie ŋutɔ be woade dzesi dɔlélea kaba eye woadze edada gɔme kaba.

Aleke nèdi be yealé be na ye ɖokui hã?

Esi wònye be amyloidosis ƒomevi vovovowo li ta la, mɔnu ɖeka aɖeke meli si dzi woato akpɔ dɔlélea gbɔ o. Bia wò ɖɔkta be afɔɖeɖe siwo sɔ na wò hã.

Esia ate ŋu alɔ:

• Wò ŋutilã ƒe lãmesẽ dzi kpɔkpɔ to nuɖuɖu nyui siwo me nunyiame le ɖuɖu kple kamedede edziedzi me .
• Tsɔ wò susu ƒe lãmesẽ ɖo nɔƒe gbãtɔ . Bia wò ɖɔkta tso kpekpeɖeŋunaha siwo naa amesiwo ŋu amyloidosis le ŋu. Ate ŋu anye mɔ nyui aɖe si dzi nàto anɔ te ɖe nuteɖeamedzi kple akogotsitsi si le agbenɔnɔ kple dɔléle si mebɔ o alea me nu, eye nàdo ka kple ame bubu siwo le nu ma ke me tom abe wò ene.

Ɣekaɣie wòle be nàkpɔ ɖɔkta?

Esi wònye be dzesiawo to vovo ale gbegbe ta la, ate ŋu asesẽ be woanya ɣeyiɣi si wòle be woakpɔ ɖɔkta. Gake le goawo katã me la, ne dzesi aɖewo le ŋuwò si gɔme mèse o eye medzo le ɣeyiɣi aɖe megbe o la, ele be nàyi ɖɔkta gbɔ godoo. Ðewohĩ menye amyloidosis ye o, gake ne ele nenema la, zi alesi nènyae kaba la, zi nenemae wòanyo wu.

Nya kawoe wòle be nàbia ɖɔktaa?

Nya aɖewo siwo nàte ŋu abia wò ɖɔktae nye esi:

• Amyloidosis ƒomevi ka ƒomevie le ŋunye?
• Nukatae amyloidosis dze dzinye?
• Atikewɔwɔ ka ƒomevie mehiã?
• Nukawoe nye atikewɔwɔa ƒe nugbegblẽ le ame ŋu?
• Nukae nye nye ŋgɔdonya le atikewɔwɔ vɔ megbe?

Mlɔeba la, nusiwo dzi wòle be woaɖo ŋkui (Take-Home Message) .

Amyloidosis nye dɔléle sesẽ ŋutɔŋutɔ elabena edzea amesiame dzi le mɔ vovovowo nu. Le go aɖewo me la, woate ŋu akpɔ edzi. Le go bubuwo me la, ate ŋu ade ame ƒe agbe afɔku me. Esia ate ŋu anye nuteƒekpɔkpɔ si tɔtɔa ame eye wòagbɔ eme. Azɔ hã, esi wònye dɔléle si mebɔ o ta la, àte ŋu ase le ɖokuiwò me be ye ɖeka koe le ɣeyiɣi si me yehiã kpekpeɖeŋu wu.

Nu vevitɔe nye be nàɖo ŋku edzi be menye wò ɖeka koe o. Wò ɖɔkta ate ŋu aɖe atikewɔmɔnu nyuitɔwo me na wò, le amyloidosis ƒomevi si le ŋuwò nu. Woate ŋu afia asi dɔwɔnuwo kple kpekpeɖeŋunaha siwo ate ŋu akpe ɖe ŋuwò hã. Wò atikewɔlawo ƒe ƒuƒoƒo ate ŋu afia mɔ wò be nàdi atike nyuitɔ kekeake kple kpekpeɖeŋu si nèhiã be nàtsɔ awɔ avu kple dɔléle sia.

` Amyloidosis, proteinwo, protein siwo mesɔ o, ŋutinuwo ƒe gbegblẽ, dzidɔ, ayikudɔ, ahɔhɔ̃medɔlélewo