He natna hi i lung leh kal pahnih a ruala khawih thei hi i hria em? Goodpasture Syndrome chungchang hi han sawi ila!

A chang chuan khuh reng reng i nei em? Chu khuh chuan a chang chuan thisen tlem a rawn chhuah thin em? A nih loh leh thawk harsa i ti em, i chau a, i zun chhuah pawh a tlahniam em? Chûngte chu thil pawi tak chhinchhiahna a ni thei. Vawiin hian chutiang dinhmun hmuh tur awm lo pakhat, mahse i hriat chian tur pakhat kan sawi dawn a ni.

Goodpasture Syndrome hi eng nge ni?

A awlsam zawngin, Goodpasture Syndrome hi a awm lo hle a, mahse nunna atana hlauhawm thei autoimmune disease a ni . "Autoimmune" tih awmzia chu kan taksa invenna system, immune system hian tihsualin kan cell leh tissue hrisel tak takte chu a rawn bei tan tihna a ni.

He natnaah hian kan immune system hian protein pakhat collagen an tih chu a bei a. Collagen hi kan taksaa building block ang mai a ni. Collagen hian kan vun, kan taksa ruh, kan ruh (tendon), kan ruh (ligament) leh kan ruh te a tichak reng a ni.

Chuvangin, Goodpasture syndrome i neih chuan i taksain protein a siam thenkhat, antibodies kan ti a, i lung leh kal chhunga collagen-ah kal la, a inzawm khawm thin. Chutiang a nih chuan chutiang hmunah chuan inflammation a intan a, chu tissue chu zawi zawiin a chhe tan ta a ni. Enkawl loh chuan hei hian kal a ti na hle (glomerulonephritis) a thlen thei a, chu chuan kal a tichhe thei a ni. Lung atanga thisen chhuak nasa tak i chhuak tan thei bawk (pulmonary hemorrhage). Thil tam takah chuan he natna avanga thihna thlentu ber chu lung atanga thisen chhuak a ni.

Hei hi a hming dang chu:

• Anti-glomerular basement membrane (anti-GBM) natna a ni
• Goodpasture natna a ni

Hei hi engtiang chiahin nge a hluar?

Hei hi natna hmuh tur awm lo tak a ni. Doctor-te sawi dan chuan kum khatah mi 100,000 zelah he natna vei thar pakhat atanga pahnih chauh an awm thin. Chuvangin a tlem zia chu i ngaihtuah thei ang.

Eng nge a lan chhuah dan?

Goodpasture syndrome hi `(pulmonary-renal)` natna a ni. Chumi awmzia chu lung leh kal (kidney) pahnih hi a rualin a nghawng tihna a ni. Vawi tam tak chu a lan chhuah hmasak ber chu lung nen a inzawm a ni. Chungte chu:

• Thâwk harsa (dyspnea): Stairs chhoh lai chauh ni lovin, chawlh lai pawhin.
• Chest pain : Thâwk emaw khuh emaw hian rilru na a awm.
• Khuh : Khuh awm reng, a chang chuan na tak tak.
• Thâwk chhuah laiin rilru aṭangin thawm a lo chhuak : Chu chu gurgling sound a ni thei a, rilru chhunga thil awm ang mai a ni.
• Ka chau hlein ka hria.`(chauhna)`: Engmah tih theih loh khawpa chak lo anga inhriatna.
• Hnar thisen chhuak (epistaxis).
• Thisen khuh : Hei hi mi tam tak tihlauhawmtu chhinchhiahna a ni a, damdawi lam pan vat a ngai a ni.
• Pallor : Thisen tlakchham vangin vun a rawn sen thin.

Kidney nena inzawm symptoms pawh a hnuah a lang thei bawk. Chung zingah chuan:

• Thisena thisen sen (red blood cells) tlahniam, chu chu thisen tlakchhamna ( anemia) tihna a ni .
• Zun chhunga thisen (hematuria): Zun chu a sen emaw, a dum emaw a lo ni thei.
• A tlangpui aia zun chhuah tlem zawk .
• Thisen sang sang .
• Nausea leh vomiting : Hei hi ei duh lohna nen pawh a thleng thei bawk.

Eng thilin nge hei hi a thlen?

Goodpasture syndrome hi kan immune system siam antibodies te hian kidney-a glomerular basement membrane (GBM)-a collagen an beih hian a awm thin. He GBM hi kan kal chhunga thisen filtering unit te tak te, glomeruli-a tel a ni. Heng antibodies te hian lung chhunga air sacs-a collagen awmte pawh an bei bawk. Hei hian lung tissue a tichhia a, thisen a chhuah tir a, thawk a ti harsa bawk.

Immune system hian a collagen a beih chhan chiang tak chu hriat a la ni lo. Environment factors leh kan genes te hi he dinhmunah hian a inrawlh thei a ni. Zirna hrang hrangah he natna vei te hian genetic marker Human Leukocyte Antigen (HLA) DR15 tih nen inzawmna nghet tak an nei tih hmuhchhuah a ni. Hei hi protein a ni a, immune system chu kan tissue leh foreign substance te thliar hrang turin a pui a ni.

A châng chuan, he natna hi hri kai hnuah a lo lang thei a, chu chu khawsik emaw, flu emaw a ni thei. Tin, nang pawhin:

• Meizial i zuk chuan .
• Cocaine ang chi ruihhlo i inhnim chuan .
• He natna vei theihna hi metal dust emaw hydrocarbon chemicals methane leh propane te i tawrh chuan a sang zawk a ni.

Hei hian tute nge nghawng nasa ber?

Goodpasture syndrome hi kum eng pawhah a awm thei a, mahse naupang zawk, kum sawm leh sawmthum inkar ah a awm tam ber a, chumi hnuah kum 60 leh 70 inkar ah a awm leh bawk.

Engtin nge diagnosis hi siam a nih?

Doctor i hmuh hunah i natna lan chhuah dan an zawt ang a, taksa enfiahna an nei ang. Chu bakah, Goodpasture syndrome finfiah nan a hnuaia test te hi an ti thei bawk:

• Thisen test : Hengte hian i estimated glomerular filtration rate (eGFR) an check thin a, hei hian i kidney te hnathawh that dan a teh thin.
• Zun test a niUrinalysis: Hei hian i zun chhunga thisen awm leh awm loh emaw protein level sang (proteinuria) a enfiah thin.
• Imaging test : Chest X-ray emaw CT scan emaw hmangin lung a chhiat leh chhiat loh enfiah thin a ni.
• Bronchoscopy : Hetiang tih danah hian doctor chuan tube te tak te, sei tak (bronchoscope) a dah a, chutah chuan camera leh light i hnar emaw i hmui emaw hmangin a dah a, chu chuan i lung chhunga chhiatna a awm leh awm loh a enfiah thin.
• Kidney biopsy : Hetah hian kal atanga tissue te tak te lakchhuah a ni a, microscope hmangin kal natna a awm leh awm loh enfiah a ni.

Goodpasture syndrome hi tihdam theih a ni em?

Ni e, hei hi natna hi dik taka hmuhchhuah a, enkawl tan thuai a nih chuan tihdam theih a ni.

Engtin nge enkawl a nih?

Goodpasture syndrome enkawl dan hi a natna nasat dan azirin a inang lo. A natna a na lutuk loh chuan doctor-te chuan damdawi an pe thei a, chungte chu:

• Corticosteroids : Entirnan, damdawi prednisone ang chi te. Hengte hian lung chhunga thisen chhuah tihtawp nan a pui thin.
• Immunosuppressant damdawi : Cyclophosphamide ang chi damdawi. Hengte hian i immune system-in a tissue a beih loh nan a titawp a ni.
• Thisen sang damdawi (antihypertensives): Hengte hian thisen sang control-naah a pui a, kal (kidney) chhiatna a ti tlem bawk.

Chu bâkah, doctor-te chuan plasmapheresis tih damdawi an hmang bawk. Hei hian i kut chhunga vein pakhat atanga thisen chu needle hmanga lak a, thisen chhunga liquid part, plasma an tih chu thisen cell atanga then hran a huam a ni. He plasma ah hian anti-GBM antibodies tha lo tak tak te hi a awm a. Chumi hnuah chuan plasma chu lakchhuah a ni a, mi hrisel atanga plasma chu i thisen cell-ah dah lehin i hnenah pek leh a ni. A awlsam zawngin i taksa atanga antibody tha lo tak tak te chu a paih chhuak thin.

Enkawl hnua dam leh theih nan eng chen nge a duh?

Immunosuppressant damdawi thla 6 atanga thla 12 vel i ei a ngai dawn a ni. Thil tam takah chuan kar engemaw zat chhung chu nitin plasmapheresis i tih a ngai mai thei.

Eng nge thleng leh ang?

Goodpasture syndrome hi enkawl loh chuan lung atanga thisen chhuak leh kal (kidney failure) nunna atana hlauhawm tak a thlen thei a ni. Amaherawhchu, natna hi hriat hmasak a, enkawl tan a nih chuan a rah chhuah a tha tlangpui. I taksa hian chutiang antibody tha lo tak takte chu hun rei lote chhung chauh a siam chhuak thin – kar tlemte atanga kum hnih vel thlengin. Relapse emaw recurrence emaw hi a tlem hle.

Hei hi a harsatna lian ber chu kal (kidney failure) a ni. Chutiang a nih chuan thisen filter turin dialysis treatment a ngai a ni.Kidney transplant a ngai a ni.

Ka dam chhung hian eng nge sawi tur ka neih?

Goodpasture syndrome vei zinga 80% vel chu kum nga chhunga dam rei zat chu 80% a ni. Chu chu natna hi hriat hmasak a, uluk taka enkawl a nih chuan a ni.

Hei hi ven theih a ni em?

A pumpelh kim theih loh mai thei. Mahse, thil engemaw i pumpelh chuan i hlauhawmna chu i tihtlem thei a ni:

• Hydrocarbons, chu chu methane, propane, gasoline, kerosene, leh tar ang chi thil tihna a ni.
• Metal leivut a ni.
• Cocaine a ei thin.
• Sam rawng chi hrang hrang.

Engtin nge ka in enkawl ang?

Goodpasture syndrome i neih chuan hengte hi tih a tha ang:

• Meizial zuk kha bansan vek rawh. Midang meizukna hmuna awm loh tur (i.e. `secondhand smoke` nena inzawmna nei lo).
• Nitin minute 30 tal taksa tihchakna nei thin ang che.
• Stress tihziaawm tum ang che .

Engtikah nge Doctor ka hmuh ang?

Thisen i khuh a, thawk harsa i tih chuan, i zun a tlem a, a chunga kan sawi ang chi dang i neih chuan doctor pan nghal tur a ni. Hriat hmasak hian i dam theihna chance nasa takin a tipung a ni. Enkawl loh chuan kal (kidney) a tichhe vek thei a, lung lama harsatna nasa tak a thlen thei bawk.

Doctor hnenah eng zawhna nge i zawh tur?

Doctor i hmuh hian heng zawhnate hi i zawt thei ang:

• Engtin nge Goodpasture Syndrome ka vei tih chiang taka ka hriat theih ang?
• Ka dinhmun hi engtiang chiahin nge a nasat?
• Eng enkawlna nge i rawt?
• Ka kal enkawl nan eng nge ka tih theih ang?
• Nephrologist nge pulmonologist ka hmuh tur?
• He natna vei te tanpui thin support group chungchang min hrilh thei ang em?

Goodpasture syndrome natna langsar ber pathum (Triad) chu engte nge ni?

Damdawi lamah chuan ``triad'' tih hian damdawi lam dinhmun sawifiahtu chhinchhiahna lian pathum a kawk a ni. Goodpasture Syndrome chhinchhiahna lian pathumte chu:

• Lung atanga thisen chhuak.
• Kidney inflammation a pung chak hle.
• Chutiang antibodies ``anti-GBM antibodies'' an tih chu taksaah a awm tih hi.

He natna neih hi a lungchhiatthlak tak zet a, mahni chauhna a thlen thei a, a bik takin a chhan dik tak hriat a la nih loh avangin. Ni tin i thiltih a tibuai thei a, metal powder, gasoline, a nih loh leh tar hmanga hna i thawh chuan hna i thlak a ngai pawh a ni thei.

A tawp berah chuan eng nge hriat reng tur

Chuvangin, Goodpasture Syndrome hi hlauh tur a ni a, mahse a natna lan chhuah dan i hriat hmasak a, doctor i pan vat a, enkawl tan i nih chuan result tha tak i hmu thei a ni. I taksaa inthlak danglamna awmte chu fimkhur rawh. Thil mak i tih chuan thil tenawm tak anga hnawl lovin damdawi lam thurawn lak a pawimawh. Nangmah chauh i ni lo va, doctor-te pawhin an pui thei che a ni.

` Goodpasture syndrome, Anti-GBM natna, pulmonary hemorrhage, kal natna, autoimmune natna, thawk harsa, zun chhunga thisen awm