So wo nipadua te nka sɛ wontumi nni so? Momma yensua eyi ho ade (Autoimmune Autonomic Ganglionopathy - AAG)!

So ɛtɔ mmere bi a wo ti mpofirim? Anaasɛ wote nka sɛ biribi a ɛyɛ nwonwa wɔ wo yafunu mu, w’ano ayow, anaasɛ wuntumi nni wo home so? Ɛwom mpo sɛ eyinom betumi ayɛ te sɛ nneɛma a ɛfata de, nanso ɛtɔ mmere bi a asɛm bi a ɛyɛ den kɛse betumi aba wɔ akyi. Ɛno yɛ saa tebea horow a ɛntaa nsi, nanso ɛfata sɛ yehu, a yɛbɛka ho asɛm nnɛ no mu biako.

Dɛn ne eyi ``Autoimmune Autonomic Ganglionopathy - AAG''? Momma yɛnte ase tiawa, ɛnte saa?

Sɛ yɛbɛka no tiawa a, ``Autoimmune Autonomic Ganglionopathy'' anaa ``(AAG)'' yɛ tebea a yɛn ankasa nipadua ahobammɔ nhyehyɛe, kyerɛ sɛ, nipadua mu ahoɔden a ɛko tia nyarewa no, di mfomso tow hyɛ yɛn ankasa autonomic nervous system so. Wo de susuw ho hwɛ, eyi te sɛ nea sɛ asraafo a wɔkyerɛ sɛ wɔbɔ yɛn man ho ban no di mfomso tow hyɛɛ yɛn ankasa mmeae a ɛho hia so a anka ɛbɛba.

Yɛn nipadua no autonomic nervous system no te sɛ yɛn ``automatic system''. Ɛne sɛ, ɛhwɛ nneɛma pii a ɛba ara kwa so, a yennim. Nhwɛsoɔ:

• Wo home a wobɛhome
• Koma a ɛbɔ (koma a ɛbɔ) .
• Mogya mmoroso
• Fifiri a ɛrefifi
• Nnuan a wɔyam

Saa nneɛma yi mu pii nyɛ biribi a yedwen ho. Wɔba ara kwa. Autonomic nervous system no ne nea ɛboa ma saa automatic functions no kɔ so yɛ adwuma yiye. Wɔ AAG mu no, nhyehyɛe a ɛho hia yi asɛe. Eyi yɛ tebea bi a wɔfrɛ no autonomic neuropathy, anaa dysautonomia.

Dɛn nti na yɛn ankasa nipadua yɛ eyi? Dɛn ne nea enti a ɛte saa?

Nokwarem no, nnuruyɛfo mpo da so ara ntumi nka nea ɛde `(AAG)` yi ba ankasa. Nanso wɔahu nokwasɛm ahorow bi.

• Mpɛn pii no, kyerɛ sɛ , wɔ bɛyɛ nnipa baanum biara mu baasa a wonya ``AAG'' mu no, saa tebea yi ba bere a wɔn ho atɔ wɔn afi yare foforo mu, anaasɛ bere a wɔanya ɔyare mmoawa akyi. Ɛte sɛ nea saa yare no de ba.
• Nnipa binom betumi anya tebea bi a wɔfrɛ no paraneoplastic AAG. Eyi ne bere a nnuru bi a ɛko tia nyarewa a yɛn nipadua no mu tumi a ɛko tia nyarewa no yɛ de bua tebea bi te sɛ kokoram kɔ ​​tow hyɛ ntini a ɛma nipadua no yɛ adwuma no so.

Dɛn na eyi yɛ yɛn nipadua? Momma yɛnhwehwɛ mu yiye.

Yɛn ntini a ɛhwɛ ne ho so no wɔ ntini mu nkwammoaa akuwakuw a wɔfrɛ no autonomic ganglia . Eyinom te sɛ keteke akwan. Wɔnam eyinom so de nkrasɛm ahorow mena na wogye. Saa nkrasɛm a yɛde sesa yi ho hia na ama yɛn nipadua no nneyɛe a ɛba ara kwa, kyerɛ sɛ, nneyɛe a yennni so (reflexes) no tumi di so.

Wɔ AAG mu no, yɛn nipadua no mu tumi a ɛko tia nyarewa no tow hyɛ ntini pɔtee bi a ɛwɔ saa ganglia yi mu so. Receptors ne mmeae a wogye nkrasɛm. Enti sɛ saa ntini ahorow yi sɛe a, saa ntini mu nkrasɛm no nkɔ anaasɛ ɛnfa mu yiye. Ne saa nti, nneɛma a ɛba ara kwa, te sɛ mogya mmoroso ne fifiri a wɔhyɛ so no fi ase yɛ basabasa.

Fa no sɛ, sɛ `connection` bi a ɛwɔ wo fie `main switch board` mu no yɛ loose, kanea no rensɔ, fan no renkyinkyin, ne nea ɛkeka ho Saa na ɛte wɔ eyi ho.

Saa tebea yi na wɔfrɛ no `(AAG)` abu so dɛn?

Eyi yɛ tebea a ɛntaa nsi koraa ankasa. Sɛ nhwɛso no, wɔ United States no, nnipa bɛyɛ 100 pɛ na wohu sɛ wɔwɔ saa yare yi afe biara. Enti ɛntaa nsi koraa wɔ Sri Lanka. Enti mma nsuro sɛ wote eyi ho asɛm a, eyi nyɛ biribi a ɛto nnipa pii.

Dɛn ne `(AAG)` ho sɛnkyerɛnne? Yɛyɛ dɛn hu wɔn?

Saa tebea yi ho sɛnkyerɛnne betumi ayɛ soronko wɔ onipa biara ho. Ɛnyɛ obiara na obenya yare no ho sɛnkyerɛnne koro. Nsɛnkyerɛnne a ɛtaa ba ne:

• Akisikuru: Eyi kyerɛ akisikuru na ɛyɛ den sɛ ɔbɛkɔ tiafi.
• Aniwa a ɛtrɛw (Adie anim): Ɛtɔ mmere bi a, aniwa biako pɛ na ebetumi atrɛw na ebetumi ayɛ hann nka.
• Ano a ayow anaa aniwa a ayow: Nkate a ɛte sɛ nea nnompe anaa nusu nni aniwa no mu.
• Fainting / Syncope: Ebia wobɛtotɔ piti mpofirim a ntease biara nnim.
• Neurogenic orthostatic hypotension: Nkate a ɛma obi ho kyere no na ne ti yɛ hare bere a wosɔre fi baabi a ɔte anaasɛ woresɔre afi mpa so no. Eyi fi mogya mmoroso a ɛkɔ fam mpofirim .
• Ɛyɛ den sɛ wobɛtow wo ho / Nsu a ɛtwetwe: Nkate a ɛne sɛ wuntumi ntow wo ho ɛmfa ho sɛ wopɛ sɛ woto wo ho.

Wɔahu sɛ nnipa baasa biara mu bɛyɛ baanu a wɔwɔ AAG wɔ nnuru a ɛko tia nyarewa a wɔfrɛ no ganglionic acetylcholine receptor antibodies (g-AChR antibodies) pii wɔ wɔn mogya mu. Nhwehwɛmufo gye di sɛ abusuabɔ bi wɔ g-AChR nnuru a ɛko tia nyarewa yi dodow ne sɛnea sɛnkyerɛnne ahorow no mu yɛ den no ntam.

Ɔkwan bɛn so na oduruyɛfo hu saa `(AAG)` yare yi?

Sɛ wowɔ sɛnkyerɛnne ahorow te sɛ eyinom a, ade a oduruyɛfo bɛyɛ ne sɛ obebisa wo wo sɛnkyerɛnne ahorow no ho asɛm na wahwɛ wo. Afei, ebia wɔbɛyɛ nhwehwɛmu titiriw bi de ahwɛ sɛnea wo autonomic nervous system no yɛ adwuma.

Wɔn mu bi ni:

• Digestive system nhwehwɛmu: Sɛ nhwɛso no, ``Gastric emptying`` sɔhwɛ. Eyi sɔ sɛnea aduan fi wo yafunu mu ntɛmntɛm no hwɛ.
• `Quantitative sudomotor axon reflex test (QSART):` Eyi susuw sɛnea wo fifiri ntini no reyɛ adwuma.
• `Thermoregulatory sweat test`: Wɔyɛ saa sɔhwɛ yi de hwehwɛ sɛ nipadua no fifiri so atew anaasɛ akɔ soro, ne nea ɛde ba.
• `Tilt table test`: Wɔ sɔhwɛ yi mu no, wɔma woda mpa soronko bi so na wɔdannan mpa no nkakrankakra tẽẽ, hwɛ sɛnea wo koma bɔ ne wo mogya mmoroso sesa.
• Nsu mu nhwehwɛmu: Eyi betumi ama woahu sɛnea wo akisikuru no reyɛ adwuma yiye.

Bio nso, wobetumi ayɛ mogya mu nhwehwɛmu de ahwɛ sɛnea `(g-AChR antibodies)` a yɛadi kan aka ho asɛm no dodow te . Sɛ saa antibody yi dodow kɔ soro a, ɛyɛ adanse foforo sɛ ebia ɛyɛ `(AAG)`. Nanso, saa mogya mu nhwehwɛmu yi yɛ soronko wɔ `AChR antibody test`, a wɔde hwɛ sɛ ntini mu yare foforo bi a wɔfrɛ no `(Myasthenia Gravis).`

So aduru bi wɔ hɔ a wɔde sa eyi? Dɛn na wobetumi ayɛ?

Esiane sɛ AAG yɛ tebea a ɛntaa nsi saa nti, gyinapɛn biara nni hɔ, "eyi ne ayaresa no" de besi nnɛ. Mpɛn pii no , wɔde wɔn adwene si sɛnkyerɛnne ahorow a wɔde di dwuma so.

Nnuruyɛfo yɛ ayaresa nhyehyɛe denam akwan horow a wɔfa so sa yare a wɔde ka bom so. Ebi ne:

• Plasma exchange / plasmapheresis: Eyi hwehwɛ sɛ wobɛfa wo mogya no bi, ayi nneɛma a epira te sɛ antibodies afi plasma no mu, na afei wode mogya a wɔatew ho no asan ama wo nipadua.
• Intravenous immunoglobulin (IVIG) ayaresa: Nea ɛka eyi ho ne sɛ wode immunoglobulin (plasma fã bi a ɛko tia nyarewa) a wogye fi nnipa a wɔwɔ apɔwmuden hɔ bɛma wo nipadua. Eyi boa ma nipadua no mu tumi a ɛko tia nyarewa no dwo.
• Corticosteroids a wɔde fa ntini mu: Wɔde nnuru a wɔde twitwiw ɔyare mmoawa te sɛ methylprednisolone (Solu-Medrol®) anaa hydrocortisone (Solu-Cortef®) dodow a ɛdɔɔso ma.
• Nnuru a ɛma nipadua no tumi ko tia nyarewa: Eyinom yɛ adwuma denam nipadua no mu tumi a ɛko tia nyarewa no dwumadi a ɛtra so a ɛtew so. Sɛ nhwɛso no, nnuru te sɛ rituximab (Rituxan®).

So ɔkwan bi wɔ hɔ a wɔbɛfa so asiw saa `(AAG)` tebea yi ano?

Nokwarem no, ɔkwan biara nni hɔ a wɔahyɛ ho bɔ a wɔbɛfa so asiw eyi ano, efisɛ ɛntaa nsi koraa. Nanso, tebea horow bi te sɛ kokoram betumi ama asiane a ɛwɔ hɔ sɛ wubenya bi no ayɛ kɛse kakra. Sɛ woanya kokoram a, nneɛma bi a wubetumi ayɛ de ahwɛ w’akwahosan nyinaa so ni:

• Apɔw-mu-teɛteɛ a wɔyɛ no daa: titiriw apɔw-mu-teɛteɛ a ɛma mframa bɔ.
• Sɛ wowɔ `(Dysautonomia)` a, aduan a nkyene ne nsu pii wom a wubedi no betumi aboa ma woadi wo mogya mmoroso so. (Eyi yɛ biribi a ɛsɛ sɛ wo ne wo duruyɛfo susuw ho).
• Adwennwen a wubedi ho dwuma: Fa akwan a ɛfata te sɛ apɔw-mu-teɛteɛ, w’adamfo a wobɛkasa, ne nsɛm a wobɛkyerɛw wɔ nsɛmma nhoma mu di dwuma.
• Da yiye: Anyɛ yiye koraa no, da nnɔnhwerew 7-8 da biara.
• Ntade a wɔde hyɛ nipadua no mu: Eyinom boa ma mogya no kɔ nipadua no mu yiye na ɛtew ɔfe so (titiriw wɔ nan no mu).

So wobetumi asa saa `(AAG)` tebea yi koraa? Dɛn na ɛbɛba daakye?

Ɛsɛ sɛ meka sɛ, ɛwom sɛ me werɛ ahow de, .AAG nyɛ nea wotumi sa yare. Nanso, nnipa pii tumi di wɔn sɛnkyerɛnne ahorow no ho dwuma yiye bere tenten. Nea ɛyɛ nwonwa no, bɛyɛ nnipa baasa biara mu biako a wɔwɔ AAG no ho bɛtɔ no nkakrankakra a wɔrensa wɔn yare biara. Nanso, ɛsɛ sɛ nnipa dodow no ara di sɛnkyerɛnne ahorow bi ho dwuma wɔ wɔn nkwa nna nyinaa mu.

Dɛn bio na wopɛ sɛ wubisa oduruyɛfo no?

Sɛ wususuw sɛ wowɔ tebea bi a ɛte sɛɛ a, ɛho hia yiye sɛ wobɛkɔ oduruyɛfo pa nkyɛn na woabisa nea edidi so yi ho asɛm:

• Dɛn na ɛda adi sɛ ɛde me yare no ho sɛnkyerɛnne ahorow ba?
• Sɔhwɛ bɛn na ɛsɛ sɛ meyɛ de hu sɛ mewɔ `(AAG)` ankasa?
• Dɛn ne ayaresa a wobetumi apaw ama `(AAG)`?
• Dɛn ne nea ebetumi aba sɛ `(AAG)` ho sɛnkyerɛnne no bɛbrɛ ase mpo a wɔansa no yare?
• Nsakrae bɛn na ɛsɛ sɛ meyɛ wɔ asetra mu na ama m’akwahosan nyinaa atu mpɔn?

Nkwammoaa a ɛko tia nyarewa no tebea afoforo bɛn na ɛne autonomic neuropathy wɔ abusuabɔ?

``(AAG)`` yɛ soronko efisɛ ɛyɛ, ɔfã biako, yare a ɛfa nipadua no mu tumi a ɛko tia nyarewa ho, na ɔkwan foforo so no, ɛyɛ yare a nipadua no tumi ko tia nyarewa a ɛma nipadua no tumi ko tia nyarewa.

Bio nso, nyarewa afoforo bi wɔ hɔ a ɛfa nipadua no mu tumi a ɛko tia nyarewa ho a ɛma asiane a ɛwɔ hɔ sɛ obi benya ``dysautonomia'' (autonomic nervous system mu ɔhaw ahorow) no yɛ kɛse. Nhwɛso ahorow bi ne:

• Celiac yare a ɛma obi nya yare
• `(Guillain-Barré yare)` (Guillain-Barré yare) .
• `(Lupus / nhyehyɛe mu lupus erythematosus)` (Lupus yare) .
• `(Ntini mu yare)` (Ntini mu yare) .
• `(Sjögren yareɛ)` (Sjögren yareɛ) .

Enti wubetumi ahu, eyinom yɛ tebea horow a wobetumi de abɔ mu.

Nea etwa to no, nneɛma a ɛsɛ sɛ wokae (Take-Home Message) .

Okay, enti seesei wunim sɛ `(Autoimmune Autonomic Ganglionopathy - AAG)` yɛ yareɛ a ɛntaa nsi a yɛn ankasa defense system tow hyɛ yɛn autonomic nervous system so. Nsɛnkyerɛnne no betumi ayɛ sɛ obi adwene bɛyera mpofirim, `nhyɛso` a ɛso tew bere a ogyina hɔ, ne yafunu mu haw.

Mma wo ho nnyɛ wo hu bere a wote eyi no. Eyi ho yɛ na koraa. Afei nso, wɔde nnuru a ɛma nipadua no tumi ko tia nyarewa ne `plasmapheresis` di dwuma sɛ eyi ho ayaresa. Ɛwom sɛ wontumi nsa koraa de, nanso nnipa dodow no ara tumi di sɛnkyerɛnne ahorow no so yiye na wɔtra ase sɛnea ɛsɛ.

Nea ɛho hia sen biara ne sɛ wobɛkɔ oduruyɛfo a ɔfata nkyɛn ntɛm ara sɛ wowɔ saa sɛnkyerɛnne ahorow yi a. Sɛ wuhu wɔn ntɛm a, wubetumi afi ayaresa ase na woatew nkɛntɛnso a enya wɔ w’asetra so no so. Ɛnyɛ wo nko ara, na nnuruyɛfo bi wɔ hɔ a wobetumi aboa.

` Autoimmune Aneurysmal Ganglionopathy, AAG, Ntini a ɛko tia nyarewa, Ntini a ɛko tia nyarewa, Ntini mu yare, Nsɛnkyerɛnne, Ayaresa