So yɛbɛka yare bi a ɛntaa nsi a wɔfrɛ no C3G (Complement 3 Glomerulopathy) , a ɛka asaabo no ho asɛm?

So ɛtɔ mmere bi a wote mogya wɔ wo nsu mu, anaasɛ wo nan ho ahonhon? Anaasɛ wote nka sɛ woabrɛ bere nyinaa? Ɛtɔ mmere bi a eyinom betumi afi ɔhaw bi a ɛwɔ w’asaabo ho. Ɛnnɛ yɛrebɛka tebea bi a ɛntaa nsi nanso ɛho hia paa a ɛka asaabo ho asɛm. Ɛno ne C3G (C G Abiɛsa) anaa Complement 3 Glomerulopathy.

Dɛn ne C3G (Complement 3 Glomerulopathy)?

Sɛ yɛbɛka no tiawa a, C3G yɛ nyarewa kuw bi a ɛka sɛnea w’asaabo yɛ adwuma yiye. Wunim, asaabo no te sɛ afiri abien a ɛwɔ yɛn nipadua mu no. Wɔn adwuma titiriw ne sɛ wobeyi mogya no mu, ayi nneɛma fĩ a ɛho nhia ne nsu a ɛboro so afi hɔ, na wɔayɛ nsu.

"Complement 3" yɛ protein soronko bi a ɛwɔ yɛn mogya mu. Ɛho hia paa ma nipadua no mu tumi a ɛko tia nyarewa a ɛwɔ yɛn nipadua mu no. "Glomerulopathy" yɛ glomeruli a ɛyɛ mogya ntini nketenkete a ɛwɔ asaabo no mu a ɛte sɛ nhama nketewa a ɛsɛe. Saa glomeruli yi na ɛma mogya no yiyi mu.

Afei C3G yɛ yareɛ a ɛfa complement 3 protein ho, ɛsɛe glomeruli na ɛsɛe asaabo no dwumadi. Bere bi a atwam, kyerɛ sɛ, ansa na afe 2013 reba no, nnuruyɛfo frɛɛ eyi Membranoproliferative glomerulonephritis (MPGN) type I, II anaa III anaa Mesangioproliferative glomerulonephritis. Nanso seesei wɔfrɛ nyarewa kuw yi C3G.

So C3G ahorow bi wɔ hɔ?

Yiw, C3G ahorow atitiriw abien na ɛwɔ hɔ. Nnuruyɛfo kyerɛ saa ahorow abien yi denam asaabo no fã ketewaa bi a wɔfa (yɛfrɛ no asaabo mu nhwehwɛmu ) na wɔhwɛ no wɔ afiri a wɔde hwɛ nneɛma nketenkete ase, a egyina ɔsɛe ne ɔfe a ɛwɔ glomeruli no so.

• Dense deposit disease (DDD): Kan no na wɔfrɛ no MPGN type II, na na ɛda adi sɛ nneɛma a ɛyɛ den a ɛte sɛ ribbon a ɛwɔ glomerular basement membrane (GBM), a ɛyɛ glomeruli a ɛkata so no mu. Ɛtaa ka mmofra ne mmerante ne mmabaa a wɔadu mpanyin afe so a wɔadi mfe 20 mfiase no.

• C3 glomerulonephritis (C3GN): Kan no na wɔfrɛ no MPGN type I anaa III. Wɔ eyi mu no, wontumi nhu nneɛma a ɛyɛ den te sɛ nea wohu wɔ DDD mu no. Eyi ka nnipa a wɔadi boro mfe 30 no kɛse.

So C3G yɛ yare a enni sabea?

Yiw, C3G yɛ yare a enni sabea. Eyi kyerɛ sɛ ɛnyɛ biribi a ebenyin mpofirim na ayɛ yiye, na bere kɔ so no, nneɛma a ɛsɛe asaabo no betumi ayɛ kɛse. Enti, ɛho hia paa sɛ yɛhwɛ yiye wɔ eyi ho.

C3G yi abu so dɛn?

Eyi yɛ yare a ɛntaa nsi koraa ankasa.Nnuruyɛfo bu akontaa sɛ eyi ba bɛyɛ nnipa baanu kosi baasa wɔ mpem ɔha biara mu. Enti ebia woante ho asɛm pii. Nanso ɛwom mpo sɛ ɛntaa nsi de, nanso ɛho hia sɛ wohu.

Dɛn ne C3G yare no ho sɛnkyerɛnne?

Afei ma yɛnhwɛ nea C3G yare yi ho sɛnkyerɛnne yɛ. Nneɛma abien no nyinaa wɔ nneɛma pii a ɛyɛ pɛ, a ɛho hia sɛ yehu:

• Mogya a ɛwɔ nsu mu ( hematuria ): Ɛtɔ mmere bi a, nsu no betumi ayɛ kɔkɔɔ, anaasɛ ebetumi ayɛ kakraa bi a wotumi hu wɔ afiri a wɔde hwɛ nneɛma nketenkete mu nkutoo.
• Protein a ɛboro so wɔ nsu mu (proteinuria): Eyi betumi ama nsu no ayɛ te sɛ nea ahuru.
• Nsu kakraa bi a wɔde fa mu sen sɛnea ɛte daa (oliguria).
• Protein dodow a ɛso tew wɔ mogya mu (hypoalbuminemia).
• Ɔhonhon (edema): Titiriw wɔ nsa, nansoaa, ne anan mu. Fa no sɛ, sɛ wosɔre anɔpa na wo nan te nka sɛ ahonhon a, ebetumi ayɛ biribi a ɛte sɛɛ.
• Mogya mmoroso (hypertension).
• Nyarewa a ɛsan ba bio.
• Ɔbrɛ kɛse a wobɛte nka (Ɔbrɛ): Ɛnyɛ ɔbrɛ nko, na mmom atenka a ɛne sɛ woabrɛ ɛmfa ho sɛnea wobɛda.
• Ebia nnipa binom benya tebea horow te sɛ gout .

Saa sɛnkyerɛnne abien yi nyinaa betumi ama asaabo ayɛ adwuma yiye . Asaabo a entumi nyɛ adwuma yiye nso betumi de sɛnkyerɛnne ahorow te sɛ:

• Bere nyinaa na ɔbrɛ.
• Akisikuru, ɔfe.
• Ɛyɛ den sɛ obi besi n’adwene, abasamtu.
• Nipadua a ɛho ahonhon.
• Nsu pii a wɔde fa mu sen sɛnea wɔtaa yɛ (ɛtɔ mmere bi a wobetumi ahu eyi wɔ asaabo a entumi nyɛ adwuma yiye no mfiase).

Wɔ asaabo akyi no, C3G nso betumi de sɛnkyerɛnne afoforo aba. Eyinom bi ne:

• Aniwa mu haw ahorow: Anisoadehu betumi asɛe esiane protein ne calcium a ɛwɔ hɔ (drusen) a ɛboaboa ano nti, titiriw wɔ macula no mu.
• Lipodystrophy: Eyi yɛ tebea a ɛma srade yera wɔ nipadua no mmeae bi (titiriw anim ne akwaa).

Dɛn nti na wɔhyehyɛ C3G yi?

Okay, afei momma yɛnhwɛ nea enti a wɔhyehyɛɛ saa C3G yi. Eyi yɛ nea ɛyɛ den kakra, nanso mɛkyerɛkyerɛ mu tiawa. Eyi ba bere a complement system a ɛwɔ yɛn nipadua mu no ntumi nyɛ adwuma yiye no. Saa complement system yi yɛ protein ahorow a wɔaboaboa ano wɔ yɛn mogya mu. Eyi yɛ yɛn nipadua mu ahoɔden a ɛko tia nyarewa no fã. Eyi boa ma wɔko tia nneɛma te sɛ mmoawa ne mmoawa a efi abɔnten ba.

Nea ɛto obi a ɔwɔ C3G ne sɛ saa complement system yi yɛ adwuma dodo, anaasɛ entumi nni so. Afei complement 3 (C3) protein no sɛe. Saa C3 protein asinasin a asɛe yi kɔhyɛ glomeruli mu. Afei ɛbɛyɛ den ma glomeruli no sɛ wɔbɛyi mogya na wɔayɛ nsuo. Sɛ wɔansa a, saa ɔsɛe yi betumi akɔ so na ama asaabo no ayɛ adwuma yiye.

Mpɛn pii no, ɛyɛ den sɛ wobɛkyerɛ nea enti pɔtee a saa akamfo nhyehyɛe'' yi kɔ haywire. Nanso ɛtɔ mmere bi a:

• C3G betumi aba esiane nsakrae a ɛba yɛn awosu mu nkwaadɔm mu (nsakrae a ɛba awosu mu nkwaadɔm a ɛfa complement nhyehyɛe no ho, te sɛ C3 ne CFH).
• Mogya mu yareɛ bi a wɔfrɛ no Monoclonal gammopathy nso betumi asɛe asaabo no wɔ ɔkwan a ɛnteɛ so, asɛe complement nhyehyɛe no dwumadi, na ama C3G ayɛ. Saa monoclonal gammopathy yi yɛ tebea a ɛka plasma nkwammoaa a ɛwɔ yɛn nnompe mu ntini mu.
• Aduruyɛ mu nhwehwɛmufo susuw sɛ biribi a wɔfrɛ no autoantibodies'' nso betumi adi dwuma bi. Autoantibodies'' yɛ protein a yɛn ankasa nipadua mu ahoɔden a ɛko tia nyarewa na ɛyɛ a edi mfomso tow hyɛ yɛn ankasa ntini a ɛte apɔw so. Nanso, nhwehwɛmu a ɛdɔɔso nni hɔ a ɛbɛma wɔaka no yiye sɛ saa autoantibodies'' yi na ɛde C3G ba.

Henanom na C3G yare yi ka no kɛse?

Saa C3G yare yi betumi aba obiara mu. Ɛka mmarima ne mmea nyinaa pɛpɛɛpɛ. Sɛ wɔkyekyem pɛpɛɛpɛ a, mfe a obi adi ansa na wɔahu yare no bɛyɛ mfe 23. Nanso, DDD su a yɛadi kan aka ho asɛm no betumi ada adi wɔ mfe a esua mu sen C3GN su no.

Dɛn ne nsɛnnennen a ebetumi afi C3G mu aba?

Ɔhaw titiriw ne nea ɛyɛ hu sen biara ne sɛ nnipa a wɔwɔ C3G no mu bɛyɛ 50%, anaasɛ baanu biara mu biako, benya asaabo a entumi nyɛ adwuma yiye wɔ mfe 10 akyi bere a wɔahu sɛ wɔanya bi no. Eyi ne nea ɛsɛ sɛ yɛn nyinaa suro paa.

Ɔkwan bɛn so na wohu C3G yare no?

Oduruyɛfo bɛyɛ nhwehwɛmu pii de ahwɛ sɛnea w’asaabo te ne sɛnea ɛreyɛ adwuma yiye. Ebia eyinom bi ne:

• Mogya mu nhwehwɛmu: Wɔfa mogya ketewaa bi fi ntini bi a ɛwɔ wo nsa mu na lab no hwɛ sɛ wo complement proteins no yɛ nea ɛfata anaasɛ nneɛma a wɔsɛe no boro so (e.g. creatinine) wɔ wo mogya mu anaa.
• Estimated Glomerular Filtration Rate (eGFR): Eyi yɛ sɔhwɛ a ɛkyerɛ sɛnea w’asaabo reyɛ wo mogya yiye, a egyina serum creatinine anaa cystatin C dodow a ɛwɔ mogya mu so. Eyi betumi ama woanya sɛnea w’asaabo reyɛ adwuma yiye no ho adwene pa.
• Nsuo mu nhwehwɛmu: Ɛho behia sɛ woto nsuo gu kuruwa soronko bi mu na wode nhwɛsoɔ no ma wo duruyɛfoɔ. Lab no bɛhwɛ sɛ protein anaa mogya wɔ nsu no mu, a wobetumi ahu wɔ microscope nkutoo mu.
• Asaabo mu nhwehwɛmu: Eyi ne neaƆkwan a eye sen biara a wɔbɛfa so ahu C3G yare no yiye ne sɛ wɔbɛfa nipadua no mu nhwehwɛmu so. Wɔ eyi mu no, oduruyɛfo de ade a ɛyɛ fɛ anaasɛ nea wɔatwa ketewaa bi yi ntini ketewaa bi fi w’asaabo mu na ɔsɔ hwɛ wɔ aduruyɛdan mu. Sɛ saa nhwɛsode yi kura C3 protein a asɛe no asinasin wɔ wo glomeruli mu a, ebetumi ayɛ C3G yare. Saa biopsy yi nso betumi akyerɛ wo C3G ko a wowɔ (DDD anaa C3GN), a egyina sɛ ebia wowɔ saa solid deposits no anaasɛ wonni bi so.

So wobetumi asa C3G yare koraa?

Nea ɛyɛ awerɛhow no, mprempren wonni aduru biara a wɔde sa C3G yare no. Nanso mma ɛnhaw wo, nnuruyɛfo ne nhwehwɛmufo kɔ so sua yare yi ho ade na wɔreyɛ adwuma de ahwehwɛ ayaresa foforo. Yɛwɔ anidaso sɛ daakye ayaresa a eye sen saa bɛba.

Dɛn ne ayaresa ahorow a wɔde sa C3G?

Mprempren ayaresa pɔtee biara nni hɔ a U.S. Aduan ne Nnuru Dwumadibea (FDA) apene so ama C3G. Nanso, nnuruyɛfo betumi akyerɛw ayaresa ahorow ne ɔhwɛ a ɛboa ma C3G no akɔ so brɛoo, asiw yare no ho sɛnkyerɛnne ahorow so, na asaabo a ɛsɛe no so atew. Eyinom bi ne:

• Mogya mmoroso nnuru: Nnuru te sɛ angiotensin-converting enzyme (ACE) inhibitors ne angiotensin II receptor blockers (ARBs) brɛ nhyɛso a ɛwɔ glomeruli no mu ase. Eyi ma protein dodow a ɛwɔ nsu mu no so tew na ɛtew nhyɛso a ɛwɔ asaabo no so no so.
• Nnuru a ɛtew srade so: Ebia wo duruyɛfo bɛkyerɛw nnuru te sɛ statin a ɛbɛma srade a ɛwɔ wo mu no so atew na ama w’asaabo a ɛsɛe wo no so atew.
• Nsakrae a ɛba aduan mu: Adwene mu aduan ho ɔbenfo bɛboa wo ma woayɛ aduan ho nhyehyɛe a ɛfata wo na aboa wo ma woakura mu duru a ɛfata. Sɛ wotew nkyene (sodium), srade a ɛdɔɔso, ne srade a ɛwɔ wo mu so a, ebetumi aboa ma asaabo a ɛsɛe no so atew.
• Corticosteroids: Wɔsan frɛ no "steroids," eyinom yɛ nnuru a ekum ɔyare mmoawa a ɛyɛ adwuma denam nipadua no mu tumi a ɛko tia nyarewa no a esiw ano sɛ ɛbɛtow ahyɛ wo glomeruli so no so.
• Eculizumab: Eyi yɛ aduru a ɛko tia nyarewa a wɔfrɛ no monoclonal antibody a wɔde sa mogya mu nyarewa a ɛntaa nsi. Ebia ɛbɛboa ma asaabo no dwumadi atu mpɔn denam protein a ɛwɔ nsu mu a ɛbɛtew so ne glomerular filtration rate a ɛbɛma akɔ soro wɔ C3G ayarefo binom mu no so.
• Dialysis: Wɔkyerɛ sɛ nnipa a wɔwɔ C3G no mu bɛyɛ 50% na wɔn asaabo ntumi nyɛ adwuma yiye. Dialysis yɛ bere a asaabo no di huammɔ, na wɔde afiri mmoa na ɛyɛ adwuma a ɛsɛ sɛ asaabo no yɛ (ɛhohoro mogya no ho, yi nneɛma fĩ, ne nsu a ɛboro so) no.
• Asaabo a wɔde si ananmu: Eyi yɛ aduru foforo a wɔde sa asaabo a entumi nyɛ adwuma yiye. Oduruyɛfo a ɔyɛ oprehyɛn yi w’asaabo a adi nkogu no na ɔde asaabo a ɛte apɔw a efi obi a ɔde mae hɔ si ananmu.

Sɛ wowɔ DDD anaa C3GN na wowɔ monoclonal gammopathy nso a, wo duruyɛfoɔ bɛkamfo akyerɛ sɛ wɔnyɛ nhwehwɛmu foforɔ mfa nhwɛ sɛ tebea foforɔ a ɛhyɛ aseɛ anaa.

Dɛn ne C3G yare no ho anidaso?

Ayarefo a wɔwɔ C3G no mu bɛyɛ fã nya asaabo a entumi nyɛ adwuma yiye wɔ mfe 10 akyi bere a wɔahu sɛ wɔwɔ bi no. Mprempren wonni aduru biara a wɔde sa asaabo a entumi nyɛ adwuma yiye. Nanso, sɛ wuhu yare no pɛpɛɛpɛ na wosa wo yare ntɛm a, wubetumi atra ase akyɛ na woanya apɔwmuden a nsakrae akɛse biara nni mu. Enti, ɛho hia sɛ wo bo dwo na wudi wo duruyɛfo afotu akyi.

So wobetumi asiw C3G yare ano?

Nea ɛyɛ awerɛhow no, mprempren ɔkwan biara nni hɔ a yɛbɛfa so asiw C3G yare ano, efisɛ mpɛn pii no nneɛma a yentumi nyɛ ho hwee na ɛde ba.

Mɛyɛ dɛn ahwɛ me ho?

Sɛ wowɔ C3G a, wo duruyɛfo bɛyɛ ayaresa nhyehyɛe a eye sen biara ama wo. Ebia nea ɛka eyi ho ne nnuru, ɔhwɛ a wɔde boa, ne asetra mu nsakrae. Ebia nea edidi so yi bɛka w’ayaresa nhyehyɛe no ho, na ɛho hia sɛ wudi anammɔn yi akyi yiye:

• Fa w’aduru no sɛnea wo duruyɛfo akyerɛ wo no pɛpɛɛpɛ. Mma da koro mpo nmpa wo ti so.
• W’aduan a wobɛsakra, titiriw nkyene, ngo, ne asikre a wobɛtew so, na woadi nnuaba ne nhabannuru pii.
• Kwati sɛ wode nnuru a ɛnyɛ steroid a ɛko tia ɔyare mmoawa (NSAIDs) (sɛ nhwɛso no, nnuru a wɔde kum ɛyaw te sɛ ibuprofen ne diclofenac) bedi dwuma a wunni aduruyɛfo afotu, efisɛ ebetumi asɛe asaabo no na ama asaabo no adi huammɔ ntɛmntɛm.
• W’adwene mu akwahosan a wobɛhwɛ so. Yare a ɛntaa nsi a ɛte saa a wubenya no betumi ama woate nka sɛ woayɛ ankonam na ehu aka wo. Afei nso, nim a wunim sɛ C3G betumi ama asaabo ayɛ adwuma yiye no ma asiane a ɛwɔ hɔ sɛ wubenya adwenemhaw no yɛ kɛse. Wo ne wo duruyɛfo nka w’adwene mu akwahosan ho asɛm na sɛ ɛho hia a, hwehwɛ afotu ne ayaresa. Bisa wo duruyɛfo no akuw a wɔboa nnipa a wɔwɔ C3G ho asɛm. Sɛ wohyia nnipa afoforo a wɔwɔ C3G a, ebetumi aboa ma ankonamyɛ so atew na woakyɛ osuahu ahorow.

Bere bɛn na ɛsɛ sɛ mekɔ oduruyɛfo nkyɛn?

Sɛ wowɔ C3G ho sɛnkyerɛnne ahorow, titiriw nsakrae a ɛba wo nsu mu (mogya, ahurututu, kakraa bi), wo hohoro, anaasɛ wobrɛ kɛse a, kɔ oduruyɛfo nkyɛn ntɛm ara. Eyinom betumi ayɛ nsɛnkyerɛnne a ɛkyerɛ sɛ w’asaabo ntumi nyɛ adwuma yiye.

Sɛ wohu sɛ wowɔ C3G a, wo duruyɛfo de wo bɛkɔ asaabo mu yare ho ɔbenfo bi nkyɛn (asaabo ho ɔyaresafo). Ɔno na ɔbɛma wo ayaresa nhyehyɛe a eye sen biara.

Nsɛm bɛn na ɛsɛ sɛ mibisa me duruyɛfo?

Sɛ wokɔ oduruyɛfo nkyɛn a, ntwentwɛn wo nan ase sɛ wubebisa nsɛm te sɛ eyinom:

• Mɛyɛ dɛn ahu no yiye sɛ mewɔ C3G?
• Sɛ minni C3G a, yare foforo bɛn na metumi anya?
• C3G bɛn na mewɔ (DDD anaa C3GN)?
• Ayaresa bɛn na wokamfo kyerɛ me? So nsunsuanso bɔne bi wɔ hɔ?
• Nsakrae bɛn na ɛsɛ sɛ meyɛ wɔ m’aduan mu?
• Wususuw sɛ m’asaabo bedi huammɔ? Dɛn na metumi ayɛ na amma ɛno ansi?
• So metumi akɔ nephrologist nkyɛn?

Awiei koraa no, ɛsɛ sɛ meka sɛ...

C3G yɛ yare a ɛntaa nsi na ɛyɛ den a ɛka nneɛma nketenkete a wɔde yiyi asaabo no mu no. Sɛ wowɔ C3G a, ebia wobɛte nsakrae wɔ wo nsu mu, wo ho ahonhon, wo nkwaa mu ayɛ wo yaw, wo ho ayɛ wo den wɔ w’ani so, na woabrɛ.

Ebia wubenya nkate ahorow bere a wugye saa yare yi tom na woyɛ nsakrae wɔ sɛnea C3G bɛka w’asetra mu no. Mma ɛnhaw wo, ɛnyɛ wo nko ara. Ma wo ho bere ne baabi sua C3G ne w’ayaresa ahorow ho ade. Sɛ wusua nneɛma a wubetumi apaw ne nea wobɛhwɛ kwan a, ebetumi aboa wo ma woadi wo nkate horow ho dwuma wɔ ɔkwan a ɛfata so na ɛsow aba so. Nnuruyɛfo wɔ hɔ sɛ wɔde nsɛm ne akwankyerɛ bɛma. Sɛ wowɔ nsɛmmisa bi, wuhia afotu anaa mmoa a, wo ne wɔn nkasa. Kae sɛ, sɛ wuhu yare yi ntɛm na wosa wo yare yiye a, wubetumi adi saa yare yi so na woabɔ bra pa.