Sɔntɛnde, yu kin fil lɛk se yu ed de tɔn wantɛm wantɛm? Ɔ yu kin fil lɛk se sɔntin strenj de na yu bɛlɛ, yu mɔt dɔn dray, ɔ yu nɔ ebul fɔ kɔntrol aw yu de blo? Pan ɔl we dɛn tin ya kin tan lɛk nɔmal tin, sɔntɛnde, wan stori kin de biɛn dɛn we kɔmplikt mɔ. Dat na wan pan dɛn kɔndishɔn dɛn we nɔ kin apin so ɔltɛm, bɔt i fayn fɔ no, we wi go tɔk bɔt tide.
Wetin na dis ``Autoimmune Autonomic Ganglionopathy - AAG''? Lɛ wi ɔndastand am simpul wan, nɔto so?
Fɔ tɔk am simpul wan, ``Autoimmune Autonomic Ganglionopathy'' ɔ ``(AAG)'' na wan kɔndishɔn we wi yon bɔdi in difɛns sistɛm, dat na di imyun sistɛm, kin mistek atak wi yon ɔtonomik nεv sistɛm. Imajin, dis na laik wetin go hapun if di ami we sopoz to protekt wi kontri mistek atak wi oun impotant ples.
di כtonom nεv sεstem na wi bכdi lεk wi ``otomatik sistεm''. Dat min se, i de kɔntrol bɔku tin dɛn we kin apin ɔtomɛtik wan, ɛn wi nɔ kin no. Fɔ ɛgzampul:
- Di we aw yu de blo
- At rit (at rit) .
- Blɔd prɛshɔn
- We pɔsin de swet
- Di we aw pɔsin de digest
Bɔku pan dɛn tin ya nɔto sɔntin we wi kin tink bɔt. Dɛn kin apin ɔtomɛtik wan. di כtonom nεv sεstem na in de εp fכ mek dεn כtomatik fכnshכn dεm de rכn fayn fayn wan. Insay AAG, dis impɔtant sistɛm kin pwɛl. Dis na wan sik we dɛn kɔl ɔtonomik nyuropathy, ɔ dysautonomia.
Wetin mek wi yon bɔdi de du dis? Wetin na di rizin dɛn?
Infakt, ivin dɔktɔ dɛn stil nɔ ebul fɔ tɔk ɛksaktɔli wetin na di ɛksaktɔl kɔz fɔ dis `(AAG)`. Bɔt dɛn dɔn kam fɔ no sɔm tru tin dɛn.
- Bɔrku tɛm, dat na , pan lɛk tri pan ɛvri fayv pipul dɛm wae gɛt ``AAG'', dis kin apin afta dɛn dɔn wɛl frɔm ɔda sik, ɔr afta dɛn dɔn gɛt infɛkshɔn. I tan lɛk wan sayd ɛfɛkt we da sik de gɛt.
- Sɔm pipul dɛn kin gɛt wan sik we dɛn kɔl paraneoplastic AAG. dis na we sכm antibodi dεm we wi imyun sistεm de mek fכ ansa wan kכndyushכn lεk kεnsar go εn atak di כtonom nεv sεstem.
Wetin dis kin du to wi bɔdi? Lɛ wi tek tɛm luk gud wan.
wi כtonom nεv sεstem gεt grup dεm fכ nεv sεl dεm we dεn kכ l כtonom ganglia . Dɛn tin ya tan lɛk say dɛn we tren kin jɔyn. Dɛn kin sɛn ɛn gɛt difrɛn mɛsej dɛn tru dɛn wan ya. dis chenj fכ mεsej implεnt fכ di כtomatכk akshכn dεm na wi bכdi, dat na di akshכn dεm we wi nכ de kכntro (rifleks), fכ kכntro.
insay AAG, wi imyun sistεm de atak spεsifi k rεsεpכta dεm na dεn ganglia dεm ya. Risepta na di ples dɛn we dɛn kin gɛt mɛsej. so we dεn rεsεpכta dεm ya dεn dכn pwεl, dεn nεv mεsej dεm nכ de go כ pas fayn fayn wan. Dis kin mek tin dɛn we kin apin ɔtomɛtik wan, lɛk fɔ kɔntrol blɔd prɛshɔn ɛn fɔ swet, kin bigin fɔ go haywire.
Imajin, if wan `kɔnɛkshɔn` na yu os in `mɛn switch bɔd` lɔs, di layt dɛn nɔ go ɔn, di fan nɔ go spin, ɛn ɔda tin dɛn Na so i bi wit dis.
Aw kכmכn dεn kכl dis kכndyushכn `(AAG)`?
Dis na rili wan sik we nɔ kin apin so ɔltɛm. Fɔ ɛgzampul, na Amɛrika, na lɛk 100 pipul dɛn nɔmɔ dɛn kin gɛt dis sik ɛvri ia. So i nɔ kin apin so ɔltɛm na Sri Lanka. So nɔ fred we yu yɛri bɔt dis, dis nɔto sɔntin we kin apin to bɔku pipul dɛn.
Wetin na di simptom dɛm fɔ `(AAG)`? Aw wi kin no dɛn?
De sayn dɛm fɔ dis sik kin difrɛn frɔm wan pɔrsin to ɔda pɔrsin. Nɔto ɔlman go gɛt di sem kayn sik. De sayn dɛm wae kin kam pan pɔrsin na:
- Kɔnstipɛshɔn: Dis min se pɔsin kin gɛt bɛlɛ ɛn i nɔ kin izi fɔ go na tɔylɛt.
- di pupil dεm we dilayt (Adie in pupil dεm): sכmtεm na wan yay in pupil dεm kin dilayt εn i kin sεnsitiv to layt.
- Dray mɔt ɔ dray yay: Na filin lɛk se nɔ saliva ɔ kray wata nɔ de na di yay.
- Fainting / Syncope: Yu kin faint wantɛm wantɛm fɔ no rizin.
- Nyurojɛnik ɔrtɔstatik haypɔtɛnshɔn: Na filin fɔ diziz ɛn layt ed we yu tinap frɔm we yu sidɔm ɔ we yu kɔmɔt na bed. Dis kin kam bikɔs di blɔd prɛshɔn kin go dɔŋ wantɛm wantɛm .
- Difikulti fɔ pis / Urinary retention: Na filin fɔ nɔ ebul fɔ pis pan ɔl we yu want fɔ pis.
Dɛn dɔn si se lɛk tu pan ɛvri tri pipul dɛn we gɛt AAG gɛt ay levul antibodi dɛn we dɛn kɔl ganglionic acetylcholine receptor antibodies (g-AChR antibodies) na dɛn blɔd. Risach pipul dεm biliv se link de bitwin di lεvεl dεm fכ dεn g-AChR antibodi dεm ya εn di siriכs simptom dεm.
Aw dɔktɔ kin no dis `(AAG)` sik?
If yu gɛt sayn dɛn lɛk dis, di fɔs tin we dɔktɔ go du na fɔ aks yu bɔt yu sik ɛn chɛk yu. Dɔn, dɛn kin du sɔm spɛshal tɛst dɛn fɔ chɛk aw yu ɔtonomik nɛvɔ sistɛm de wok.
Na sɔm pan dɛn:
- di dijestiv sistεm tεst dεm: fכ egzampl, na ``Gastric emptying`` tεst. Dis kin tɛst aw it kin kɔmɔt na yu bɛlɛ kwik kwik wan.
- `Quantitative sudomotor axon reflex test (QSART)`: dis de mכsu aw yu swet gland dεm de wok fayn fayn wan.
- `Thermoregulatory sweat test`: dεn kin du dis tεst fכ no if di bכdi in swet dכn dכn כ inkrεs, εn wetin na di kכz.
- `Tilt table test`: Insay dis tɛst, dɛn kin mek yu ledɔm pan spɛshal bed ɛn dɛn kin rɔta di bed sloslo vertikal, fɔ wach aw yu at rit ɛn blɔd prɛshɔn kin chenj.
- Urinalysis: Dis kin gi yu wan aidia bɔt aw yu blad de wok fayn fayn wan.
pan tap dat, dεn kin du bכdi tεst fכ chεk di lεvεl fכ di `(g-AChR antibodies)` we wi bin dכn tכk bכt . if di lεvεl fכ dis antibodi hכy, i de fכda pruf se i kin bi `(AAG)`. כltu, dis bכdi tεst difrεn frכm di `AChR antibodi tεst`, we dεn kin yuz fכ chεk fכ כda nyurolכjik sik we dεn kכl `(Myasthenia Gravis).`
Yu tink se tritmɛnt de fɔ dis? Wetin dɛn kin du?
Bikɔs AAG na so wan rare kɔndishɔn, nɔ standad de, "dis na di tritmɛnt" yet. Bɔrku tɛm , di fɔs tin na fɔ mɛn di sik.
Dɔktɔ dɛn kin mek wan tritmɛnt plan bay we dɛn kin jɔyn difrɛn tritmɛnt dɛn. Sɔm pan dɛn na:
- Plasma exchange / plasmapheresis: Dis min se yu fɔ tek sɔm pan yu blɔd, pul tin dɛn we de ambɔg yu lɛk antibodies frɔm di plasma, ɛn afta dat yu gi di blɔd we dɛn dɔn klin bak to yu bɔdi.
- Intravenous immunoglobulin (IVIG) therapy: Dis na fɔ gi yu bɔdi immunoglobulin (wan pat pan plasma we de fɛt sik) we dɛn tek frɔm pipul dɛn we gɛt wɛlbɔdi. Dis kin ɛp fɔ mek di imyun sistɛm kol.
- Intravenous corticosteroids: Dɛn kin gi ay dos fɔ anti-inflammatory steroids, lɛk methylprednisolone (Solu-Medrol®) ɔ hydrocortisone (Solu-Cortef®), bay injɛkshɔn.
- Immunosuppressive medications: Dɛn wan ya de wok bay we dɛn de ridyus di ɔva aktiviti na di imyun sistɛm. Fɔ ɛgzampul, mɛrɛsin dɛn lɛk rituximab (Rituxan®).
We de fɔ mek dis `(AAG)` sityueshɔn nɔ apin?
Rili no garanti nɔr de fɔ mek dis nɔr apin, as i nɔr kin apin so ɔltɛm. Bɔt sɔm tin dɛn lɛk kansa kin mek yu gɛt am smɔl. If yu dɔn gɛt kansa, na sɔm tin dɛm wae yu kin du fɔ kia fɔ yu ɔl yu wɛl bɔdi:
- Ɛksesaiz ɔltɛm: mɔ we yu de du ɛksɛsayz we yu de yuz aerobik.
- If yu gɛt `(Dysautonomia)`, fɔ it it we gɛt bɔku sɔl ɛn wata kin ɛp fɔ kɔntrol yu blɔd prɛshɔn. (Dis na sɔntin we yu fɔ tɔk to yu dɔktɔ.)
- Fɔ kɔntrol yu strɛs: Yuz fayn fayn we dɛn lɛk fɔ du ɛksɛsayz, fɔ tɔk to yu padi, ɛn fɔ rayt na jɔnal.
- Gɛt gud slip: Slip fɔ at le 7-8 awa insay di de.
- We yu wɛr kɔmpreshɔn klos: Dɛn tin ya kin ɛp fɔ mek di blɔd go fayn ɛn fɔ mek yu nɔ swel (espɛshali na di leg dɛn).
Dis `(AAG)` situeshɔn kin wɛl kɔmplit wan? Wetin go apin tumara bambay?
A fɔ se, pan ɔl we a sɔri, .AAG nɔ kin mɛn. Bɔt bɔku pipul dɛn kin ebul fɔ kɔntrol dɛn sik dɛn fayn fayn wan fɔ lɔng tɛm. I sɔprayz fɔ no se lɛk wan pan ɔl tri pipul dɛn we gɛt AAG go bɛtɛ smɔl smɔl if dɛn nɔ gɛt ɛni tritmɛnt. Bɔt, bɔrku pipul dɛn go gɛt fɔ dil wit sɔm sayn dɛm ɔlsay na dɛn layf.
Wetin bak yu wan aks di dɔktɔ?
If yu sɔspɛkt se yu gɛt dis sik, i rili impɔtant fɔ go to gud dɔktɔ ɛn aks bɔt dɛn tin ya:
- Wetin na di tin we kin mek a gɛt dis sik?
- Us tɛst a fɔ du fɔ no if a gɛt `(AAG)`?
- Wetin na di tritmɛnt opshɔn dɛm fɔ `(AAG)`?
- Wetin na di chans fɔ mek di sayn dɛm fɔ `(AAG)` go shɔt ivin if dɛn nɔ gɛt tritmɛnt?
- Us chenj dɛn a fɔ mek na mi layf fɔ mek a gɛt wɛlbɔdi ɔlsay?
Us ɔda imyun kɔndishɔn dɛn we gɛt fɔ du wit ɔtonomik nyuropathy?
``(AAG)`` na spεshal biכs na, na wan say, sik we de rilet to di imyun sistεm, εn na di כda say, na כtoimyun sik na di nεv sεstem.
apat frכm dat, sכm כda sik dεm de we de fכm di imyun sistεm we de inkrεs di risk fכ divεlכp ``dysautonomia'' (dizכrd dεm fכ di כtonom nεv sεstem). Sɔm ɛgzampul dɛn na:
- Siliak sik we dɛn kin gɛt
- `(Gilain-Barré sindrom)` (Gilain-Barré sindrom)
- `(Lupus / sistεmik lupus εrytematosus)` (Lupus sik)
- `(Rɔmatɔyd atraytis)` (Rɔmatɔyd atraytis) .
- `(Sjögren in sindrom)` (Sjögren in sindrom)
So yu kin si, dis na situeshɔn dɛn we kin gɛt sɔntin fɔ du wit dɛnsɛf.
Fɔ dɔn, tin dɛn fɔ mɛmba (Take-Home Message) .
Okay, so naw yu no se `(Autoimmune Autonomic Ganglionopathy - AAG)` na wan sik we nɔ kin apin so ɔltɛm we wi yon difɛns sistɛm de atak wi ɔtonomik nɛvɔ sistɛm. di simptom dεm kin bi fכ lכs kכnsεns wan wan, fכ dכn pan `prεshכn` we yu tinap, εn bεlε prכblεm.
Nɔ fred we yu yɛri dis. Dis nɔ kin apin so ɔltɛm. כlso, dεn de yuz imyunosuprεshכn dεm εn `plasmafεresis` as tritmεnt fכ dis. Pan ɔl we dɛn nɔ kin ebul fɔ mɛn am ɔl, bɔku pipul dɛn kin ebul fɔ kɔntrol di sik dɛn fayn fayn wan ɛn liv nɔmal layf.
Di tin we impɔtant pas ɔl na fɔ go to dɔktɔ we sabi du di wok kwik kwik wan if yu gɛt dɛn sik ya. If yu no dɛn kwik, yu kin bigin fɔ trit yu ɛn ridyus di impak pan yu layf. Nɔto yu wan de, ɛn dɔktɔ dɛn de we go ɛp yu.
` Awtoimyun Anyurismal Ganglionopathy, AAG, Awtoimyun Nεv Sistem, Imyun Sistem, Nyurolכjik Sik, Simptom, Tritmεnt
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