Chair emaw khum atanga kan tho chuan engtik lai pawhin kan lu a buai emaw, kan lu a na emaw kan ti vek. A tam zawkah chuan second tlemte chhungin a bo vek. Mahse hetiang hi i chungah a thleng fo a nih chuan, emaw, i muhil leh tlu mai dawn nia i hriat chuan thil pangngai a ni lo mai thei. Vawiin hian heng symptoms thlentu natna hlauhawm tak kan sawi dawn a ni. Chu chu Pure Autonomic Failure, a tawi zawngin PAF a ni.
A awlsam zawngin, Pure Autonomic Failure (PAF) hi eng nge ni?
Ngaihtuah teh, kan taksaah hian kan thunun theih loha automatic-a lo awm thin process tam tak a awm a. Entirnan, thawk, lungphu, chaw ei dan, taksa lum leh vawt thununna leh thisen sang thunun. Heng zawng zawng hi kan Autonomic Nervous System-in a thunun vek a ni. Kan taksaa ‘auto-pilot’ system ang mai a ni.
Pure Autonomic Failure (PAF) hi thluak lama natna awm lo tak a ni a, hun kal zelah autonomic nervous system hnathawh a tichak lo zauh zauh thin. Hetiang thila thil thleng ber chu ṭhut emaw, mu emaw aṭanga i ding nghal chuan i thisen sang chu nasa takin a tlahniam a ni . Damdawi lam hawi chuan hei hi orthostatic hypotension kan ti a ni. Mahse hei chauh hi buaina a ni lo, PAF hian harsatna dang tam tak a thlen thei a ni.
Hetiang dinhmun hi kum 40 leh 60 inkar puitlingah hmuh a ni tlangpui a, tun hma chuan he natna hi Bradbury-Eggleston syndrome an ti bawk.
PAF natna lan chhuah dan chu engte nge ni?
Kan sawi tawh ang khan a lan chhuah dan tlangpui leh a langsar ber chu ding chunga luak chhuak leh muhil (orthostatic hypotension) a ni. Mahse, he autonomic nervous system hian kan taksa peng tam tak a thunun avangin a natna lan chhuah dan chu pakhat chauh a ni lo.
A pawimawh ber chu heng symptoms te hi mi zawng zawng tan a inang lo emaw, a awm dan tur a inang lo emaw a ni. Mi thenkhat chuan an ding chhuah huna an luak hmain symptom dang an nei thei.
PAF hian eng symptom dang nge a thlen theih tih i lo en ang u.
| System/process a nghawng dan | Symptoms awm thei te |
|---|---|
| Taksa thawk hah | A tlangpuiin thawk hahna a tlahniam (anhidrosis) emaw, a chang chuan thawk hah lutuk (hyperhidrosis) emaw a awm thin. |
| Mit hmuh theihna | Mihringte a lian tawk loh avangin mit a fiah lo (blurred vision). |
| Urinary system a ni | Bladder khar vek harsatna (urinary retention) emaw, zun control theih lohna (urinary incontinence) emaw. |
| Chaw ei dan (digestive system) a ni | Constipation emaw fecal incontinence emaw a awm thei. |
| Mipat hmeichhiatna hman dan | Harsatna, erectile dysfunction emaw anorgasmia emaw ang chi te. |
Tin, PAF vei zinga a chanve vel chuan thisen tlakchhamna (mild anemia) an nei thei bawk.
PAF din chhan hi eng nge ni?
Hei hi a chhan ber chu kan autonomic nerve cell-a protein pakhat alpha-synuclein an tih chu a dik lo taka a punkhawm vang a ni tih hmuhchhuah a ni. Pipe bawlhhlawh a tihkhawtlai ang bawkin, he protein hi nerve cell-ah a awm khawm chuan an hnathawh a tichhe thin.
He protein hi spinal cord sir lehlamah emaw, organs bulah emaw nerve bundles (autonomic ganglia) ah a awm a. Hun a kal zel a, heng nerve cell te hi a tlahniam a, autonomic system hnathawh a tibuai bawk. Mahse a pawimawh ber chu PAF-ah hian thluak emaw, ruhro emaw a chhe lo . Chuvangin, hriatrengna leh ngaihtuah thiamna ang chi thil a nghawng lo.
He protein hi engvangin nge hetiang hian a deposit tih hi zirchiangtute chuan an la hre chiang lo hle.
Natna dang nena inzawmna
Hetah hian hriat tur pawimawh tak a awm. He protein tho, alpha-synuclein hi Parkinson’s disease, Multiple System Atrophy, leh Lewy Body Dementia ang chi natnaah te thluaka a lo awm khawm thin a ni. Chuvangin, PAF vei thenkhat (a zawng zawng ni lo) hian hun kal zelah heng natna zinga pakhat hi an vei theihna chance an nei a ni. Hemi chungchang hi i doctor chuan a hriattir ang che.
He natna hi engtin nge kan hriat theih ang?
PAF hriatchhuah hi thil buaithlak deuh a ni a, doctor-te chuan i natna lan chhuahna thlentu tur thil dang, a bik takin i ding chhuah huna thisen sang tlahniam chu an paih chhuak hmasa ang.
Diagnostic process-ah hian:
- I natna lan chhuah dan leh i damdawi lam chanchin uluk taka zawhna zawh.
- Taksa enfiahna kimchang leh thluak enfiahna.
Chumi hnuah chuan natna hriat chian nan special test eng emaw zat neih a rawt thei bawk.
| Fiahna | Hei hi ti mai rawh... |
|---|---|
| Tilt Table Test a ni | Khum bik chungah dah i ni ang a, chutah chuan ding thei dinhmunah sawn nghal i ni ang. I thisen sang leh heart rate te a enfiah ang. |
| QSART (sweat test) tih a ni. | Hei hian thawmhnaw control tu nerve te hnathawh dan a en a ni. Hei hian autonomic nervous system-a harsatna awmte hriatchhuahna kawngah a pui a ni. |
| Imaging test (MRI ang chi) 1.1. | Brain MRI scan ang chi thil hi damdawi lam thil dang, natna lan chhuahna thlentu tur a awm loh nan an ti thin. |
| Laboratory Test (Thisen leh Zun) 1.1. | Thisen leh zun chhunga chemical norepinephrine an tih level hi mut lai leh ding chhuak hnua teh thin a ni. PAF-ah chuan heng level-ah hian characteristic change a awm a. |
Test tam tak neih hi a harsa thei hle a, mahse natna dik tak kan hriatchhuah chauhvin i tan enkawlna ruahmanna tha ber kan siam thei ang.
PAF enkawlna tur engte nge ni?
Hei hi damdawi a la awm lo. Mahse, lungngai suh. I symptoms enkawl dan tur leh nun pangngaia nunpui turin thil tam tak i tih theih a ni. I doctor leh medical team te chuan i tan chauh enkawl dan tur ruahmanna siam turin an thawk ho ang che.
Orthostatic Hypotension (ding laia thisen sang hniam) control
Hei hi buaina ber a ni. Hei hi damdawi ni lo enkawlna leh damdawi pawh a awm vek.
- Hmun i thlak dawnin fimkhur rawh: Mut emaw, thut emaw atanga i tho chuan zawi zawiin ti rawh . Tho thut suh.
- Tui leh chi: I doctor chuan ni khatah tui litre 3 vel in turin a rawt mai thei. I chaw eiah chi tlem tal dah belh turin a hrilh thei bawk che a ni.
- Khum buatsaih dan: Khum lu chu inches 4-6 vel chawi sangin mu rawh. Chutiang chuan i thawh hunah thisen sangah danglamna lian tham i nei lovang.
- Compression Garments: Special tight stockings (compression garments) kawr emaw, kekawr emaw thlenga lo thleng hian kea thisen awmkhawm a tihziaawm a, thisen sang a ti nghet thei bawk.
- Hei bakah hian doctor chuan thisen sang tisang thei damdawi bik a rawt bawk.
Efecation leh zun chhuahna lama harsatna
- Constipation atan: Fiber tamna ei tur (thlai, thei, bran rice) ei leh stool softeners hman ang chi thil i ti thei bawk .
- Zun lama harsatna nei tan: Zun harsatna i neih chuan catheter hman a ngai mai thei. I zun i control theih loh chuan damdawi bik (Oxybutynin, Mirabegron ang chi) a awm a.
Mipat hmeichhiatna hmansualna
Hei atan hian sildenafil emaw tadalafil ang chi damdawi awm mahse, heng damdawi te hian orthostatic hypotension hlauhawmna a tipung thei a ni. Chuvangin, he thu hi i Doctor nen zalen takin sawipui la, hengte hi a thurawn hnuaiah chauh hmang rawh.
He natna vei tan hian nakin lawkah eng nge thleng ang?
He natna vei mi pakhat hmalam hun tur chungchangah hian chhanna pakhat chauh pek a harsa a, a chhan chu mi hrang hrangah a inang lo.
PAF hi natna zual zel a ni. Mahse mi tam tak tan chuan zawi zawiin a thleng thin a, kum tam tak chhung, kum sawm tam tak chhung pawhin a thleng thin. Zirna thenkhatah chuan PAF hmuhchhuah a nih hnua dam rei zawng chu kum 12.5 vel a ni tih hmuhchhuah a ni. Mahse hei hi a zatve chauh a ni a, i natna lan chhuah dan leh hriselna a zirin a danglam thei hle. Chuvangin chutiang number-ah chuan inkhung lutuk suh.
PAF i neih chuan i enkawlna a thawk tha em tih leh i dinhmun a kal zel dan tur hriat chian nan doctor pan fo a pawimawh. Parkinson natna lanchhuahna (khuh, kal harsatna ang chi) i neih chuan doctor hnenah i hrilh nghal tur a ni.
Take-Home Thuchah a ni
- Pure Autonomic Failure (PAF) hi natna hmuh tur awm lo tak a ni a, kan taksaa automatic process control tu nervous system a nghawng a ni.
- A lan chhuah dan ber chu thisen sang tlahniam vanga thut emaw, mut emaw atanga tho chhuah hian luak chhuak leh muhil a ni.
- He natna hi tihdam kim theih ni lo mah se, a symptoms te chu uluk taka thunun theih a ni a, nunphung sang tak a vawng reng thei bawk.
- Hei hi ven dan tur a awm lo va, a lo thleng hi i sualna a ni lo.
- Doctor nen inbiak reng a, i enkawlna tur ruahmanna dik taka zawm a pawimawh hle. I zawhna leh lungkhamnate chu tlang taka sawi chhuah hlau suh.
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