So wo ba taa nya atiridii a ntease biara nnim? Momma yensua saa SAID (Systemic Autoinflammatory Diseases) yi ho ade!

So ɛtɔ mmere bi a wo ba kumaa no nya atiridii kɛkɛ a atiridii anaa yare foforo biara nni ne ho? So atiridii no ba, brɛ ase wɔ nna kakraa bi mu, na afei ɛsan ba nna kakraa bi akyi? Awofo binom hyia osuahu ahorow a ɛtete saa. Enti ɛnnɛ yɛrebɛka ade a ɛtaa de atiridiinini ba a ɛyɛ den sɛ wobehu saa ho asɛm. Wɔfrɛ eyi SAIDs (Systemic Autoinflammatory Diseases) . Bere bi a atwam no, na wɔfrɛ eyi `(Periodic Fever Syndromes)` anaa `(Recurrent Fever Syndromes)`.

Dɛn ne saa SAID ahorow yi? Momma yɛnte ase kɛkɛ!

Sɛ yɛbɛka no tiawa a, SAID yɛ nyarewa kuw bi a yɛn nipadua mu nipadua mu ahoɔden a ɛko tia nyarewa a wɔde awo yɛn no ntumi nyɛ adwuma yiye anaasɛ ɛyɛ nhyehyɛe ho haw bi na ɛde ba . Wɔ eyi mu no, sɛ yɛannya ɔyare mmoawa biara a efi akyi (te sɛ mmoawa anaa mmoawa bi) a, yɛn nipadua no de ɔfe ba kɛkɛ. Ɛte sɛ nea yɛn nipadua no ahobammɔ nhyehyɛe no rekɔ overdrive mu.

Susuw ho hwɛ, yɛwɔ asraafo kuw bi wɔ yɛn nipadua mu (ɛno ne nipadua no mu tumi a ɛko tia nyarewa no). Wɔn adwuma ne sɛ wɔbɛko atia mmoawa a wɔde nyarewa ba bere a wɔaba mu Nanso wɔ obi a ɔwɔ SAID nipadua mu no, ɛtɔ mmere bi a asraafo yi ho yeraw wɔn kɛkɛ na wɔde ɔhaw ahorow ba nipadua no mu.

Afei ebia woresusuw ho sɛ ebia eyi yɛ biribi te sɛ `(autoimmune diseases)` (kyerɛ sɛ, autoimmune nyarewa). Sɛ nhwɛso no, `(rheumatoid arthritis)` anaa `(lupus)` te sɛ nea ɛte saa ara. Dabi, saa abien yi yɛ soronko kakra. Wɔ nyarewa a nipadua no tumi ko tia nyarewa mu no, nea ɛba ne sɛ yɛn nipadua no nipadua mu ahoɔden a ɛko tia nyarewa a wɔanya anaasɛ ɛsakra no di mfomso tow hyɛ n’ankasa nkwammoaa a ɛte apɔw so. Nanso wɔ SAID ahorow mu no, ɔhaw no fi awosu mu nipadua mu ahoɔden a ɛko tia nyarewa no.

SAIDs ho yɛ na koraa sen nyarewa a nipadua no tumi ko tia nyarewa. Wɔn mu dodow no ara yɛ awosu , a ɛkyerɛ sɛ awosu mu nsakrae anaa ɔkwan foforo bi na ɛde ba.

SAIDs taa fi ase wɔ nkokoaa anaa mmofraase . Nanso ɛnyɛ bere nyinaa na ɛte saa. Ebia wo ba no benya atiridiinini ne sɛnkyerɛnne afoforo bere bi anaasɛ ɔbɛtow ahyɛ no so. Ebia wo ba no nni sɛnkyerɛnne biara koraa wɔ ntua ntam. Aduru biara nni hɔ a wɔde sa SAIDs. Nanso, ayaresa ahorow bi wɔ hɔ a ebetumi aboa ma wɔadi yare no ho sɛnkyerɛnne ahorow so wɔ nsɛm dodow no ara mu.

Dɛn ne SAID ahorow titiriw?

Mprempren nhwehwɛmufo ahu SAID ahorow bɛyɛ 60, na wɔrehu pii. Momma yɛnhwɛ ahorow a wɔtaa yɛ no bi.

• Abusua mu Mediterranea Atiridii (FMF): Eyi ne nea abu so sen biara wɔ atiridiinini a wonya fi awosu mu a ɛsan ba bio no mu. Ebetumi ama abofra no yafunu, ne koko, ne ne nkwaa ayɛ hyew a ɛyɛ yaw .
• Atiridii a ɛba bere ne bere mu, aphthous-stomatitis, pharyngitis, adenitis (PFAPA): Eyi taa fi ase wɔ mmofra nkumaa so ansa na wɔadi mfe 4. Saa tebea yi betumi ano abrɛ ase ara kwa bere a wɔadi mfe 10 akyi.
• Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): Eyi betumi aba wɔ mmofraase, mmerantebere ne mmabaabere mu, ne ɛtɔ mmere bi mpo a mpanyin afe so.
• Mevalonate kinase deficiency (MKD): Kan no na wɔfrɛ no hyper-IgD syndrome, na ɛtaa fi ase wɔ nkokoaa a wonnii afe 1 so.
• Nyarewa a ɛma obi ho hyehye no a ɛne NLRP3 wɔ abusuabɔ: Kan no na wɔfrɛ eyi sɛ cryopyrin-associated periodic syndromes (CAPS). Subtypes afoforo abiɛsa wɔ eyi mu.
• Still’s yare a efi ase wɔ mpanyimfo mu (AOSD): Eyi yɛ nhyehyɛe mu mmofra ntini mu yare (JIA) a efi ase wɔ mpanyimfo mu.
• NOD-2-associated granulomatous disease (Blau syndrome): Eyi taa fi ase ansa na wadi mfe 4. Ɛka abofra no honam ani, n’ani, ne ne nkwaa titiriw .

Dɛn ne SAIDs ho sɛnkyerɛnne? Yɛyɛ dɛn hu wɔn?

SAIDs ho sɛnkyerɛnne taa fi ase wɔ mmofraase. Nsɛnkyerɛnne a ɛtaa ba na ɛda adi kɛse ne atiridii a ɛba bere ne bere mu anaasɛ ɛba bere ne bere mu . Mpɛn pii no, nkurɔfo kɔ so yɛ nea wonni sɛnkyerɛnne biara wɔ ntua ntam. Nanso, ebia sɛnkyerɛnne afoforo bi wɔ hɔ a ɛfa SAID ahorow biara ho.

Momma yɛnhwɛ SAID ahorow pii a yɛaka ho asɛm wɔ atifi hɔ no ho sɛnkyerɛnne pɔtee:

• FMF: Eyi betumi ama abofra no yafunu, ne koko, ne ne nkwaa ate ɛyaw kɛse, ne ɔfe . Ɛtɔ da bi a, akisikuru betumi aba anan ase anaa ananse ase. Fa no sɛ, abofra bi nya yafunu ne atiridiinini mpofirim. Sɛ awofo no haw na wɔde wɔn kɔ ayaresabea a, ebia wobesusuw sɛ ɛyɛ appendicitis. Nanso afei ɛbrɛ ase na ɛsan ba nna kakraa bi akyi.
• PFAPA: Eyi betumi de sɛnkyerɛnne ahorow te sɛ menewa a ɛyɛ yaw, ano a ɛyɛ kurukuruwa, ne ntini a ɛho ahonhon wɔ kɔn mu aba .
• MFIA: Eyi betumi ama atiridiinini, ntini mu yaw wɔ koko ne abasa mu , ne akisikuru kɔkɔɔ a ɛyɛ yaw a efi ase wɔ nsa ne nan so na ɛtrɛw kɔ nipadua no nyinaa mu.
• MKD: Wɔ eyi mu nsoEbia wo ho nyɛ wo den esiane atiridiinini, tiyɛ, yafunu a ɛyɛ wo yaw, aduan ho akɔnnɔ a ɛyera, ne sɛnkyerɛnne ahorow a ɛte sɛ atiridiinini nti.
• NLRP3 nyarewa: Eyinom betumi de sɛnkyerɛnne ahorow te sɛ honam ani akisikuru, tiyɛ, adwenemhaw, nkwaa mu yaw, ne aniwa kɔkɔɔ (aniwa a ɛyɛ mmerɛw) aba .
• AOSD: Eyi yɛ tebea a ɛtaa ma honam ani akisikuru, nkwaa mu yaw, ne ntini mu yaw . Nnipa binom nso betumi anya menewam yaw, yafunu mu yaw, ɔbrɛ a ɛtra so, ne nipadua mu yaw.
• Blau syndrome: Eyi betumi ama wo ba no nsa, ne nan, anaa ne nipadua honam ani akuru . Ebetumi nso ama nkwaa ayɛ no yaw na aniwa ayɛ no yaw .

Dɛn nti na saa SAID ahorow yi ba? Dɛn na ɛde ba?

SAID dodow no ara yɛ awosu mu nyarewa . Ɛne sɛ, nsakrae (variant) bi a ɛwɔ awosu mu abɔde pɔtee bi mu na ɛde ɔyare biara ba.

Momma yɛnhwɛ awosu mu nkwaadɔm a ɛwɔ SAID ahorow atitiriw pii so nkɛntɛnso:

• FMF: Eyi fi awosu mu abɔde bi a wɔfrɛ no `(MEFV gene)`. Saa awosu mu abɔde yi kyerɛ sɛnea wɔyɛ protein a wɔfrɛ no `(pyrin)`.
• PFAPA: Wɔnnya nhuu nea enti pɔtee a ɛte saa .
• TRAPS: Eyi fi awosu mu abɔde bi a wɔfrɛ no `(TNFRSF1A gene)`. Eyi ne nea ɛma akwankyerɛ a wɔde bɛyɛ protein a wɔfrɛ no `(tumor necrosis factor receptor - TNFR)`.
• MKD: Nea enti a ɛte saa ne awosu mu abɔde bi a wɔfrɛ no `(MVK gene)`. Eyi kyerɛ sɛnea wɔyɛ protein bi a wɔfrɛ no `(mevalonic kinase)`.
• NLRP3 nyarewa: Eyi fi awosu mu abɔde bi a wɔfrɛ no `(NLRP3 awosu mu abɔde)`. Eyi ma akwankyerɛ sɛ wɔbɛyɛ protein bi a wɔfrɛ no `(cryopyrin)`.
• AOSD: Wɔnnya nhuu nea enti pɔtee a ɛte saa .
• Blau syndrome: Eyi fi awosu mu abɔde bi a wɔfrɛ no `(NOD2 gene)`. Eyi kyerɛ sɛnea wɔyɛ protein bi a wɔfrɛ no `(NOD2)`.

So saa SAID ahorow yi betumi de nsɛnnennen afoforo aba?

Yiw, sɛ wɔansa eyi yiye a, ebetumi de ɔhaw ahorow aba . Sɛ ɔfe kɔ so wɔ nipadua no mu a, ebetumi ama tebea bi a wɔfrɛ no ``amyloidosis.`` Ɛne sɛ, protein bi kɔ yɛn asaabo mu. Eyi betumi asɛe asaabo no daa . Ɛno nti na ɛho hia sɛ wohu yare no ntɛm na wonya ayaresa.

Ɔkwan bɛn so na oduruyɛfo hu saa SAIDs tebea yi?

Nokwarem no, saa nyarewa a ɛma obi ho hyehye no yi a wobehu no betumi ayɛ den kakra , efisɛ wɔn sɛnkyerɛnne betumi asuasua tebea afoforo a emu yɛ den te sɛ lupus anaa kokoram te sɛ lymphoma. Enti, ɛho hia sɛ wokɔ oduruyɛfo a wanya ntetee titiriw wɔ nyarewa a ɛma obi ho hyehye no nkyɛn, oduruyɛfo a ɔyɛ akisikuru, na ama wahu wo ba no tebea no yiye na wahwɛ so.

Wo ba no ntini ho ɔyaresafo de nneɛma pii bedi dwuma de ahu SAID ahorow. Obebisa wo wo ba no yare no ho sɛnkyerɛnne ne sɛ ebia obi wɔ w’abusua mu a ɔwɔ saa tebea horow yi (biological family history). Ebia oduruyɛfo no besusuw sɛ ebia wo ba no benya atiridiinini a ɛba bere ne bere mu sɛ:

• Sɛ abofra no taa nya atiridii a .
• Sɛ obi wɔ abusua no mu a ɔwɔ abakɔsɛm a ɛkyerɛ sɛ ɔtaa nya atiridii te sɛ eyi a .
• Sɛ abofra no yɛ nnipa kuw bi a wɔtaa nya atiridii bere ne bere mu te sɛ eyi muni a .

Sɔhwɛ ahorow bɛn na wɔreyɛ ama eyi?

Nea ɛbɛyɛ na wo ba no akisikuru ho ɔbenfo betumi akamfo akyerɛ sɛ wɔnyɛ nhwehwɛmu pii. Ebia eyinom bi ne:

• Lab nhwehwɛmu: Wobetumi de sɔhwɛ te sɛ ``C-reactive protein (CRP)`` ne ``complete blood count (CBC)`` adi dwuma de ahwɛ sɛ ɔfe wɔ nipadua no mu anaa. Saa nhwehwɛmu ahorow yi yɛ papa bere a atiridii "ntua" reba na ɛsan kɔ nea ɛte kan bere a "ntua" no aba awiei no.
• Nsu mu nhwehwɛmu: Wobetumi ayɛ nsu mu protein nhwehwɛmu de ahwɛ sɛ protein a ɛboro so wɔ nsu mu anaa. Wɔ MKD mu no, nsu mu nhwehwɛmu a ɛhwɛ organic acid (acid a carbon atom biako wom) betumi akyerɛ sɛ mevalonic acid dodow akɔ soro.
• Awosu mu nhwehwɛmu: Eyi betumi ahwɛ sɛ awosu mu nsakrae ahorow wɔ hɔ anaa. Nanso ebia mmofra binom a wɔwɔ SAID no nni awosu mu nsakrae a wɔahu no, na ebetumi ama nhwehwɛmu no mu aba a enye.

Dɛn ne ayaresa ahorow a wɔde sa SAID ahorow yi?

Ɛsono sɛnea wɔsa SAID ahorow no gyina tebea no su ne sɛnea emu yɛ den so . Asa biara nni hɔ a wɔde sa tebea horow yi. Nanso , mpɛn pii no wobetumi de nnuru adi wo ba no yare no ho sɛnkyerɛnne ahorow so . Sɛ wo ba no nya ntua kakraa bi pɛ afe biara a, ebia wubetumi ama no nnuru a wɔde kum ɛyaw te sɛ nnuru a ɛnyɛ steroid a ɛko tia ɔyare mmoawa (NSAIDs) na aboa ma wɔn yare no ho sɛnkyerɛnne so atew. Nanso, sɛ tebea no mu yɛ den kɛse a, wo duruyɛfo betumi akyerɛ wo ayaresa foforo.

Wɔn mu bi ni:

• FMF: Wobetumi de aduru bi a wɔfrɛ no `(colchicine)` adi dwuma de asiw eyi ano. Eyi ma ɔfe so tew. Sɛ abofra no ntumi nnom colchicine a, wobetumi asɔ aduru bi a wɔfrɛ no `(biologic)` a wɔfrɛ no `(canakinumab)` ahwɛ.
• PFAPA: Wobetumi de steroid (a mpɛn pii no prednisone) ama de atew bere tenten a PFAPA "ntua" no di so. Wɔ mmofra binom fam no, cimetidine, aduru bi a wɔde sa yafunu mu akisikuru nso betumi aboa ma wɔadi yare no ho sɛnkyerɛnne ahorow so.
• MFIA: Aduru bi a wɔfrɛ no ``canakinumab'' taa yɛ adwuma yiye ma eyi. Afei nso, wobetumi de nnuru a ekum ɔyare mmoawa a oduruyɛfo akyerɛw ama no, te sɛ ``glucocorticoids'' agyae yare no ho sɛnkyerɛnne.
• MKD: 1 .Canakinumab nso yɛ aduru a etu mpɔn a wɔde sa MKD. NSAID anaa steroid a wɔde ma bere a "ntua" bi rekɔ so no nso betumi aboa.
• NLRP3 nyarewa: Wɔde nnuru a ɛma nipadua no tumi ko tia nyarewa te sɛ canakinumab, rilonacept, ne anakinra di dwuma yiye ma eyi.
• AOSD: Wɔde nnuru ahorow a ekum ɔyare mmoawa di dwuma ma AOSD. Nhwɛso ahorow bi ne steroid, nnuru a ɛsakra yare a ɛko tia akisikuru (DMARDs), ne nnuru a wɔde yɛ abɔde a nkwa wom.
• Blau syndrome: Ɛdenam wo ba no sɛnkyerɛnne so no, wobetumi asɔ nnuru a ɛma nipadua no tumi ko tia nyarewa, nnuru a esiw tumor necrosis factor (TNF) ano, ne/anaasɛ nnuru a wɔde gu aniwa so ahwɛ.

So saa SAIDs tebea horow yi yɛ nea ɛtra hɔ daa? Anaasɛ wɔbɛkɔ?

Tebea horow bi a ɛma obi ho hyehye no yɛ nkwa nna nyinaa . Ebia afoforo nso bɛtra hɔ mfe kakraa bi na bere a wɔrenyin no akɔ. Tebea horow bi di nkwa nna nyinaa, nanso bere kɔ so no, mpɛn dodow a ntua ba ne sɛnea emu yɛ den no so betumi atew. Wo ba no oduruyɛfo betumi ama wo wo ba no tebea ho nsɛm pɔtee.

Abofra a ɔwɔ yare a ɛma obi ho hyehye no a wɔbɛtete no betumi ayɛ den. Sɛ wowɔ SAID a, wubetumi de w’ankasa osuahu ahorow adi dwuma de aboa wo ba no hwɛ yiye. Wubetumi nso de wo suahu ahorow aboa wo ba no ma wayɛ nsakrae wɔ ne nkwa nna nyinaa tebea yi mu.

Ade a ɛho hia sen biara ne sɛ wobɛhwehwɛ ayaresa afi nnuruyɛfo a wɔn ho akokwaw a wɔwɔ SAID ho nimdeɛ hɔ. Nnuruyɛfo a wɔte saa no betumi aboa ma wɔadi wo ba no yare no ho sɛnkyerɛnne ahorow ho dwuma na wɔaboa no ma wanya mmofraberem a eye sen biara.

Nkrasɛm a Etwa To a Wɔde Kɔ Fie

Enti, mewɔ anidaso sɛ wowɔ adwene bi wɔ SAIDs a yɛkaa ho asɛm nnɛ no ho. Kae sɛ, sɛ wo ba taa nya atiridiinini a wontumi nkyerɛkyerɛ mu a, akyinnye biara nni ho sɛ wo ne oduruyɛfo nkasa ho asɛm . Mma w’ani ngu so kɛkɛ, nsusuw sɛ ɛyɛ ade a ɛfata.

• SAID yɛ nyarewa kuw bi a ɛtaa de atiridiinini ba na ɔhaw bi a ɛwɔ nipadua no mu tumi a ɛko tia nyarewa no mu na ɛde ba.
• Eyinom taa fi awosu mu na efi ase wɔ mmofraase.
• Ɛwom sɛ wonni eyi ano aduru a ɛtra hɔ daa de, nanso ayaresa ahorow bi wɔ hɔ a ebetumi adi yare no ho sɛnkyerɛnne ahorow no so.
• Ɛho hia sɛ wɔhwehwɛ sɛ wobehu yare no yiye na wɔasa no yare fi oduruyɛfo a ɔyɛ ɔbenfo (Rheumatologist) hɔ.
• Sɛ wofi ayaresa ase ntɛm a, wobetumi asiw nsɛnnennen te sɛ amyloidosis ano na abofra no atumi atra ase sɛnea ɛsɛ.

Sɛ wowɔ nsɛmmisa foforo bi fa eyi ho a, ntwentwɛn wo nan ase sɛ wubebisa w’abusua oduruyɛfo anaa mmofra oduruyɛfo.

` SAIDs, Systemic Autoinflammatory Diseases, Atiridii a Ɛtaa Ba, Awosu mu Nyarewa, Atiridii wɔ Mmofra mu, Nkwammoaa a ɛko tia nyarewa, Ntini mu yare ho ɔbenfo