Pulmonary Alveolar Proteinosis (PAP) chungchang hi i zir mai ang u, he natna hian lung chhunga boruak bawm a tikhawlo thin.

A chang chuan thawk harsatna mai mai, i rilru a khauh ang maiin i inhria em? Rei vak lo i kal hnuah pawh i chau tih i hria em? Heng thilte hi kan theihnghilh fo thin a, hahdamna pangngai mai mai niin kan ngai thin. Mahse, vawiinah hian damdawi lama harsatna lian tak, heng symptoms hnuaia awm thei, a tlem hle nachungin kan sawi dawn a ni. Chu chu Pulmonary Alveolar Proteinosis, a tawi zawngin PAP a ni.

A awlsam zawngin, Pulmonary Alveolar Proteinosis (PAP) hi eng nge ni?

Kan lung chu boruak bawm te tak te maktaduai tam takin a khat ang tih han ngaihtuah teh. Heng air sacs te te hi alveoli kan ti thin. Kan thawh hian heng air sacs hmang hian oxygen kan la lut thin.

Tunah chuan PAP-ah chuan thil thleng chu heng boruak bawm te tak te chhungah hian protein, thau leh thil dangte chu bawlhhlawh ang maiin a awm khawm a ni. A chhan chu heng air sacs bang chhunga awm, oil ang chi surfactant an tih, a pianphung anga awm, tifai tu cell te hian hna an thawk tha lo a ni. Tichuan he surfactant hian a khawlkhawm a, air sacs te chu a block ta a ni. Chuvangin, boruak a lut tha thei lo va, chuvangin thisen hian a mamawh oxygen a hmu lo. Hei hian thawk harsatna a thlen thin.

PAP chi hrang hrang lian ber ber a awm em?

Ni e, PAP hi chi thum-ah then a ni ber. A chi hrang hrang chhan hi a inang lo.

Tute nge he natna vei theihna sang zawk?

Autoimmune leh secondary PAP hian kum 30 leh 60 inkar mi a nghawng tlangpui a, heng chi hnih hi mipaah ​​a tam zawk . Meizial zu leh leivut emaw, chemical emaw nena inhnim hnawih te hi an hlauhawm zawk.

Congenital PAP hi naupang kum 10 hnuai lam ah a tam ber a, mahse kum eng pawhah a awm thei.

PAP natna lan chhuah dan chu engte nge ni?

He natna lan chhuah dan ber leh langsar ber chu thawk hah (dyspnea) . Mi tam zawk chuan exercise emaw hnathawh lai emaw hian thawk hahna an nei thin. Mahse, mi ṭhenkhat chuan an ding reng lai pawhin he harsatna hi an tawk thei a ni.

Chu bakah, heng symptoms te hi i hmu thei bawk:

• A rilru a na
• Khuh (phlegm emaw thisen emaw nen pawh a ni thei) .
• Vun leh nail blue discoloration (cyanosis) - Hei hi thisenah oxygen a awm tlem chuan a thleng thin.
• Chauhna awm reng
• Khawsik
• Lung infection awm fo thin
• Kut zungtang clubbed tak tak te
• Chhan awm lovin taksa rihna tihtlem

He natna hi engtin nge doctor hian a hriat theih ang?

PAP natna i vei nia i rinhlelh chuan i doctor chuan a enfiah hmasa ang che a, i lungte chu stethoscope hmangin a ngaithla ang che. Tichuan i chhungkaw medical history leh i nunphung chungchang zawhna an zawt ang che. Meizial i zu a, leivut emaw chemical emaw i tawk a nih chuan i doctor hnenah i hrilh ngei ang .

Tichuan, natna hriat chian nan test eng emaw zat tih theih a ni:

• Thisen test: Thisen a oxygen level enfiah thin. Tin, PAP natna thlen chhan hriat nan GM-CSF autoantibody ang chi test bik te pawh tih theih a ni bawk.
• Pulmonary function testing: I lung hnathawh that dan tehna a ni.
• Chest X-ray emaw CT scan emaw: Chuap dinhmun chiang taka hmuh theih nan.
• Bronchoscopy hmanga enfiah a ni:Camera dahna tube te tak te chu anesthesia hmangin ka chhungah dahin thawkna kawng leh lung chhung lam enfiah a ni.
• Lung biopsy: Bronchoscopy neih lai emaw, surgery neih lai emawa enfiah turin lung atanga tissue te tak te lak chhuah a ni.

PAP enkawlna tur engte nge ni?

Tunah hian PAP hi damdawi a la awm lo a, mahse i natna lan chhuah dan tur enkawl thei tur leh nun tha zawk nei thei tur enkawlna tha tak tak a awm a ni.

PAP enkawlna ber leh hman tlanglawn ber chu "Whole-Lung Lavage" (WLL) a ni.

He lung lavage hi eng nge ni?

Hemi kawngah hian thil thleng chu doctor chuan bronchoscope (camera tube) hmangin lung chu saline (sterilized salt water) hmangin pakhat khatin a silfai a, chu chuan protein leh thau tangte chu a paih chhuak thin. Hemi hnu hian thawk a awlsam phah hle.

He enkawlna hi kan tih hian:

• Anesthetized vek i ni dawn a, chuvang chuan engmah i hre lo ang.
• Doctor chuan bronchoscope chu ka chhungah a thun a, lung pakhatah a kawhhmuh a.
• Oxygen chu lung dangah machine hmangin a pe chhuak a.
• A chang chuan i rilruah vibrating vest i bun thin. He vibration hian i air sacs-a thil awmte chu a tizau a ni.
• Tichuan saline solution chu lung chhungah an inject a, an lakchhuah chuan thil lo awm tawh nen a rawn chhuak ta a ni.
• Lung dang pawh chu a rualin, a nih loh leh ni engemaw zat hnuah silfai a ni mai thei.

Enkawlna dang

• Bronchodilators: Heng damdawi te hian thawkna kawng vel muscle te a ti hahdam a, thawk a ti awlsam hle.
• GM-CSF replacement therapy: Hei hi nebulizer hmanga inhnim emaw, injection anga pek emaw a ni. Thlawhna kawng tifaitu cell te hnathawh a titha a ni.
• Lung transplant: Mite natna khirh tak tak leh an lung a chhiat tawhte chuan a hnuhnung ber atan lung transplant hrisel tak an mamawh mai thei.
• Plasmapheresis leh plasma exchange: Hei hian thisen a liquid part (plasma) chu lakchhuah a, mi hrisel atanga plasma hmanga thlak a ni. Autoimmune PAP-ah chuan hei hian immune system-in cell hrisel a beih loh nan a pui a ni.
• Supplemental oxygen: Oxygen hi mask emaw, hnar chhunga tube te tak te dahin thawk a awlsam zawk nan pek a ni.

PAP vei ka nih angin eng nge ka tih theih ang?

PAP i neih chuan i doctor-in a pek dan zawm a pawimawh hle a, chubakah i nunphung pawh enkawl a pawimawh hle bawk.

• Meizial zu hi pumpelh vek la, midangte zukna hmuna awm loh tur.
• I doctor-in a rawt angin influenza (flu) leh pneumonia vaccine hi la rawh.
• Damlote lakah inthiarfihlim rawh.
• Ei leh in tha tak ei la, i taksa rihna control rawh.
• Toilet i hman zawh hnu leh ei hma leh ei zawhah i kut te uluk takin silfai thin ang che.
• I doctor nen inbia la, i duh ang exercise plan him tak siam rawh.