Heng harsatnate hi i lungah i nei em? LAM (Lymphangioleiomyomatosis) chungchang hi han sawi ila

He natna hming sei deuh, LAM (lymphangioleiomyomatosis) tih hi i hre tawh em? A hming chu mak i ti mai thei. Mahse natna hmuh tur awm lo tak a ni a, chu chuan lung a tibuaitu a ni. Vawiin hian awlsam takin, i hriatthiam theih dan tur angin kan sawi dawn a ni. Lungngai suh, engkim kan hrilhfiah vek ang.

LAM (Lymphangioleiomyomatosis) hi eng nge ni tak tak?

A awlsam zawngin LAM hi natna hmuh tur awm lo tak a ni a, i lung chhungah tuiin a khat cyst a siam thin. Hei hian i lung a tichhe thei a ni. Tin, a chang chuan i kal leh lymphatic system-ah cyst a siam thei bawk. I taksaa smooth muscle cells te chu thunun theih lohvin a lo thang lian nghal a nih chuan eng nge thleng ang tih han ngaihtuah teh. Chu chu hetah hian a thleng thin. Genetic mutations avanga lo awm a ni.

He natna, LAM (“limf-AN-gee-oh-ly-oh-my-oh-muh-TOH-sis” tia lam tur) tia koh hian hmeichhiate a nghawng tam ber . Hmeichhia kum 20 leh 40 inkar, emaw, puitlin hun kal tawh leh menopause hnaih tawhte a nghawng tlangpui. Chumi avang chuan doctor-te chuan hormone estrogen emaw, nausen pum awmna emaw hian heng tumor lo piang tur hian inzawmna a nei thei niin an ngai a.

LAM chi hrang hrangte chu engte nge ni?

LAM hi chi hnih a awm a, chungte chu:

1. TSC-LAM: Hmeichhe thenkhat chuan an genetic condition ``Tuberous Sclerosis Complex - TSC'' an nei a. LAM an siam chhuah chuan TSC-LAM an ti a.

2. Sporadic LAM: Hei hi random takin a thleng a, chu chu a dam chhungin gene pakhatah random takin mutation a awm tihna a ni. A pawimawh ber chu he Sporadic LAM chi hi i fate hnenah i pass loh tur a ni.

He natna hi LAM an tih hi engtiang chiahin nge a hluar?

LAM hi a takah chuan natna hmuh tur awm lo tak a ni . Hmeichhia 140,000 zinga pakhat velin he natna hi an vei thei nia chhut a ni. Mahse, ``Tuberous Sclerosis`` vei hmeichhia 30% leh 80% inkar chuan LAM an nei thei.

LAM natna lan chhuah dan chu engte nge ni?

LAM vei chuan a natna lan chhuahna a nei thei a, chungte chu:

• Thâwk harsa (dyspnea) : A châng chuan hei hi zawi zawiin a pung thei.
• Wheezing hi rilru atanga whistling ri lo chhuak a ni .
• A rilru a na .
• Khuh .
• A châng chuan thisen emaw chyle (`(chyle)` - digestive system atanga lymphatic fluid) chu mucus nen a chhuak thin .

Heng symptoms i neih vang mai mai hian LAM i nei tih ngaihtuah suh, chu chu lung natna dang symptom a ni thei bawk. Chuvangin damdawi lam thurawn lak a pawimawh hle.

Engvangin nge he LAM hi a lo awm? A chhan chu eng nge ni?

LAM thlentu ber chu kan taksaa gene pahnih `(TSC1)` leh `(TSC2)` an tihte mutations a ni. Heng gene pahnih te hi tumor suppressor gene ang mai an ni a, kan taksaah hian cancer cell ang chi thil a tul lo taka lo thang lian lo turin a titawp a ni. Chuvangin, heng gene pahnih zinga pakhatah hian chhiatna a awm a nih chuan, chu smooth muscle cells ka sawi tawhte chu thunun theih lohvin an lo thang lian tan thin. Chumi avang chuan hei hi a ni:

• Lung tumor (pulmonary lymphangioleiomyomatosis) a lo awm a.
• Kidney-ah hian tumor (angiomyolipomas) a lo awm a.
• Lymphatic system ah hian cyst a lo awm thin.

Engtin nge mi pakhatin LAM a neih theih?

Miin LAM a tihhmasawn theih dan kawng hnih a awm. Pakhat chu `(TSC1)` emaw `(TSC2)` gene-a chhiatna nei nu leh pa hnen atanga gene chhia chu rochun a ni. He inheritance hian `(Tuberous Sclerosis)` natna a thlen a, chu chuan LAM vei theihna a thlen bawk. Hmeichhia `(Tuberous Sclerosis)` vei zinga 30% aia tam chuan LAM an nei.

Kawng dang chu `(TSC2)` gene-a mutation hi a takin a thleng a, hereditary connection engmah a awm lo. Hei hi a chhan dik tak hriat a la ni lo. Chutianga LAM lo thanglian chu Sporadic LAM an ti a. Hetiang a nih chuan `(Tuberous Sclerosis)` symptom dangte a lang lo.

Tute nge LAM vei theihna hlauhawm zawk?

Hei hi mak ni mah se, LAM hi hmeichhiaah a awm tam ber . Tun thleng hian mipa tlemte chauh LAM vei hmuhchhuah an la ni. Mi pakhat chauh hian sporadic LAM a nei tih hriat a ni.

Tin, naupai lai leh estrogen awmna damdawi hman lai pawhin LAM hi a na zawk thei bawk.

LAM avanga harsatna awm thei te chu engte nge ni?

LAM natna vei tam ber chu pneumothorax a ni . Balloon puak ang maiin lung atang hian boruak a luang chhuak a, lung a tikehsawm a ni. LAM vei hmeichhia zinga a chanve aia tam chuan an dam chhungin pneumothorax episode khat tal an nei dawn a ni. A châng chuan hetiang hi a thleng nawn leh thei bawk. He episode hnuah hian LAM hi hmuhchhuah a ni fo thin.

Harsatna dangte chu:

• Lung vel a chyle an tih tui (chylothorax) a awm khawm.
• Lung vel a tui awmkhawm (pleural effusion).
• Lymphatic system tihkhawtlai a ni.

LAM natna hi engtin nge an hriat theih?

LAM hriat theih nan doctor chuan a enfiah hmasa ang che a, i symptoms te a zawt ang che. Chumi hnuah chuan test leh imaging test an order ang a, a hnuaia mite hi an hre thei ang:

• I lung a thawh that zia en rawh.
• Thisen a oxygen level enfiah thin ang che.
• Lung chhunga danglamna a awm leh awm loh enfiah thin ang che.
• Thisenah hian ``protein'' thenkhat chu a tlangpui aia tam a awm em tih enfiah rawh.

LAM natna lan chhuah dan hi lung natna tlanglawn dangte nen a inang a, a chang chuan doctor-te tan hriat a harsa thei hle.

LAM hriat nan eng test nge hman thin?

Hetiang test hi i tih a ngai mai thei:

• Lung function test : Hengte hian i lung hian thawk luh leh chhuah a puih dan a enfiah thin.
• Thisen a oxygen level enfiah (`(pulse oximetry)`) : Test awlsam tak, i kut zungtang-ah clip ang chi thil i buntir thin.
• Imaging test : Chung zingah chuan CT scan (Computed Tomography scan), MRI (Magnetic Resonance Imaging), emaw HRCT (High-Resolution Computed Tomography) scan te a tel a ni. HRCT scan hian lung hmun tlemte tak tak pawh chiang takin a hmu thei a, chu chuan lung natna thenkhat hriat a awlsam phah hle.
• VEGF-D test : I doctor chuan thisen test a ti thei a, natna hrik awm leh awm loh te, i taksa peng dangte hnathawh that dan te, VEGF-D (Vascular Endothelial Growth Factor-D) tih protein i level te a enfiah thei bawk. LAM vei tam tak chuan he protein hi an nei sang hle a, mahse mi zawng zawng hian an nei vek lo.
• Lung biopsy : Hei hi lung atanga tissue te tak te la chhuakin laboratory-ah test a ni. He sample hi bronchoscopy (camera nei tube chu lung chhunga luhtir) emaw, video-assisted thoracic surgery (VATS) emaw hmangin lak theih a ni.

LAM natna hi engtin nge enkawl a nih?

Vanduaithlak takin tun dinhmunah LAM damdawi a la awm lo . Mahse, damdawi sirolimus (rapamycin tia hriat bawk, ​​brand name Rapamune®) hian natna hi a ti nghet thei a, a na zual loh nan a pui thei bawk. Sirolimus hian kidney tumor (angiomyolipomas) a ti tlem thei a, lymphatic system-a tumor leh fluid awm zat a tihtlem bakah i symptoms a tihziaawm thei bawk.

Enkawlna dang angin:

• Oxygen therapy : Thâwk harsate tan.
• Inhaled bronchodilators : Hengte hi inhaler ang mai a ni. Lung chhunga thawkna kawngte an hawng a, thawk a ti awlsam bawk.
• Pulmonary rehabilitation : Hei hi exercise, zirna leh counseling te inzawmkhawmna programme a ni.
• Lung transplant : Natna a lo zual zel tawhte tan chuan a tawp ber atan.

Enkawlna avanga side effect leh complication awm dan te pawh i zir dawn bawk ang.

Sirolimus hian a chang chuan kal a tichhe nasa thei a , natna khirh tak tak kai theihna a tipung thei bawk . Chuvangin, thutlukna siam hmain he damdawi hman thatna leh that lohna te doctor nen sawiho hmasak a pawimawh hle.

LAM nei hian eng ang hmalam hun nge a beisei theih ang?

LAM hi hun kal zelah a na zual thei. I enkawlna tur ruahmanna chu i tana tha tur enkawlna a zirin a danglam thei. I dinhmun enfiah dan tur tha ber tur chu i pulmonologist (pulmonologist) nen in thawk dun ang. I lung hnathawh dan a enfiah fo ang. I enkawlna tur ruahmanna chu:

• i kum zat.
• I menopausal status (menopause i paltlang tawh emaw, i paltlang tawh lo emaw).
• Engtiang chiahin nge lung hnathawh a tlahniam.
• I kal emaw i lymphatic system emaw a nghawng em tih.

LAM hi engtiang chiahin nge a darh chak?

LAM darh chak dan hi mi hrang hrangah a inang lo thei . Hei hi nghawng thei thil engemaw zat a awm a:

• LAM i neih chi : `(TSC-LAM)` vei te hian `(Sporadic LAM)` vei te aiin lung function tha zawk nei mahse, an pahnih ah hian lung function tlahniam rate hi a tam zawk chu a inang vek a ni.
• I kum : Mi thenkhat tan chuan LAM hi menopause hnuah a darh tawh lo a, chutih lai chuan taksaa estrogen level a tlahniam thin.
• Enkawlna i dawn dan : Damdawi Sirolimus hian mi tam takah natna hi a ti nghet thei a ni.

Engtikah nge lung transplant hi a tul?

LAM vei tam tak (64% vel) chu an hmuhchhuah hnuah kum 20 emaw a aia rei emaw chhung chu lung transplant ngai lovin an dam thei a ni. Mahse, LAM cells hi lungah a intan loh avangin lung transplant hnuah pawh cyst te hi a la lo awm thei tho a ni .

LAM nen hian engzat nge dam rei zawng? (Nun rei zawng) .

LAM nen hian eng chen nge i dam theih ang tih chu natna nasat dan leh a darh chak danah a innghat a ni. Mahse, a pum puiin tunah chuan a hma aia dinhmun a tha zawk hle . LAM vei zinga 90% aia tam chu an hmuhchhuah atanga kum 10 hnuah an dam tawh a ni.

Engtin nge ka in enkawl ang?

I pulmonologist (pulmonologist) nen eng nge i beisei tur tih leh LAM nena nun dan tur ruahmanna siam dan tur sawipui rawh. I doctor chu a hun takah hmu la, i damdawi ei tur chu a tih angin ei la, i doctor hnenah symptom thar i neih chuan, i symptoms a na zual a nih chuan, i enkawlna atanga side effect i neih chuan i doctor hnenah hrilh nghal rawh.

Engtikah nge doctor i hmuh ang? Engtikah nge emergency care-ah i kal ang?

Thâwk harsa, khuh emaw, thil dang ngaihtuahawm tak tak, a awm reng emaw, a na zual emaw pawh ni se, doctor pan ngei ngei tur a ni.

Emergency Treatment Unit (ETU) -a kal hun tur :

Lung tlakchham (pneumothorax) emaw, natna dang na tak tak emaw i neih chuan damdawi inah kal nghal rawh:

• Thâwk harsa i neih chuan.
• I rilru natna nasa tak i neih chuan .
• Thisen i khuh chuan .
• Vun, hmui leh nail te chu a sen (`(cyanosis)`) .

Doctor hnena zawh tur pawimawhte chu engte nge ni?

I doctor hnenah hetiang zawhna hi zawh hi a \angkai thei ang:

• `(Sporadic LAM)` emaw `(TSC-LAM)` emaw ka nei em?
• Eng enkawlna kawng nge ka neih?
• Engtin nge ka damdawi hi ka hman ang?
• Eng symptom thar emaw, a zual zel emaw nge ka fimkhur tur?
• Lung transplant ka mamawh dawn em?

LAM hi cancer a ni em?

LAM hi cancer anga ngaih a ni tlangpui lo . Mahse, kawng engemaw takah chuan taksa hmun khat atanga hmun danga cancer (metastatic cancer) darh ang maiin a che thin. Chumi awmzia chu cell te chu hmun khatah an intan a, thisen kalna kawng leh lymphatic system kaltlangin lung leh kal thlengin an kal tihna a ni. Hetiang a lo thang lian hi a then chuan benign cell metastasis an ti bawk.

Mahse scientist te chuan heng cell te hi khawi atanga tan nge an nih tih erawh an la hre chiang lo. Tin, heng tumor te hi cancer ang chi taksa peng dang, thin emaw thluak emaw ah pawh a darh zau lo niin a lang bawk. Microscope hmanga en chuan heng cell te hi cancer cell aiin cell pangngai ang deuhin an lang.

Enkawlna thar leh LAM hriatthiamna a san avangin tunah chuan lung thlak ngai lovin mi an dam rei tawh hle. Mahse he nunphung thara insiamrem tur hian hun a duh a ni. Nun chhung zawnga natna chungchang hriat hian i ke hnuai atanga lei chu a hrual chhuak ang maiin a lang thei. Mahse i medical team – leh i personal support team te hian i kea ding leh turin an pui thei che a ni.

A tawp berah chuan eng nge hriat reng tur

LAM hi natna khirh tak a ni thei a, mahse nangmah chauh i ni lo. Medical science lama hmasawnna a awm tawh avangin he natna hi enkawl dan tur tam tak a awm a. A pawimawh ber chu positive taka awm a, doctor thurawn zawm a, beiseina bansan loh hi a ni. I doctor leh i hmangaih tak takte nen i ngaihtuahna leh hlauhthawnna te sawipui rawh. He zinkawngah hian tanpui thei tur che mi tam tak an awm tih hre reng ang che.

` LAM, lung natna, natna hmuh tur awm lo, thawk harsa, sirolimus, lung transplant, genetic mutations