Fa no sɛ ntease biara nnim a, wunya atiridiinini, nkwaa a ɛyɛ yaw, ne akisikuru nketenkete wɔ wo nipadua nyinaa so bere nyinaa. Eyinom yera wɔ nnansa anaa nnansa mu, afei ɛsan ba bio. Sɛ biribi a ɛte sɛɛ to wo anaa wo ba a, ebia nea ɛde ba ne yare bi a wɔntaa nhu a nnipa pii nte ho asɛm mpo. Ɛnnɛ yɛreka yareɛ a ɛte saa baako ho asɛm, CAPS.
Dɛn na CAPS kyerɛ?
Sɛ yɛbɛka no tiawa a, Cryopyrin-Associated Periodic Syndromes, anaa CAPS, yɛ awosu mu nyarewa kuw bi a wɔntaa nhu koraa a ɛka yɛn nipadua mu tumi a ɛko tia nyarewa. Mpɛn pii no, yɛn nipadua mu tumi a ɛko tia nyarewa no bɔ yɛn ho ban fi mmoawa ne nyarewa afoforo ho. Nanso, wɔ CAPS tebea yi mu no, esiane sintɔ bi a ɛwɔ nipadua no mu tumi a ɛko tia nyarewa no ankasa mu nti, efi ase tow hyɛ yɛn ankasa nipadua so. Wɔfrɛ eyi ‘autoinflammatory disease’ wɔ aduruyɛ ho nyansahu mu. Eyi de ɔfe a ɛho nhia ba nipadua no mu, na ɛde sɛnkyerɛnne ahorow te sɛ honam ani akisikuru, atiridiinini, nkwaa mu yaw ne ɔfe, ne aniwa a ɛyɛ kɔkɔɔ ba.
CAPS ahorow atitiriw abiɛsa na ɛwɔ hɔ. Ma yɛnhwɛ nea wɔyɛ.
| Syndrome Edin a Wɔde Di Dwuma | Nkyerɛkyerɛmu ne sɛnea emu yɛ den |
|---|---|
| Abusua mu Atiridiinini a Ɛma Ɔhaw (FCAS) . | Eyi ne nea emu nyɛ den koraa wɔ yare ahorow abiɛsa no mu. Sɛnea edin no kyerɛ no, atiridii a obi de ne ho hyɛ mu no na ɛkanyan yare no ho sɛnkyerɛnne. Nsɛnkyerɛnne titiriw ne atiridiinini, nkwaa mu yaw, ne honam ani akuru. Mpɛn pii no, ɛnsɛe nipadua no bere tenten. |
| Muckle-Wells Syndrome (MWS) a Ɛyɛ Fɛ a Ɛyɛ Fɛ (MWS) . | Eyi yɛ aniberesɛm kakra sen FCAS. Ɛnyɛ atiridii nko na ebetumi akanyan sɛnkyerɛnne ahorow no, na mmom adwennwen ne apɔw-mu-teɛteɛ nso . Nsɛnkyerɛnne no betumi ayɛ kɛse, na nnipa binom nso betumi ate asɛm a ɛyɛ den . |
| Nkokoaa Foforo a Efi Ase Multisystem Inflammatory Disease (NOMID) . | Eyi ne nyarewa kuw yi mu nea emu yɛ den na ɛho yɛ na sen biara .Korɔ. Eyi fi ase bere a wɔawo abofra no ara pɛ. Ɛde ɔfe ba nipadua no akwaa pii mu. Wɔsan frɛ no CINCA. Sɛ wɔansa no yiye a, ebetumi asɛe amemene, aniwa, ne akwaa afoforo kɛse . |
Dɛn na ɛde CAPS ba?
Nea enti titiriw a ɛte saa ne awosu mu nkwaadɔm bi a ɛsakra wɔ yɛn nipadua mu. Sɛ yɛbɛka no pɛpɛɛpɛ a, saa yare yi fi nkwaadɔm mu nsakrae a ɛba awosu mu abɔde a wɔfrɛ no `NLRP3` mu .
Susuw saa `NLRP3` gene yi ho sɛ blueprint a ɛkyerɛ yɛn nipadua sɛ yɛnyɛ protein bi a wɔfrɛ no `cryopyrin`. Mpɛn pii no, saa `cryopyrin` protein yi boa yɛn nipadua ma ɛyɛ ade bi a wɔfrɛ no `interleukin-1 (IL-1)`, a ɛboa yɛn nipadua ma ɛko tia nyarewa.
Nanso esiane sɛ wɔayɛ `NLRP3` gene a ɛwɔ obi a ɔwɔ CAPS mu no mu nsakrae nti, `cryopyrin` protein no nso ntumi nyɛ adwuma yiye. Ɛyɛ sɛnkyerɛnne bere nyinaa sɛ nipadua no nyɛ `IL-1` pii dodo. Saa `IL-1` a wɔyɛ boro so yi ne nea ɛde ɔfe a ɛho nhia ba nipadua no mu na ɛde CAPS ho sɛnkyerɛnne ahorow ba.
Sɛ abofra no nya awosu mu nkwaadɔm a wɔayɛ no foforo no fi ɔwofo biako pɛ hɔ, ɛna no anaa agya no hɔ a, saa yare yi betumi aba. Ɛtɔ mmere bi a, awosu mu abɔde no tumi sesa ne ho wɔ awotwaa no mu, a awofo no nka ho.
Dɛn ne sɛnkyerɛnne ahorow no?
CAPS ho sɛnkyerɛnne titiriw ne nea ɛtaa ba ne akisikuru a ɛda adi sɛ akisikuru nketenkete a ɛnyɛ ɛyaw wɔ nipadua no nyinaa so . Mpɛn pii no, eyi da adi wɔ awo mu. Nnipa binom nya saa akuru yi bere nyinaa, nanso emu yɛ den kɛse bere a ogya rehyew no. Nsɛnkyerɛnne afoforo gu ahorow gyina CAPS ko a wowɔ so.
Nsɛnkyerɛnne a wobetumi ahu wɔ FCAS mu
Saa nsɛnkyerɛnne yi taa di nnafua 1-2.
- Huraeɛ
- Aniwa a ɛho ahonhon
- Awɔw a ɛyɛ nwini
- Nkwaa mu yaw
- Abofono
- Tipaeɛ
Nsɛnkyerɛnne a wohu wɔ MWS mu
Saa sɛnkyerɛnne ahorow yi ba na ɛkɔ. Wobetumi atra hɔ nnafua 1-3 wɔ bere koro mu.
- Wora a ɛyɛ mmerɛw
- Atiridii ne awɔw
- Aniwa a ɛyɛ kɔkɔɔ
- Nkwaa mu yaw
- Tiyɛ a emu yɛ den
- Aso a ɛyɛ den (mmofra a wɔwɔ MWS betumi ahwere wɔn aso nyinaa anaa ne fã bi bere a wodu mpanyin afe so no) .
- Yafunu mu yaw
Nsɛnkyerɛnne a wohu wɔ NOMID mu
Eyinom ne sɛnkyerɛnne ahorow a emu yɛ den sen biara.
- Tiyɛ a emu yɛ den
- Aniwa a ɛrehuruhuruw
- Nkwaa mu yaw ne ɛho ahonhon
- Ɔfe a wɔfe
- Anisoadehu a wɔhwere
- Aso a ɛyɛ mmerɛw
Nea ɛho hia: Sɛ wɔansa NOMID yiye a, protein bi a wɔfrɛ no amyloid betumi akɔhyɛ akwaa a ɛho hia te sɛ asaabo ne nkwaa mu, na bere kɔ so no, asɛe ade kɛse.
Ɔkwan bɛn so na wobetumi ahu yare no?
Sɛ wokɔ wo duruyɛfo nkyɛn a ɔwɔ saa sɛnkyerɛnne ahorow yi a, obedi kan abisa wo w’ayaresa ho abakɔsɛm ne w’abusua de ho asɛm. Afei, wɔbɛhwehwɛ wo mu na sɛ ɛho hia a, wobetumi de wo akɔ sɔhwɛ ahorow bi te sɛ:
- Mogya ne nsu mu nhwehwɛmu: Saa nhwehwɛmu ahorow yi hwehwɛ mogya mu nkwammoaa fitaa ne C-reactive protein a ɛkyerɛ sɛ ɔfe no dodow a ɛkɔ soro, ne protein a ɛboro so wɔ nsu mu.
- Skin biopsy: Wɔfa wo honam ani fã ketewaa bi na wɔde afiri a wɔde hwɛ nneɛma nketenkete hwehwɛ mu.
- Awosu mu nhwehwɛmu: Saa nhwehwɛmu yi betumi asi so dua sɛ ebia wowɔ nkwaadɔm mu nsakrae bi wɔ wo NLRP3 awosu mu nkwaadɔm mu anaa. Nanso, ɛtɔ mmere bi a, ebia nnipa a wɔwɔ CAPS no benya saa awosu mu abɔde yi sɛnea ɛte biara.
- Aniwa ne aso mu nhwehwɛmu: Wɔyɛ nhwehwɛmu yi de hwɛ sɛ ebia w’aniwa anaa w’aso aka wo anaa.
- Lumbar puncture: Woyi akyi berɛmo mu nsu kakraa bi fi akyi berɛmo no mu na wɔhwehwɛ mu hwɛ sɛ ebia ɛho ayɛ hyew anaa.
- MRI scan: Eyi betumi atwa nneɛma a ɛwɔ amemene ne aso mu no ho mfonini a ɛkɔ akyiri ahwɛ sɛ nsakrae bi aba anaa.
Nsɛmmisa a ɛsɛ sɛ wobisa oduruyɛfo no
Ɛyɛ papa sɛ wobɛkyerɛw nsɛmmisa bi a wowɔ wɔ krataa so bere a worekɔ oduruyɛfo nkyɛn no. Saa kwan no so no, wo werɛ remfi biribiara. Wubetumi abisa nsɛm te sɛ eyinom:
- CAPS bɛn na mewɔ (anaa me ba)?
- Nsɛnkyerɛnne bɛn na ebia me yare no de bɛba?
- Ayaresa ahorow bɛn na wokamfo kyerɛ? Wɔbɛyɛ dɛn aboa me?
- So ayaresa no mu nsunsuanso bɔne bi wɔ hɔ?
- Dɛn na metumi ayɛ wɔ fie na ama yare no ho sɛnkyerɛnne so atew?
- Sɔhwɛ afoforo bɛn na anka ɛsɛ sɛ meyɛ? Bere bɛn na ɛsɛ sɛ mema wɔyɛ wɔn?
- So ɛsɛ sɛ mekɔ awosu ho ɔfotufo bi nkyɛn?
Dɛn ne ayaresa ahorow no?
Ɛho hia paa sɛ wofi ase sa yare yi ntɛm ara, efisɛ sɛ aniwa, aso, nnompe, anaa amemene no sɛe pɛ a, ɛyɛ den yiye sɛ wɔbɛsan de ayɛ nea ɛfata.
Ayaresa titiriw ne nnuru a esiw ade a wɔfrɛ no IL-1 a nipadua no yɛ dodo no kwan. Eyinom di ɔfe so.
- Anakinra (Kineret) .
- Canakinumab (Ilaris) a wɔde di dwuma wɔ ɔkwan a ɛyɛ nwonwa so.
- Rilonacept (Ahyɛnsode Ho Ɔbenfo) .
Eyinom akyi no, wo duruyɛfo betumi akamfo ayaresa afoforo nso akyerɛ wo, te sɛ:
- Nhama a wɔde kyekyere nkwaa ho a wɔhyɛ ma nkwaa mu yaw ne nea ɛho ahonhon
- Nipadua mu ayaresa
- Nnuru te sɛ NSAID, steroid, anaa methotrexate a wɔde bɛtew atiridii ne ɔfe so
- Mmoa a wɔde boa aso ma wɔn a wɔnte asɛm yiye
- Oprehyɛn sɛ nkwaa no asɛe a
Wobɛyɛ dɛn atumi ahwɛ wo ho?
Nea ɛka ayaresa ho no, nsakrae nketenkete a wobɛyɛ wɔ w’asetra kwan mu no betumi aboa kɛse ma woadi yare no ho sɛnkyerɛnne ahorow so.
- Kwati awɔw: Sɛ wowɔ tebea bi te sɛ FCAS a, awɔw betumi ama wo yare no ho sɛnkyerɛnne no asɛe kɛse. Enti bɔ mmɔden sɛ wobɛtwe wo ho afi nneɛma te sɛ AC dan mu a wobɛtra ne mmeae a awɔw wom a wobɛtra ho.
- Tew adwennwen so: Nneɛma te sɛ adwennwen, apɔw-mu-teɛteɛ a wɔde home kɔ akyiri, ne yoga betumi aboa kɛse wɔ eyi mu.
- Asetra a ahoɔden wom:
- Da yiye.
- Di aduan a ahoɔden wom.
- Kwati sigaretnom, nsa, ne nnuan a wɔayɛ no foforo.
Nkrasɛm a Wɔde Kɔ Fie
- CAPS yɛ awosu mu tebea a ɛntaa nsi a efi nipadua no mu tumi a ɛko tia nyarewa no mu sintɔ bi na ɛde ba.
- Ne sɛnkyerɛnne atitiriw ne atiridiinini a ɛsan ba bio, honam ani akuru a ɛnyɛ yaw, ne nkwaa mu yaw.
- Eyi fi awosu mu nkwaadɔm a wɔfrɛ no
NLRP3mu nsakrae bi. - Ɛho hia sɛ wohu yare no na wofi ase sa yare ntɛm ara sɛnea ɛbɛyɛ yiye na wɔasiw aniwa, aso, ne akwaa afoforo a ɛbɛsɛe koraa no ano.
- Sɛ wo anaa wo ba no wɔ saa sɛnkyerɛnne yi bi a, mmu w’ani ngu so da na kɔ wo duruyɛfo nkyɛn ntɛm ara kɔhwehwɛ afotu.
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