Hetiang harsatna hi i lungah i nei tawh em? (Pulmonary Alveolar Proteinosis - PAP) Hemi chungchang hi han sawi ila!

Thâwk a harsa deuh tih i hria em? Nge i chau mai mai em ni? A chang chuan heng thilte hi thil pangngai anga ngaih a ni a, mahse a tlem berah chuan lung natna chhinchhiahna a ni thei. Vawiin hian lung natna hmuh tur awm lo tak, mahse pawimawh tak, hriat tur pawimawh tak chungchang kan sawi dawn a ni. Hei hi `(Pulmonary Alveolar Proteinosis)` emaw `(PAP)` emaw an ti a. A hming hi a buaithlak deuhin a lang thei a, mahse awlsam takin han sawifiah ila.

`(Pulmonary Alveolar Proteinosis - PAP)` tih hi eng nge ni? A awlsam zawngin...

Okay, tunah chuan he `(PAP)` tia koh condition hi eng nge a nih kan en ang. A awlsam zawngin sawi ila, kan lung chhunga boruak bawm te tak te (alveoli)-ah protein, thau leh thil dang thenkhat a lo awm khawm a, a tikhawlo a nih chuan natna hmuh tur awm lo tak a ni. Kan lungphu hi boruak bawm te tak te maktaduai tam tak atanga siam a ni tih han ngaihtuah teh. Heng air sac te te hi `alveoli` kan tih hi an ni . Kan thawk chhuah hian heng air sacs hmang hian oxygen hi a lut a, thisenah a chhuak thin.

Chuvangin, `(PAP)` chungchangah chuan heng air sacs te hi thil tul lo takin a khat a, chu chu drain khar ang maiin a khat a, oxygen chu thisen a thlen theihna tur kawng a tikhawlo a ni. Chuti a nih chuan eng nge thleng? Kan thisen hian oxygen a hmu tlem hle. Hei hian thawk harsatna (`dyspnea`) a thlen thei a ni.

Hei hi lung natna khirh tak a ni. Chuvangin hetiang diagnosis i dawn hian hlauhna tlem i neih hi thil pangngai a ni. Mahse, lungngai suh. Enkawlna mumal tak a awm chuan natna leh a lan chhuah dan te i thunun thei a ni. `(PAP)` i neih chuan pulmonary medicine doctor pan a, enkawl a pawimawh hle. Tin, nunphung hrisel tak i tihdanglam theih dan tur pawh a fuih ang che. Chûngte chuan i nunphung a ti ṭha thei a ni.

He `(PAP)` natna chi hrang hrang a awm em?

Ni e, `(PAP)` natna hi chi thum a awm a. Eng nge an nih i en ang u:

1. Autoimmune PAP: Hei hi PAP chi khat a ni ber. Puitling 90% velin he chi hi an vei thin. Heta thil thleng hi kan taksa ngeia immune system hian a tihsualin thawkna kawng thianghlimna tura puitu cells te chu a bei sual a ni. Kan invenna (defense) hian kan cell te a do tih angin ngaihtuah rawh.

2. Secondary PAP: Hetiang chi hi kan thawkna kawng chhunga cell hnathawh a nghawng a, natna dang vang emaw, thil tha lo (e.g. leivut, chemical) kan tawrh vang emaw a ni. Chu chu chhan dang vangin a thleng tihna a ni.

3. Congenital PAP: Hei hi pian tirh atanga awm thin a ni. A châng chuan nau piangah natna lan chhuahna a awm thei a, kum hrang hrangah pawh a lang thei bawk. Hei hi kan lung chhunga cell hnathawh control tu gene thenkhat inthlak danglam vang a ni. Hei hi nu leh pa atanga fate rochun theih a ni.

He `(PAP)` natna hi tuin nge vei tam zawk?

A tlangpuiin a nghawng tam ber chi hnih chu ``Autoimmune PAP'' leh ``Secondary PAP'' te an ni.Kum 30 leh 60 inkar tan.Tin, heng chi hnih hi mipaah ​​a tam zawk . Meizial zu thinte leh leivut emaw chemical chi hrang hranga inhnamhnawih te hian he natna hi an vei theihna a sang zawk a ni.

`(Congenital PAP)` an tih chi hi naupang kum 10 hnuai lam ah hmuh tam ber a ni a, mahse, a hmaa kan sawi tawh ang khan kum eng pawhah a awm thei.

PAP natna hi engtiang chiahin nge a hluar?

Dik tak chuan `(PAP)` hi natna hmuh tur awm lo tak a ni. Khawvel pumah he natna hian mi maktaduai thum zinga sawmli vel a vei a ni. Chumi awmzia chu Sri Lanka ramah hian he natna vei hi an tlem hle mai thei tihna a ni.

Engvangin nge he `(PAP)` natna hi a lo awm? Eng nge a chhan?

Kan sawi tawh ang khan kan air sacs (alveoli) bang hi a thim hle. Heng bang te tak te hmang hian oxygen chu lung atanga thisenah a kal lut thin. Heng air sac te bang hian a pianphungah oil layer a nei a , chu chu surfactant an ti a ni . Heng surfactant te hian air sacs te chu a tikhawlo lova hawn reng turin a pui thin. Chutah chuan oxygen chu awlsam takin thisenah a lut thei a ni.

Tunah chuan kan lung chhungah hian cell chi khat bik `alveolar macrophages` tih a awm ta a ni. Heng cell te hi tifai hnathawk ang mai an ni. An tih tur chu heng `(surfactants)` te hi air sacs atang hian a hun hunah an paih chhuak a, an inkharkhip loh nan an veng thin.

Mahse, PAP vei chunga thil thleng chu heng macrophage an tih cell te hian air sacs bang te uluk taka tifai turin signal an hmu lo. Chuti a nih chuan eng nge thleng? Chu surfactants te chu an pungkhawm a, air sacs te chu an block ta a ni. Sink chhunga drain khar ang maiin. Tichuan thisenah oxygen a thlen theihna tur kawng chu a inkhar ta a ni.

A awlsam zawngin sawi ila, he dinhmun hi lungphu "trash cleaning system" chak lohna vang a ni.

`(PAP)` natna lan chhuah dan chu engte nge ni?

PAP chhinchhiahna ber leh langsar ber chu thawk hah (dyspnea) a ni. Mi tam zawkin exercise an tih laiin thawk hah an tawk laiin, mi thenkhat chuan an ding reng lai pawhin he harsatna hi an tawk thei a ni.

Tin, a lan chhuah dan dang engemaw zat a awm bawk:

• A rilru a na tih hriat.
• Khuh. A chang chuan mucus nen hian thisen tlem a chhuak thei bawk.
• Vun leh nail-a blue discoloration (cyanosis) (hei hi thisenah oxygen a awm loh vang a ni).
• Chau tih hriat reng (chau lutuk).
• Khawsik.
• Lung infection awm fo thin.
• Kut zungtang tip chu a lian a, drum ang deuh (`clubbed fingers`) a lo awm ta a ni.
• Chhan awm lovin taksa rihna tihtlem.

Heng symptoms pakhat emaw a aia tam emaw i neih chuan doctor thurawn lak a pawimawh hle.

PAP natna hian thihna a thlen thei em?

A nasat chuan PAP hian nunna atana hlauhawm tak thawkna lama harsatna a thlen thei a ni.Chumi awmzia chu lungte hian an hna an thawk tha thei lo tihna a ni. Amaherawhchu, he natna awm dan hi mi hrang hrangah a inang lo. Mi thenkhat chu enkawl ngai (`spontaneous remission`) nei lovin an dam thei a ni. Midang tan chuan natna hi a level ang chiah hian a awm reng a ni. A na zawkah chuan thawk lama harsatna emaw, natna dang emaw avanga thihna a thleng thei a ni. Chuvang chuan natna hi hriat hmasak a, enkawlna lak a pawimawh hle.

Engtin nge PAP hi hmuhchhuah a nih? Eng test nge tih thin?

Doctor-in PAP i vei nia a rinhlelh chuan a enfiah hmasa ang che a, i lung a ngaithla ang. Tin, i mimal leh chhungkaw medical history leh i nunphung chungchang zawhna a zawt bawk ang che. Meizial i in emaw, leivut emaw chemical emaw i tawk a nih chuan doctor hnenah hrilh ngei ang che.

PAP nemnghehna atana test-naah hian:

• Thisen test: I thisena oxygen awm zat enfiah thin ang che.
• Thisen test bik: `(granulocyte-macrophage colony-stimulating factor - GM-CSF) autoantibody` tih hming pu indicator zawng rawh. Hei hian `(PAP)` natna thlentu zawn chhuah nan a pui a ni.
• Pulmonary function testing: I lung hnathawh that dan teh rawh.
• Imaging exam: Heng zingah hian chest X-ray emaw CT scan emaw a tel. Chungte chuan lung dinhmun chiang takin a tarlang thei a ni.
• Bronchoscopy: Hetah hian tube te tak te (camera nei) chu ka kaltlangin thawkna kawngah dahin, lung chhung lam chu enfiah a ni.
• Lung biopsy: Lung atanga tissue te tak te chu enfiah nan lak chhuah a ni a, chu chu bronchoscopy neih lai emaw, operation neih lai emaw a ni.

Heng test te hi doctor ten PAP dik taka an hriat theihna tur a ni.

Natna `(PAP)` tihdamna famkim a awm em?

Tun thlengin PAP damdawi hi a la awm lo. Mahse, lungngai suh. I natna lan chhuah dan thunun thei tur leh nun tha zawk nei thei tur enkawlna tam tak a awm a.

PAP natna hi engtin nge enkawl a nih?

PAP enkawlna ber leh hman tlanglawn ber chu Whole-Lung Lavage (WLL) a ni. Hei hi "lung lavage" an ti bawk. I lung sil ang maiin ngaihtuah rawh.

Doctor chuan bronchoscope (camera nei tube te tak te) leh saline solution (sterile salt water) hmangin lung pakhat chu a khatin a tifai thin. Hei hian i thawk a ti awlsam ang. Hetiang hian darkar li atanga darkar nga vel a awh thei a ni. I natna lan chhuah dan nasat dan azirin thla engemaw zat danah emaw, kum khatah vawi khat emaw WLL i neih a ngai mai thei. Mahse, PAP vei thenkhat chuan WLL an mamawh lo mai thei. Hei hi i dinhmun nasat danah a innghat a ni.

`(Whole-Lung Lavage - WLL)` i tih hian eng nge thleng?

• General anesthesia emaw sedatives emaw pek i ni ang.
• Doctor chuan i hmui kaltlangin i thawkna kawngah bronchoscope a thun a, i lung pakhatah a kawhhmuh a.
• Machine pakhat hian lung dang hnenah oxygen a pe chhuak thin.
• I rilruah vibrating vest i ha a. He vibration hian air sacs-a tang surfactant te chu a tizau a ni.
• Chumi hnuah doctor chuan saline solution chu lung chhungah a inject a, surfactant te nen chuan a chhuah leh (suctions) a ni.
• A chang chuan lung dang chu a rualin silfai theih a ni a, a nih loh leh ni rei lo te chhung silfai theih a ni bawk.

He `(WLL)` enkawlna bakah hian enkawl dan dang a awm a:

• Bronchodilators: Heng damdawi te hian i thawkna kawng vel muscle te chu a ti hahdam a, thawk a awlsam phah a ni.
• Granulocyte-macrophage colony-stimulating factor (GM-CSF) replacement therapy: Hei hi nebulizer (damdawi tui tak chu vapor-a chantirna hmanrua) hmanga inhalation emaw, injection angin emaw pek a ni. He enkawlna hian thawkna kawng tifaitu cell (macrophages) te hnathawh thatna hmangin hna a thawk a ni.
• Lung transplant: PAP natna na tak leh lung chhiatna nei te chuan lung transplant an mamawh fo thin. Hetiang tihnaah hian i lung natna pakhat emaw, pahnih emaw chu lakchhuah a ni a, donor hrisel tak atanga lung hmanga thlak a ni.
• Plasmapheresis leh plasma exchange: Hei hian i thisen a liquid part, plasma an tih chu la chhuakin, chu chu thisen petu hrisel tak plasma hmanga thlak a ni. Autoimmune PAP vei tan chuan he enkawlna hian immune system-in cell hrisel a beih loh nan a pui a ni.
• Supplemental oxygen: Oxygen therapy hian thawk a ti awlsam thei hle. I hmaiah mask hmangin emaw, i hnar chhunga tube te tak te dah hmangin emaw pek theih a ni.
• Clinical trials: Enkawlna thar zirchiannaah pawh tel theihna hun i nei thei bawk. Hemi chungchang hi i doctor nen sawiho rawh.

PAP natna hi ven theih a ni em?

Kan sawi tawh ang khan PAP chi hrang hrang a awm a. Chuvangin, Autoimmune PAP emaw Congenital PAP emaw pawh hi kan taksa chhunga thil kalphung nena inzawm a nih avangin kan veng thei lo.

Mahse, eng emaw chen chu `(Secondary PAP)` hi ven theih a ni. Engtin nge chutiang chu i tih theih? A theih ang anga toxic substance i exposure tihtlem tur a ni . Chu chu a theih loh chuan mask tha tak (`respirator` emaw, facemask insiam tha tak) i hmui leh hnar tha taka khuh tur i hmang tur a ni.

PAP vei tan chuan eng nge a hmalam hun?

Inenkawlna mumal tak `(Whole-Lung Lavage - WLL)` te hian thawk hahdamna leh natna lanchhuahna dangte a tiziaawm thei a ni. Mi tlemte chauh hi `(PAP)` atanga enkawl dam loin an dam chhuak thin. Mahse, enkawl loh chuan `(PAP)` natna khirh tak takte chuan thawk lama harsatna emaw, thihna emaw a thlen thei a ni. Chuvang chuan doctor thupek zawm a pawimawh hle.

PAP ka neih chuan keimah tan eng nge ka tih theih ang?

PAP i neih chuan i hriselna vawng reng turin thil engemaw zat i tih theih a awm a:

• PAP enkawlna atana clinical trial chungchang i doctor zawt rawh.
• Meizial zu suh. Tin, mi dangin an zuk laiin secondhand smoke hi thawk suh .
• Flu leh pneumonia shot lak dan tur chungchanga doctor thupek zawm rawh.
• Damlote lakah inthiarfihlim rawh.
• Ei leh in tha tak ei rawh. I taksa rih zawng control rawh.
• Toilet i hman zawh hnuah, chaw ei hma leh ei zawhah i kut te uluk takin silfai thin ang che.
• I doctor nen thawk ho la, i tana tha tur exercise plan him tak siam rawh.

Pulmonary Alveolar Proteinosis (PAP) hi lung natna hmuh tur awm lo tak a ni tih hre reng ang che, chu chuan thawk harsa leh chhinchhiahna dang a thlen thei a, chu chuan lung chhunga boruak bawm a tikhawlo thei a ni. Mahse, PAP nghawng thunun thei tur enkawlna tha tak tak engemaw zat a awm. Hetiang dinhmun hi i neih chuan multispecialty team nena thawh dun hi ngaihtuah la. An pahnih hian i nunphung tihchangtlunna kawngah an pui thei a ni.

Take-Home Message (Hriat reng tur pawimawh ber) .

Okay, chuvangin `(Pulmonary Alveolar Proteinosis - PAP)` chungchang hi kan sawi nasa hle. Hei hi a buaithlak deuh niin a lang a, mahse hriat reng tur ber chu hei hi natna hmuh tur awm lo tak ni mahse, hlauthawng lo tur leh damdawi lam thurawn leh enkawlna dik tak lak a pawimawh hle.

• Thâwk harsa, khuh reng emaw, hah fo emaw ang chi symptom i neih chuan doctor pan rawh.
• `(PAP)` i vei tih hmuhchhuah a nih chuan pulmonologist enkawlna hnuaiah enkawlna zawng rawh .
• Enkawlna a awm a, chuvangin beiseina bansan suh. `(Whole-Lung Lavage - WLL)` ang enkawlna hian hahdamna nasa tak a pe thei a ni.
• Damdawi lam thurawn dik tak zawm leh nun hrisel taka nun hian natna thunun kawngah kawng thui tak a zawh a ni.

Hemi chungchanga zawhna dang i neih chuan doctor nen inbiakna neih hreh suh. Hrisel takin awm rawh!

` Pulmonary Alveolar Proteinosis, PAP, Lung Disease, Thâwk harsa, Alveoli, Surfactants, Whole-Lung Lavage