Taksa chhunga protein awmte hi a buai em? He amyloidosis chungchang hi kan sawi a ni!

Tunlai hian i chau a, i chau a, sawifiah theih lohvin i taksa rihna i tlahniam pawh a ni mai thei? A châng chuan kan taksaa inthlâk danglamna tenau te hian thil lian tak tak a thlen thei a. Vawiin hian kan taksaa protein chi khat chu a â deuh a, a pianzia a dik lo va, kan taksa peng pawimawh tak takah a awm a, a lo awm ṭhin natna chungchang kan sawi dawn a ni. Hei hi amyloidosis kan tih chu a ni . Ngaihtuah buai suh, he thu hi awlsam tak leh i hriatthiam theih tur angin kan sawi dawn a ni.

Amyloidosis hi eng nge ni tak tak?

A awlsam zawngin, amyloidosis hi kan taksaa amyloid protein (a takah chuan protein pangngai lo tak tak) te chu an inzawm khawm dik lo va, an inzawm khawm a, fibrils an tih, fibrous clumps te tak te, fibrous clumps an siam a, chu chu kan thinlung, kal leh thin ang chi organ pawimawh tak takah a awm a ni. An tih chuan, leh enkawl loh chuan heng protein fibril pangngai lo tak takte hian kan taksa peng hrang hrangte chu nasa takin a tichhe thei a ni.

A châng chuan heng protein pangngai lote hi taksa peng pakhat hmun tlemteah chauh a awm khawm thei a ni. Hei hi localized amyloidosis an ti a . Hei hian kan vun, zunthlum hmun tenau emaw, thawkna kawng emaw a nghawng fo thin. Mahse, a tam zawkah chuan heng fibril te hi taksa peng pum puiah emaw, taksa pum puia taksa peng hrang hrangah emaw a awm thin. Hei hi systemic amyloidosis an ti a .

Chanchin tha a awm. Doctor-te chuan amyloidosis thlentu protein pangngai lo siam chhuah chu an titawp thei a ni. Amyloidosis chi thenkhatah chuan kan taksa hriselna (immune system) hian heng protein te hi a tifai thei a ni. Hei hian i natna lan chhuah dan a tihziaawm thei a, a natna taksa peng hrang hrangte hriselna a ti tha thei bawk.

Amyloidosis chi hrang hrang a awm em?

Ni e, doctor-te chuan he natna hi a natna protein chi hrang hrang a zirin an thliar hrang thin. A chi hrang hrang hmuh tur lian ber berte chu:

• AL amyloidosis : Hei hi i taksain light chain proteins (antibodies-a tel ve lian ber) a siam chhuah dan a ni. Heng fibrils te hi thinlung (cardiac amyloidosis) leh kal (kidney) ah te an lo awm khawm tlangpui. AL amyloidosis hian i nerve, vun leh chaw ei kawng a nghawng thei bawk. Hei hi amyloidosis chi khat a ni ber.
• AA amyloidosis : Hetah hian serum A protein chi hrang hrang i taksa peng hrang hrangah a lo awm khawm thin. Hei hi hun rei tak chhunga inflammatory condition i neih chuan a thleng thei a, chu chu taksaa inflammation thlentu natna (entir nan, rheumatoid arthritis, inflammatory bowel disease, emaw hun rei tak chhunga infection) tihna a ni. Hei hian i kal (kidney), chaw ei kawng (digestive organs) emaw, i thinlung emaw a nghawng thei a ni.
• ATTR amyloidosis: Hetiang dinhmunah hian i liver hian transthyretin protein chu a tam lo hle a ni . He transthyretin protein pangngai lo hi thinlungah a awm a, a chang chuan nerve-ah a awm bawk. A chi thenkhat chu rochun (familial amyloidosis) a ni a, thenkhat erawh chu mi kum a upat deuh deuh hnuah a lo piang chhuak thung (wild-type ATTR).

He natna hi engtiang chiahin nge a hluar?

Amyloidosis hi a takah chuan natna hmuh tur awm lo tak a ni . Entîr nân, United States-ah pawh doctor-te chuan kum tin AL amyloidosis vei thar 1,275 aṭanga 3,200 chauh an awm niin an chhut. Chuvangin, natna tlanglawn tak a ni lo.

He natna lan chhuah dan engte nge ni?

Amyloidosis natna lan chhuah dan hi mi hrang hrangah a inang lo thei a, chu chu protein pangngai lo chi leh fibrils awmna hmun a zirin a ni.

Ngaihtuah teh, heng protein te hi thinlungah (`(cardiac amyloidosis)`) a awm a nih chuan, a lan chhuah dan chu chak lohna, thawk harsa, muhil (hei hi thinlung rhythm pangngai lo chhinchhiahna a ni thei) emaw, ke ruh hring nen thinlung chak lohna emaw a ni.

Tin, heng protein te hi kal (renal amyloidosis)-ah a awm chuan ke leh ke a hring leh zun chhunga bubble hmuh ang chi symptoms a awm thei bawk.

Amyloidosis-ah hian a lan chhuah dan tlangpui engemaw zat a awm a:

• Ngaihtuah phak loh khawpa chau tih hriatna.
• Chhan awm lovin taksa rihna tihtlem.
• General weakness or numbness in the limbs, thil pakhat dik taka man theih lohna.
• Kut ah chuan a rilru a buai tih hriat.
• Vun inthlak danglam: Hei hian vun a hliam awlsam leh mit vel a purple spot (purpura) te ang chi thil a kawk a ni.

Engvangin nge he amyloidosis hi a lo awm?

Amyloidosis hi kan taksaa protein awmte chu a inzawm sual a, a sticky a nih chuan a lo awm thin. An inzawm khawm a, clump, a nih loh leh fibril an siam a, taksa peng leh tissue-ah an dah khawm a ni. Doctor-te chuan hei hi "protein misfolding disorder" tiin an sawi. Protein a awm tur, chain sei takah chuan a inzawm khawm a, taksain a ti chhe tha thei lo va, harsatna a thlen thin.

Hei hi a thlen thei thilte chu:

• Gene changes (mutations) : Gene change (mutation) i rochun thei a, chu chuan amyloid proteins pangngai lo a siam thei. A nih loh leh, heng gene mutations te hi mi dam chhungin a thleng thei a, chhan hriat loh vang a ni.
• Damdawi lam dinhmun hnuaia awm dangte: A chang chuan amyloidosis hi damdawi lam dinhmun hran avanga lo awm thei a ni. Hei hi AA amyloidosis-ah chuan a thleng thin. Hei hi hun rei tak natna vei emaw, rheumatoid arthritis ang chi chronic inflammatory conditions nei te tan a tam zawk.

Hetiang hi tuin nge nei tam zawk? (Risk Factor hrang hrang) 1.1.

Amyloidosis lo awm theihna tur tichhe thei risk factor engemaw zat a awm a:

• Kum : He natna hi kum 60 aia upa te zingah a awm tam ber .
• Gender : Mipa hian he natna hi an vei tam zawk.
• Hnam : United States-ah hian mipa hringte hian `ATTR amyloidosis`, thlahtute atanga lo piang chhuak, genetic mutation hi an rochun tam zawk tih hmuhchhuah a ni. (Kan ramah hian hemi chungchangah hian data chiang tak a awm tlem hle a, mahse hnam thenkhat chu chi thenkhatah an inhmang zawk niin an sawi tlangpui.)
• Damdawi lam thil dang : Chronic inflammatory condition neih hian AA amyloidosis vei theihna a tipung. Tin, thisen cancer chi khat multiple myeloma vei zinga 12% leh 15% inkar chuan AL amyloidosis an vei bawk.
• Hun rei tak chhung dialysis : Kal natna khirh tak nei, rei tak dialysis nei tawhte pawhin amyloidosis chi thenkhat an nei thei bawk.
• Chhungkaw chhunga Amyloidosis : Amyloidosis chi thenkhat chu chhungkuaah a kal thei. Chumi awmzia chu rochun theih an ni tihna a ni.

Hei hian eng harsatna nge a thlen theih?

Heng amyloid deposit te hian a nghawng taksa peng te chu a thawk tha thei lo. Harsatna lo awm thei te chu:

• Lung hnathawh a tlahniam a, a tawpah chuan thinlung a chak lo.
• Kidney natna leh a tawpah chuan kidney failure .
• Neuropathy (chu chuan rilru hahna leh chak lohna a thlen thin).

Mahse, medical team dik tak nena enkawlna i zawn chuan heng harsatnate hi i tihziaawm thei tih hre reng ang che.

Engtin nge he natna hi dik taka hriat theih a nih? (Diagnosis) tih a ni.

Doctor-te chuan biopsy an ti a, amyloidosis thlentu protein chu an hmuchhuak a ni. Hei hian a natna taksa peng atanga tissue te tak te emaw, heng fibrils te hi an lo awm fo thinna hmun (entir nan, i pum vun hnuaia thau emaw, i ruhro emaw) atanga lak chhuah a ni.

Chu bakah, test dang i tih theih a awm bawk:

• Thisen leh zun test : Heng test te hian i thisen emaw zun emaw ah protein level dik lo a awm leh awm loh a enfiah thei a ni. Tin, a nghawng taksa peng hrang hrangte hnathawh dan hriat nan pawh hman theih a ni bawk.
• Imaging test: Heng test te hian doctor-te chu taksa peng natna (organs) tihchhiat dan a hmuh theih nan a pui thei a ni. Fibrils awmna hmun azirin, echocardiogram , thinlung lam entu, MRI , taksa peng leh tissue chipchiar taka tarlanna emaw, test dang emaw i mamawh thei bawk.
• Genetic tests : I doctor-in gene mutation avanga amyloidosis i vei a rinhlelh chuan he test hi tih theih a ni.

Natna hi stage hrang hrang a awm em?

Ni e, doctor-te chuan natna hi eng chen nge a darh tawh tih leh a nasat dan hriat nan amyloidosis staging an hmang thin . Natna chi hrang hrang staging system hrang hrang a awm a. Natna stage tur chuan i doctor chuan a hnuaia mite hi a ngaihtuah thei ang:

• Organ tihchhiat nasat dan.
• Thisen test-ah hian amyloid protein pangngai nena khaikhin chuan protein pangngai lo a tam zawk tih hmuhchhuah a ni.
• I symptoms leh a nasat dan.

I amyloidosis stage leh i prognosis a nghawng dan tur chungchang doctor nen inbiakna neih a pawimawh hle.

Eng enkawlna nge awm?

Doctor-te chuan amyloidosis hi a bulpui ber enkawlna hmangin an enkawl a ni. Enkawlna hian amyloidosis natna a tikhawtlai thei a, fibril thar siam a veng thei bawk. Damdawi hian i natna lan chhuah dan a tiziaawm thei bawk. AL amyloidosis-ah chuan enkawlnain amyloid deposit thar a siam loh chuan i immune system chuan a awm tawhte chu a tifai thei a ni. I taksa peng hrang hrang atanga amyloid tihfai dan tur siam that dan tur zawn chhuah mek a ni.

Amyloidosis enkawlna thenkhat chu:

• Chemotherapy : He enkawlna hian AL amyloidosis thlentu light chain protein dik lo siamtu plasma cell pangngai lo chu a tichhia a ni. Mi tam tak chuan chemotherapy damdawi nen steroid an ei dun thin.
• Targeted therapy : He enkawlna hian amyloidosis thlentu protein, gene emaw tissue bikte a target a ni. Protein te chu a inzawm sual loh nan damdawi a huam a ni.
• Organ transplant : Kal, thin emaw, thinlung emaw chu a thawk tha thei tawh lo khawpa a chhiat chuan doctor-te chuan transplant a rawt thei a ni. Hei hi amyloidosis inherited forms enkawlna a ni thei.

Amyloidosis vei chuan eng nge a ngaihtuah tur?

I doctor hian i natna lan chhuah dan a enkawl thei a, natna darh zel tur a thunun thei a, a then phei chuan natna tihdanglamna kawngah pawh a pui thei bawk. Mahse, amyloidosis chi thenkhat chu enkawl loh chuan nunna atana hlauhawm taksa peng hrang hrang a tichhe thei a ni. Chuvang chuan natna hi hriat hmasak a, enkawl tan vat a pawimawh em em a ni.

Engtin nge nangmah pawh i in enkawl duh?

Amyloidosis chi hrang hrang a awm avangin natna enkawl dan tur pakhat chauh a awm lo. I doctor hnenah eng step nge i tan a tha tih zawt rawh.

Hei hian a huam thei ang:

• Chaw tha tak tak ei leh exercise neih fo hmanga i taksa hriselna enkawl .
• I rilru hriselna chu dah pawimawh ber rawh . I doctor hnenah amyloidosis vei tan support group chungchang zawt rawh. Chutiang natna hmuh tur awm lo tak nena khawsakna avanga lungkhamna leh mahni chauha awm theihna tur kawng tha tak a ni thei a, nangmah ang bawka harsatna tawk mek midangte nena inzawmna siamna kawng tha tak a ni thei bawk.

Engtikah nge Doctor i hmuh ang?

Symptoms a inang lo em em a, engtikah nge doctor pan tur tih hriat a harsa thei hle. Mahse a tlangpuiin, i hriatthiam loh leh rei lo te hnua a reh loh tur symptom i neih chuan doctor i pan ngei ngei tur a ni. Amyloidosis a ni lo mai thei, mahse a nih chuan i hriat hma chuan a tha zawk.

Doctor hnenah eng zawhna nge i zawh tur?

I doctor zawh theih tur zawhna thenkhat chu hetiang hi a ni:

• Eng ang amyloidosis nge ka neih?
• Engvangin nge amyloidosis ka neih?
• Eng ang enkawlna nge ka mamawh?
• Enkawlna hian eng side effect nge a neih?
• Enkawlna hnuah ka prognosis chu eng nge ni?

A tawp berah chuan thil hriat reng tur (Take-Home Message) .

Amyloidosis hi natna khirh tak a ni tak zet a, a chhan chu mi zawng zawng a nghawng dan a inang lo. A then phei chuan enkawl theih a ni thei bawk. Thil dangah chuan nunna atana hlauhawm tak a ni thei bawk. Hei hi thil buaithlak leh buaithlak tak a ni thei. Tin, natna awm lo tak a nih avangin tanpuina i mamawh ber hunah nangmah chauhvin i inhria thei bawk.

A pawimawh ber chu nangmah chauh i ni lo tih hriat reng hi a ni. I doctor chuan i tan enkawlna tha ber ber a hrilhfiah thei che a, chu chu i amyloidosis chi hrang hrang a zirin a ni. Tin, i tanpui thei tur resources leh support group te pawh an kawhhmuh thei bawk. I medical team chuan he natna dona atana i mamawh enkawlna leh puihna tha ber zawng turin a kaihruai thei che a ni.

` Amyloidosis, protein, protein pangngai lo, taksa peng chhiatna, thinlung natna, kal natna, thluak lam natna