Ðe ʋu dona le wò aɖuɖɔ me ɣeaɖewoɣi, alo wò afɔwo vuvua? Alo ɖe ɖeɖi tea ŋuwò ɣesiaɣia? Esiawo ate ŋu atso kuxi aɖe si le wò ayiku ŋu gbɔ ɣeaɖewoɣi. Egbea míele nu ƒom tso nɔnɔme aɖe si mebɔ o gake wòle vevie ŋutɔ si gblẽa nu le ayiku ŋu la ŋu. Emae nye C3G (C Etɔ̃ G) alo ``Kpekpeɖeŋu 3 Glomerulopathy``.

Nukae nye C3G (Kpekpeɖeŋu 3 Glomerulopathy)?

Kpuie ko la, C3G nye dɔlélewo ƒe ƒuƒoƒo aɖe si kpɔa ŋusẽ ɖe alesi wò ayikuwo wɔa dɔ nyuie dzi. Ènyaa, ayikuawo le abe nu eve siwo le míaƒe ŋutilã me ene. Woƒe dɔ vevitɔe nye be woaɖe ʋua, aɖe gbeɖuɖɔ siwo mehiã o kple tsi si sɔ gbɔ ɖe eme, eye woawɔ aɖuɖɔ.

"Complement 3" nye protein tɔxɛ aɖe le míaƒe ʋu me. Ele vevie ŋutɔ na dɔlélenutsiŋutete si wɔa avu kple dɔlélewo le míaƒe ŋutilã me. "Glomerulopathy" nye nusiwo gblẽ le glomeruli ŋu, siwo nye ʋuka suesuesue siwo le ayiku me, siwo le abe lãgbalẽ suesuesuewo ene. Glomeruli siawoe tsia ʋua me.

Emegbe C3G nye dɔléle si lɔ `complement 3` protein ɖe eme, si gblẽa nu le `glomeruli` ŋu eye wògblẽa ayiku ƒe dɔwɔwɔ me. Tsã la, si fia be do ŋgɔ na ƒe 2013 la, ɖɔktawo yɔa esia be ``Membranoproliferative glomerulonephritis (MPGN) ƒomevi I, II alo III`` alo ``Mesangioproliferative glomerulonephritis``. Gake fifia woyɔa dɔlélewo ƒe hatsotso sia be C3G.

Ðe C3G ƒomevi aɖewo lia?

Ẽ, C3G ƒomevi vevi evee li. Ðɔktawo dea dzesi ƒomevi eve siawo to ayiku ƒe akpa sue aɖe ɖeɖe (míeyɔnae be `ayiku ƒe lãkusi` ) kple ŋkuléle ɖe eŋu le nusuekpɔmɔ̃ me, le nusiwo gblẽ le `glomeruli` ŋu kple eƒe dɔdzẽ nu.

• Dense deposit disease (DDD): Woyɔa nɔnɔme sia tsã be MPGN ƒomevi II, eye nusiwo le abe lãgbalẽ ene ƒe wɔwɔme le glomerular basement membrane (GBM), si nye glomeruli ƒe nutsyɔnu me. Edzea ɖeviwo kple sɔhɛ tsitsi siwo xɔ ƒe 20 kple edzivɔwo dzi wu.

• `C3 glomerulonephritis` (C3GN): Woyɔnɛ tsã be `MPGN ƒomevi I` alo `III`. Le esia me la, womekpɔa nusiwo le ƒuƒlu abe esiwo wokpɔ le DDD me ene o. Esia gblẽa nu le amesiwo xɔ wu ƒe 30 ŋu wu.

Ðe C3G nye dɔléle si nɔa anyi didia?

Ẽ, C3G nye dɔléle si nɔa anyi didi. Esia fia be menye nane si ava do kpata eye wòahaya o, eye nusiwo gblẽna le ayikuwo ŋu ate ŋu adzi ɖe edzi le ɣeyiɣi aɖe megbe. Eyata ele vevie ŋutɔ be míaɖɔ ŋu ɖo le esia ŋu.

Aleke gbegbee C3G sia bɔe?

Le nyateƒe me la, esia nye dɔléle si mebɔ kura o.Ðɔktawo bu akɔnta be esia dzɔna ɖe ame siwo ade eve va ɖo etɔ̃ dzi le ame akpe alafa ɖeka me. Eyata ɖewohĩ mèse nu geɖe tso eŋu o. Gake togbɔ be mebɔ o hã la, ele vevie be nànyae.

Nukae nye C3G dɔlélea ƒe dzesiwo?

Azɔ mina míakpɔ nusiwo nye C3G dɔléle sia ƒe dzesiwo ɖa. Nu geɖe siwo bɔ ɖe ƒomevi eveawo siaa si, siwo le vevie be woade dzesii:

• Ʋu si le aɖuɖɔ me ( hematuria ): Ɣeaɖewoɣi la, aɖuɖɔa ate ŋu adze abe ɖe wòbiã ene, alo ate ŋu anye agbɔsɔsɔ sue aɖe si woate ŋu akpɔ le nusuekpɔmɔ̃ me ko.
• Protein si sɔ gbɔ ɖe aɖuɖɔ me (proteinuria): Esia ate ŋu ana aɖuɖɔa nadze abe futukpɔ ene.
• Aɖuɖɔtoe si mebɔ o ƒe dodo wu alesi wòle dzɔdzɔmee (oliguria).
• Protein ƒe agbɔsɔsɔ si dzi ɖe kpɔtɔ le ʋu me (hypoalbuminemia).
• Vuvu (edema): Vevietɔ le asiwo, afɔkpodzi, kple afɔwo me. Bu eŋu kpɔ, ne èfɔ le ŋdi me eye wò afɔwo vuvu la, ate ŋu anye nane si le abe esia ene.
• Ʋusɔgbɔdɔ (ʋusɔgbɔdɔ).
• Dɔlékuiwo xɔxɔ enuenu.
• Ðeɖi te ŋuwò ŋutɔ (`Ðeɖiteameŋu`): Menye ɖeɖiteameŋu koe o, ke boŋ ɖeɖiteameŋu ƒe seselelãme eɖanye aleke kee nèdɔ alɔ̃ o.
• Ame aɖewo ate ŋu axɔ nɔnɔmewo abe gout ene .

Dzesi siawo evea siaa ate ŋu ana ayiku nawɔ dɔ nyuie o . Ayiku ƒe dɔmawɔmawɔ nyuie hã ate ŋu ahe dzesiwo abe:

• Ðeɖi tea eŋu ɣesiaɣi.
• Aɖuɖɔtoe, ʋuʋudedi.
• Ame ƒe tameɖoɖo kplikpaa ƒe sesẽ, mɔkpɔkpɔbuɖeame.
• Ŋutilã ƒe vuvu.
• Aɖuɖɔ geɖe dodo wu alesi wònɔna ɖaa (woate ŋu akpɔ esia ɣeaɖewoɣi le ayiku ƒe dɔmawɔmawɔ nyuie ƒe gɔmedzedze).

Tsɔ kpe ɖe ayiku ŋu la, C3G ate ŋu ahe dzesi bubuwo hã vɛ. Esiawo dometɔ aɖewoe nye:

• Nukpɔkpɔ ƒe kuxiwo: Nukpɔkpɔ ate ŋu agblẽ le protein kple calcium ƒe ƒuƒoƒo (drusen) ta, vevietɔ le ŋku ƒe ŋkume.
• Lipodystrophy: Esia nye nɔnɔme si me ami buna le ŋutilã ƒe akpa aɖewo (vevietɔ mo kple abɔ kple afɔwo).

Nukatae wowɔ C3G sia?

Enyo, azɔ mina míakpɔ nusita wowɔ C3G sia ɖa. Esia sesẽ vie, gake maɖe eme bɔbɔe. Esia dzɔna ne ``complement system`` si le míaƒe ŋutilã me mele dɔ wɔm nyuie o. ``Kpekpeɖeŋunaɖoɖo`` sia nye protein siwo le míaƒe ʋu me ƒe ƒuƒoƒo. Esia nye míaƒe dɔlélenutsiŋutete ƒe akpa aɖe. Esia kpena ɖe ame ŋu wòwɔa avu kple nuwo abe dɔlékuiwo kple dɔlékui siwo tso gota ene.

Nusi dzɔna ɖe ame aɖe si ŋu C3G le dzi enye be wowɔa `complement system` sia ŋudɔ akpa, alo womekpɔa ŋusẽ ɖe edzi o. Emegbe protein si nye `complement 3 (C3)` la gblẽna. C3 protein kakɛ siawo siwo gblẽ la tsia `glomeruli` me. Emegbe eva sesẽna na `glomeruli` be woaɖe ʋu ahawɔ aɖuɖɔ. Ne womewɔ atike nɛ o la, nusiwo gblẽ le ame ŋu sia ate ŋu ayi edzi eye wòana ayiku nawɔ dɔ nyuie o.

Zi geɖe la, esesẽna be woade dzesi susu si tututu ta ``kafukafu ƒe ɖoɖo'' sia yia edzi le mɔ gbegblẽ nu. Gake ɣeaɖewoɣi la:

• C3G ateŋu ado tso tɔtrɔ siwo va le míaƒe domenyiŋusẽfianuwo me (trɔtrɔ le domenyiŋusẽfianu siwo do ƒome kple kpeɖeŋutɔ ƒe ɖoɖoa me, abe ``C3`` kple ``CFH`` ene).
• Ʋumedɔ si woyɔna be ``Monoclonal gammopathy`` hã ateŋu agblẽ nu le ayikuwo ŋu le mɔ si mele tẽ o nu, agblẽ ``complement`` ɖoɖoa ƒe dɔwɔwɔ me, eye wòana C3G nadzɔ. ``Monoclonal gammopathy`` sia nye nɔnɔme si gblẽa nu le ʋumenugbagbevi siwo le míaƒe ƒumeŋuɖui me ŋu.
• Atikewɔwɔ ŋuti numekulawo susu be nane si woyɔna be ``autoantibodies'' hã ateŋu awɔ akpa aɖe. ``Autoantibodies'' nye protein siwo mía ŋutɔwo ƒe dɔlélenutsiŋutete wɔ siwo dzea mía ŋutɔwo ƒe lãmeka siwo le lãmesẽ dzi dzi vodadatɔe. Ke hã, numekuku mesɔ gbɔ be woagblɔe kple kakaɖedzi be ``autoantibodies'' siawo gbɔe C3G tsona o.

Amekae C3G dɔléle sia gblẽa nu le wu?

C3G dɔléle sia ate ŋu adze ame sia ame dzi. Ekpɔa ŋusẽ ɖe ŋutsuwo kple nyɔnuwo siaa dzi sɔsɔe. Ƒe si woxɔna hafi wokpɔa dɔlélea le mama dedie nu anɔ abe ƒe 23 ene. Ke hã, `DDD` ƒomevi si míeyɔ va yi ateŋu adze le ƒe si metsi o wu `C3GN` ƒomevi.

Nukawoe nye kuxi siwo ate ŋu ado tso C3G me?

Kuxi vevitɔ kple esi me afɔku le wue nye be amesiwo ŋu C3G le ƒe 50% lɔƒo, alo ɖeka le eve ɖesiaɖe me, axɔ ayiku ƒe dɔmawɔmawɔ nyuie le ƒe 10 me tso esime wokpɔ dɔlélea. Esiae wòle be mí katã míavɔ̃ wu.

Aleke wowɔa dea dzesi C3G dɔlélea?

Ðɔkta awɔ dodokpɔ geɖe atsɔ akpɔ wò ayiku ƒe lãmesẽ kple alesi wole dɔ wɔm nyuie. Esiawo ate ŋu anye:

• Ʋudodokpɔ: Woɖea ʋu sue aɖe tso ʋuka aɖe si le wò alɔnu me eye dodokpɔxɔa léa ŋku ɖe eŋu be wò kpeɖeŋutɔ proteinwo sɔ alo ne gbeɖuɖɔ siwo sɔ gbɔ wu (e.g. creatinine) le wò ʋu me hã.
• `Estimated Glomerular Filtration Rate (eGFR): Esia nye dodokpɔ si dzidzea alesi wò ayikuwo le wò ʋu ƒom nyuie, si wotu ɖe `serum creatinine` alo `cystatin C` ƒe agbɔsɔsɔ si le ʋu me dzi. Esia ate ŋu ana nàkpɔ alesi wò ayikuwo le dɔ wɔm nyuie.
• Aɖuɖɔtoe me dzodzro: Ahiã be nàɖe aɖuɖɔ ɖe kplu tɔxɛ aɖe me eye nàtsɔ kpɔɖeŋua ana wò ɖɔkta. Nudokpɔƒea alé ŋku ɖe protein alo ʋu si le aɖuɖɔa me ŋu, eye nusuekpɔmɔ̃ koe woate ŋu akpɔe le.
• Ayiku me lãmenugbagbeviwo ɖeɖe: Esiae nye nusiMɔ nyuitɔ si dzi woato anya C3G dɔlélea godoo enye to ŋutilã ƒe akpa aɖewo ɖeɖeɖa me. Le esia me la, ɖɔkta tsɔa aŋe nyui aɖe alo lãgbalẽ sue aɖe ɖea lãkusi sue aɖe tso wò ayiku me eye wòdonɛ kpɔna le dodokpɔxɔ me. Ne C3 protein si gblẽ ƒe kakɛwo le kpɔɖeŋu sia me le wò glomeruli me la, ate ŋu anye C3G dɔléle. Biopsy sia ate ŋu agblɔ C3G ƒomevi si le asiwò hã (DDD alo C3GN), le nenye be nu sesẽ mawo le asiwò alo mele asiwò o nu.

Ðe woate ŋu ada C3G keŋkeŋa?

Nublanuitɔe la, fifia la, womekpɔ atike aɖeke na C3G dɔlélea o. Gake megatsi dzi o, ɖɔktawo kple numekulawo yi edzi le nu srɔ̃m tso dɔléle sia ŋu eye wole dɔ wɔm be yewoadi atike yeyewo. Míele mɔ kpɔm be atikewɔwɔ si nyo wu ava le etsɔme.

Nukawoe nye atikewɔwɔ na C3G?

Fifia la, atikewɔwɔ tɔxɛ aɖeke meli si dzi U.S. Nuɖuɖu Kple Atike Ŋuti Dɔwɔƒe (FDA) da asi ɖo na C3G o. Gake ɖɔktawo ate ŋu aŋlɔ atike vovovowo kple beléle si akpe ɖe ame ŋu be C3G ƒe ŋgɔyiyi nagbɔdzɔ, aɖu dzesiwo dzi, eye woaɖe ayiku ƒe gbegblẽ dzi akpɔtɔ. Esiawo dometɔ aɖewoe nye:

• Ʋu ƒe sisi ƒe atikewo: Atikewo abe angiotensin-converting enzyme (ACE) inhibitors kple angiotensin II receptor blockers (ARBs) ene ɖea nyaƒoɖeamenu si le glomeruli me dzi kpɔtɔna. Esia ɖea protein ƒe agbɔsɔsɔ si le aɖuɖɔ me dzi kpɔtɔna eye wòɖea ayikuawo ƒe teteɖeanyi dzi kpɔtɔna.
• Atike siwo ɖea ʋumemi dzi kpɔtɔna: Wò ɖɔkta ate ŋu aŋlɔ atikewo abe statin ene na wò be wòaɖe ʋumemi dzi akpɔtɔ eye wòaɖe nusiwo gblẽ le wò ayiku ŋu dzi akpɔtɔ.
• Nuɖuɖu ƒe tɔtrɔ: Ayiku ŋuti nunyala akpe ɖe ŋuwò nàwɔ nuɖuɖu ŋuti ɖoɖo si sɔ na wò eye wòakpe ɖe ŋuwò nàlé wò lolo nyuie me ɖe asi. Dze (sodium), ami si me ami sɔ gbɔ ɖo, kple ʋumemi ɖuɖu dzi ɖeɖe kpɔtɔ ate ŋu akpe ɖe ŋuwò nàɖe ayiku ƒe gbegblẽ dzi akpɔtɔ.
• Corticosteroids: Wogayɔnae be "steroids," esiawo nye atike siwo tsia dɔdzẽ nu siwo wɔa dɔ to mɔxexe ɖe dɔlélenutsiŋutete ƒe dɔwɔwɔ nu be wòagadze wò glomeruli dzi o.
• Eculizumab: Esia nye dɔlékuiwutike si wozãna tsɔ daa ʋumenugbagbeviɣidɔ siwo mebɔ o. Ate ŋu akpe ɖe ayiku ƒe dɔwɔwɔ nyuie wu to protein si le aɖuɖɔ me dzi ɖeɖe kpɔtɔ kple glomerular filtration rate dzi ɖeɖe kpɔtɔ le C3G dɔnɔ aɖewo me.
• Dialysis: Wogblɔ be ayiku me gblẽ le amesiwo ŋu C3G le dometɔ siwo ade 50% ŋu. Dialysis nye ne ayikuawo mewɔ dɔ nyuie o, eye wotsɔa mɔ̃ aɖe wɔa dɔ si wòle be ayikuawo nawɔ (akɔ ʋua ŋu, aɖe gbeɖuɖɔwo ɖa, kple tsi si sɔ gbɔ ɖe eme).
• Ayiku dodo: Esia nye ayiku ƒe dɔmawɔmawɔ nyuie ƒe atike bubu. Amekola aɖe ɖea wò ayiku si le gbagbãm la ɖa eye wòtsɔa ayiku si le lãmesẽ me si tso amesi na wò ayiku gbɔ la ɖɔlia.

Ne `DDD` alo `C3GN` le ŋuwò eye `monoclonal gammopathy` hã le ŋuwò la, wò ɖɔkta aɖo aɖaŋu be nàwɔ dodokpɔ bubuwo atsɔ akpɔ nɔnɔme bubu siwo le eme hã.

Nukae nye C3G dɔlélea ƒe mɔkpɔkpɔ?

Dɔnɔ siwo ŋu C3G le ƒe afã lɔƒo ƒe ayiku ƒe dɔmawɔmawɔ nyuie le ƒe 10 me tso esime wokpɔ dɔlélea. Fifia womekpɔ atike aɖeke na ayiku ƒe dɔmawɔmawɔ nyuie o. Gake ne wokpɔ dɔlélea nyuie eye wowɔ atike nɛ kaba la, àte ŋu anɔ agbe didi le lãmesẽ me tɔtrɔ gã aɖeke manɔmee. Eyata ele vevie be nànɔ anyi kpoo ahawɔ ɖe wò ɖɔkta ƒe aɖaŋuɖoɖo dzi.

Ðe woate ŋu axe mɔ ɖe C3G dɔlélea nua?

Nublanuitɔe la, mɔ aɖeke meli si dzi woato axe mɔ ɖe C3G dɔlélea nu fifia o, elabena zi geɖe la, nusiwo dzi míate ŋu aɖu o gbɔe wòtsona.

Aleke malé be na ɖokuinye?

Ne C3G le ŋuwò la, wò ɖɔkta awɔ atikewɔwɔ ƒe ɖoɖo nyuitɔ kekeake na wò. Esia ate ŋu alɔ atikewo zazã, kpekpeɖeŋunana ame, kple agbenɔnɔ ƒe tɔtrɔ ɖe eme. Nusiwo gbɔna ate ŋu anɔ wò atikewɔwɔ ƒe ɖoɖoa me, eye ele vevie be nàwɔ ɖe afɔɖeɖe siawo dzi:

• No wò atike abe alesi wò ɖɔkta gblɔe ene pɛpɛpɛ. Mègana ŋkeke ɖeka pɛ hã nato ŋuwò o.
• Wò nuɖuɖu tɔtrɔ, vevietɔ dze, ami, kple sukli dzi ɖeɖe kpɔtɔ, kple atikutsetse kple amagbe geɖe ɖuɖu.
• Ƒo asa na atike siwo me steroid mele o (NSAIDs) (e.g., vevesesenutsitikewo abe ibuprofen kple diclofenac) zazã atikewɔlawo ƒe aɖaŋuɖoɖo manɔmee, elabena woate ŋu agblẽ nu le ayikuwo ŋu eye woana ayiku nawɔ dɔ kabakaba.
• Wò susu ƒe lãmesẽ dzi kpɔkpɔ. Dɔléle sia tɔgbe si mebɔ o ƒe amesinɔnɔ ate ŋu ana nàtsi akogo eye vɔvɔ̃ ɖo wò. Azɔ hã, ne ènya be C3G ate ŋu ana ayiku nawɔ dɔ nyuie la, edzia blanuiléle ƒe afɔkua ɖe edzi. Ðo dze kple wò ɖɔkta tso wò susu ƒe lãmesẽ ŋu eye nàdi aɖaŋuɖoɖo kple atikewɔwɔ ne ehiã. Bia wò ɖɔkta tso kpekpeɖeŋunaha siwo naa amesiwo ŋu C3G le ŋu. Ame bubu siwo ŋu C3G le do go ate ŋu akpe ɖe ŋuwò nàɖe akogotsitsi dzi akpɔtɔ eye nàgblɔ nuteƒekpɔkpɔwo.

Ɣekaɣie wòle be makpɔ ɖɔkta?

Ne C3G ƒe dzesiwo le ŋuwò, vevietɔ tɔtrɔ le wò aɖuɖɔ me (ʋu, futukpɔ, mebɔ o), vuvu, alo ɖeɖiteameŋu si gbɔ eme la, yi ɖɔkta gbɔ enumake. Esiawo ate ŋu anye dzesi siwo fia be wò ayikuwo mele dɔ wɔm nyuie o.

Ne wokpɔe be C3G le ŋuwò la, wò ɖɔkta aɖo wò ayi ayikudɔ ŋuti nunyala bibi aɖe (anyidɔŋutinunyala). Eyae anye amesi ana atikewɔwɔ ƒe ɖoɖo nyuitɔ kekeake wò.

Nya kawoe wòle be mabia nye ɖɔkta?

Ne èyi ɖɔkta gbɔ la, mègahe ɖe megbe le nya siawo tɔgbe biabia me o:

• Aleke mawɔ anya kple kakaɖedzi be C3G le ŋunye?
• Ne C3G mele ŋunye o la, dɔléle bubu kae ate ŋu adze dzinye?
• C3G ƒomevi kae le asinye (DDD alo C3GN)?
• Atikewɔwɔ kawoe nèkafu nam? Ðe eƒe nugbegblẽ le ame ŋu aɖewo lia?
• Tɔtrɔ kawoe wòle be mawɔ le nye nuɖuɖu ŋu?
• Èsusu be nye ayikuwo awɔ dɔ nyuiea? Nukae mate ŋu awɔ be esia nagadzɔ o?
• Ðe mate ŋu akpɔ ayikuŋutinunyala aɖea?

Mlɔeba la, ele nam be magblɔ be...

C3G nye dɔléle si mebɔ o, si sesẽ, si gblẽa nu le ayiku ƒe akpa suesuesue siwo woɖena le ayiku me ŋu. Ne C3G le ŋuwò la, àte ŋu akpɔ tɔtrɔ le wò aɖuɖɔ me, avuvu, ƒunukpeƒeveame, nukpɔkpɔ ƒe kuxiwo, kple ɖeɖiteameŋu.

Àte ŋu ase seselelãme vovovowo ne èlɔ̃ ɖe dɔléle sia dzi eye nèle tɔtrɔm ɖe alesi C3G akpɔ ŋusẽ ɖe wò agbenɔnɔ dzii ŋu. Mègatsi dzi o, menye wò ɖeka koe o. Na ɣeyiɣi kple teƒe ɖokuiwò be nàsrɔ̃ nu tso C3G kple wò atikewɔwɔ ƒe tiatiawɔblɔɖewo ŋu. Ne èsrɔ̃ nu tso tiatia siwo nàte ŋu awɔ kple nusiwo nàkpɔ mɔ na ŋu la, ate ŋu akpe ɖe ŋuwò nàkpɔ wò seselelãmewo gbɔ le mɔ si le lãmesẽ me eye wòaɖe vi nu. Ðɔktawo le afima be yewoana nyatakaka kple mɔfiame. Ne nya aɖe le asiwò, nèhiã aɖaŋuɖoɖo alo kpekpeɖeŋu la, ƒo nu kpli wo. Ðo ŋku edzi be ne èkpɔ dɔléle sia kaba hedae nyuie la, àte ŋu aɖu dɔléle sia dzi ahanɔ agbe nyui.

` C3G, ayikudɔ, kpeɖeŋutɔ 3, glomerulopathy, ayiku ƒe dɔmawɔmawɔ nyuie, aɖuɖɔ me protein, dialysis