I fa hian a rilru chhunga lymph vessels-ah harsatna a nei em? (Primary Intestinal Lymphangiectasia) Hemi chungchang hi han sawi ila!

I fate ke leh kut chu a chang chuan a hring chhuak mai mai tih i hmu ngai em? A nih loh leh a pum na vangin a phunnawi fo em? A taksa rihna a tihniam loh chuan a rihna a tlahniam em? Hengte hi kan ngaihven vak loh symptom pangngai a ni tih i ngaihtuah mai thei. Mahse, hengte hnuaiah hian ‘Primary Intestinal Lymphangiectasia’ (PIL) tih hi a awm thei a, mahse ngaihtuahawm tak a ni. A hming hi a hlauhawm deuhin a ri a ni lawm ni? Mahse, lungngai suh. Hemi chungchang hi vawiin hian chipchiar tak leh awlsam takin sawi ila.

‘Primary Intestinal Lymphangiectasia’ tih hi eng nge ni?

A awlsam zawngin sawi ila, hei hi kan fate chaw ei kawng, a bik takin small intestine-a awm, natna awm lo tak a ni. I hria em, kan ei tur tam zawk hi chu a digested a, a chhunga nutrient awmte chu he small intestine-ah hian taksaah a lut vek a ni.

Tunah chuan he (PIL) dinhmunah hian i fate intestine-a ‘lymph vessels’ an tih tube system te tak te chu a chhe tawh a, hna a thawk tha lo hle. Heng lymph vessels te hi kan taksa pum puia darh chhuak channel network tha tak tak an ni. Kan taksaah ‘pipe system’ dang ang mai a ni. An hnathawh ber chu kan tissue-a tui leh protein awmkhawm (he tui hi ‘lymph’ kan ti) te khawlkhawm a, circulatory system-a thawn kir leh hi a ni.

Mahse PIL vei naupangah chuan heng lymph vessel te hi a lo hring a, ‘leak’ a ni. Thil thleng chu heng lymph vessels atanga nutrients leh proteins te chu taksaah an lakluh ai chuan intestines ah an leak leh thin. Pipe leak atanga tui chhuak ang mai a ni.

Hetianga thil a leak hian harsatna lian tak pahnih a lo chhuak a, chungte chu:

1. Protein level hniam: Protein hi taksaa hna pawimawh tak thawktu a ni. Chuvangin hengte hi intestine-a an leak chuan taksain protein a hmu tlem hle. Hei hian taksa pum a ti na thei a ni. He natna hi damdawi lam hawi chuan ‘protein-losing enteropathy’ kan ti bawk.

2. Nutritional deficiencies: Chu tui leak rual hian vitamin leh mineral pawimawh tak tak te pawh a bo vek bawk. Hei hi uluk taka enkawl a nih loh chuan naupangin ei leh in lama harsatna chi hrang hrang a nei thei a ni.

(PIL) hian naupang tê leh tleirawl a nghawng tam ber. Kum 3 an tlin hmaa hriat chhuah a ni tlangpui a, mahse, a chang chuan puitling leh nausen pum chhunga nausen thlengin a nghawng thei a ni. Damdawi a la awm lo. Mahse, doctor-te chuan special diet plan leh damdawi hmangin symptoms control-in leakage leh swelling an tihziaawm thei a ni. Tin, taksain a mamawh vitamin leh mineral a pe thei bawk.

He (PIL) dinhmun hi hming dang engemaw zatin an ko a:

• Intestinal lymphangiectasia a awm thin
• Lymphangiectasia a awm thin
• Waldmann natna a ni

He (PIL) natna lan chhuah dan hi engte nge ni?

PIL chhinchhiahna ber leh tlanglawn ber chu tui awm vanga hring (swelling) hi a ni. Doctor te chuan hei hi lymphedema an ti a . Hei hi i fa ke leh keah i hmu hmasa mai thei. He tui hi a lo pun zel chuan i fa hmai, kut leh serh hmun thlengin a lo hring tan thei a ni.

Ngaihtuah teh, i fa chu zîngah a harh chuan an ke chu a pangngai reng a, mahse ni a lo kal zel a, tlai lamah chuan an ke chu a lo hring tawh a, pheikhawk pawh an bun thei tawh lo. Chu chu he thubuaiah hian a thleng thin.

Chu bâkah, naupang chuan a thinlung vêlah tui ( pericarditis tia koh), a rilruah tui (pleural effusion tia koh ), a nih loh leh a pum chhunga tui ( ascites emaw, abdominal effusion tia koh) emaw a nei thei bawk.

Symptom dang thenkhat chu hetiang hi a ni:

• Pum natna
• Nausea leh luak chhuak
• Diarrhea (ril a na ) .
• Chauhna awm reng (Fatigue) .
• Rihna tihtlem
• Chaw engzat pawh ei mah ila taksa rihna tihpun loh (hei hi taksain nutrients a hip dik loh vang a ni a, chu chu ‘malabsorption’ an ti a ni ) .

I fa hian heng symptoms pakhat emaw a aia tam emaw a neih chuan doctor thurawn lak a tha ber.

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(PIL) dinhmun hian harsatna lian tham a thlen em?

Ni e, a tlem hle nachungin, (PIL) hian naupang taksa pum pui a ti na lutuk thei a ni. Hei hi ‘anasarca’ an ti a ni. Hei hi natna khirh tak a ni a, nunna atana hlauhawm pawh a ni thei. Chuvang chuan a natna lan chhuah dan fimkhur a pawimawh hle.

Hetiang (PIL) dinhmun hi eng nge a chhan?

Hetiang dinhmun (PIL) hi naute lymphatic channels a dik lo vang a ni. Heng lymphatic channels te hi a hmaa kan sawi tawh ang khan naute intestine te atanga thau ang chi thil te chu liver leh taksa peng dang thlenga phur thei tube system a ni.

He thil dik lo hian lymph vessels a tikhawlo thin. Tichuan, lymph fluid an khat a, thisen kalna kawngte chu a lo hring a, a tawpah chuan an puak a, chu thisen kalna chhunga thil awmte chu naupang intestine lian (colon)-ah a khawl khawm a ni.

Mahse, damdawi lam thiamte chuan he natna hi engvangin nge a awm tih chiang takin an la hre lo. Chu chu buaina chu a ni.

Hei hi genetic a ni em? (Intestinal lymphangiectasia hi genetic a ni em?)

A tlem hle a, chhungkaw khata mi eng emaw zat chu he natna hian a nghawng tih report a awm fo. Mahse, zirchiangtute chuan hei hi genetic condition a ni em tih erawh an la chiang lo. Hemi chungchangah hian zirchianna tam zawk neih mek a ni.

He (PIL) natna hi engtin nge an hriat theih? (Diagnosis) tih a ni.

A tam zawkah chuan doctor-in a tih hmasak ber tur chu i naute taksa a enfiah a, i naute natna lan chhuah dan a zawt che a ni. A châng chuan, naute chu nausen pum chhunga a la awm laiin prenatal ultrasound , tih hian naute keah a hring (edema) a lantir thei a, chu chuan eng thil nge thleng tih hriatna engemaw chen a pe thei che a ni.

Naute a pian hnuah doctor chuan PIL a awm leh awm loh a enfiah ang a, chu chu naute chhungril lam enfiah nan hmanraw bik endoscope an tih hmangin a enfiah ang. Hei hi flexible tube a ni a, camera a inbun a ni. He test neih chhung hian naute chu anesthesia hnuaiah muttir a ni ang a, chuvangin natna an nei lo ang. Chuvangin lungngai suh.

He endoscopy neih chhung hian doctor chuan naupang rilru chhung hmun engemaw zat atang hian tissue te te ( biopsy tia koh) a la a. Tichuan, pathologist , tissue enfiahna lama tui tak doctor chuan heng tissue sample te hi microscope hmangin a en a, lymph vessels-ah hian a hring chhuak dik lo em tih a enfiah thin.

Tin, thisen test hian taksaah protein engzat nge awm tih a hriat theih bawk. Stool sample lak theih a ni bawk a, lymph node atanga tui chu small intestine-ah a luang lut em tih enfiah theih a ni bawk.

(PIL) enkawlna tha ber berte chu engte nge ni?

PIL enkawl dawnin a pawimawh ber chu naupang ei leh in thlak danglam hi a ni. Hei hi PIL laka ralthuam ber a ni. Nutrient leakage tihtawp nan ‘plug’ dah ang maiin han ngaihtuah teh.

Doctor tam tak chuan thau tlem, protein tamna ei tur an pe thin.

Thau hi a hmin a harsa deuh. Chuvangin i naute leaky gut tan chuan thau hi a absorb tha a harsa hle. Chuvangin i ei leh in tharah hian thau chi khat bik ‘medium-chain triglycerides (MCTs)’ tih chu a tel thei a ni. Heng (MCT) te hi thau chi khat, taksain awlsam taka a lakluh theih a ni. Tin, i ei leh inah protein tam zawk dah hian i taksain nutrient a lakluh zat a tipung thei bawk.

Nutrient thenkhat a bo avangin doctor chuan naupang taksain a mamawh a hmuh theih nan vitamin leh mineral supplement te pawh a rawt thei bawk.

Chu bakah, doctor chuan (PIL) natna kawng hrang hranga pui turin heng ang damdawi hi a pe thei bawk:

• Diuretics (water pills tia koh bawk): Hengte hian protein leakage avanga taksaa extra fluid awm zat tihtlem nan hna an thawk a ni.
• Somatostatin analogs (octreotide ang chi): Heng damdawi te hian lymphatic system atanga thil lo chhuak tihziaawmna turin a pui thin.

Enkawlna dang: 1.1.

• Surgery: Enkawlna dangin a puih loh chuan, leh a tlem hle a nih chuan doctor-te chuan i fate rilru natna hmun chu paih chhuah nan operation neih a rawt mai thei.
• Enkawlna dang: Hemi chungchangah hian zirchianna neih chhunzawm zel a ni. Damdawi thar eng emaw zat, sirolimus te chu PIL test mek a ni. Chûngte chu ṭhenkhatah chuan a ṭangkai thei a, mahse hman lar a la ni lo.

(PIL) dinhmun tur prognosis chu eng nge ni?

Sawi tawh ang khan (PIL) hi natna tihdam theih kim a ni lo. Amaherawhchu, naupang thisena protein level hi enkawl tan atanga kar tlemte chhungin a pung tur a ni. Mahse, naupang chuan a dam chhung zawngin thau tlem ei tur a ei a ngai dawn a, nutritional supplement a ei a ngai dawn a ni.

Hei hi harsatna ni mah se, uluk taka enkawl a nih chuan naupang chuan nun pangngai a nei thei a ni. A pawimawh ber chu Doctor thupek zawm a, naupang ei leh in tur enkawl hi a ni.

(PIL) dinhmun hi ven theih a ni em?

He (PIL) natna thlentu hi zirchiangtute chuan an la hriat loh avangin tunah hian he natna hi ven dan tur a awm lo.

Engtikah nge ka fa hian doctor a hmuh ang?

I fa ke, ke, a chunga kan sawi tak PIL chhinchhiahna eng emaw zatah a hring tih i hriat chuan i fa chu doctor hnenah hruai nghal mai a tha ber. A hmaa hriat chhuah a nih chuan enkawl tan thuai theih a ni.

Ka fa doctor hnenah eng zawhna nge ka zawh ang?

I fain PIL a neih chuan i doctor zawh tur zawhna tam tak i nei thei. Chung zinga ṭhenkhat chu hetiang hi a ni:

• He (PIL) natna atan hian eng diet nge a tha ber?
• Ka fate hi eng supplement nge ka pek ang?
• Ka fa natna lan chhuah dan tur enkawl that zawk nan eng nge ka tih belh theih ang?
• He (PIL) dinhmun hi a pawi em?
• Eng complication nge ka fimkhur tur?

Heng zawhnate hi zawh hreh suh. I ngaihtuahna i neih apiang chu i doctor nen sawiho rawh.

Primary intestinal lymphangiectasia, PIL, lymphatic obstruction, naupang pum natna, ke hring, protein tlakchham, chaw ei leh in lama natna te