I taksaah hmun te te leh i pum chhungah tumor te i nei em? Peutz-Jeghers Syndrome (PJS) chungchang hi i zir dawn teh ang!

A chang chuan kan taksaah kan beisei loh thil a thleng thei a ni lawm ni? Nangmah emaw, i fa emaw hian i vunah, a bik takin i hmui chhehvelah hmun dum te te, i rilruah harsatna i nei bawk ang tih han ngaihtuah teh. Heng thilte hi i ngaihtuah angin a awlsam lo mai thei. Vawiin hian kan sawi dawn a, natna lan chhuah dan inang chiah chiah, a tlem deuh a, mahse hriat a pawimawh hle. Chu chu Peutz-Jeghers Syndrome (PJS) natna a ni.

Peutz-Jeghers Syndrome (PJS) hi eng nge ni? A awlsam zawngin i hrethiam ang u!

A awlsam zawngin sawi ila, Peutz-Jeghers Syndrome (PJS) hi i taksa chhungah hian thil te te (polyps) a lo awm a, chubakah i hmai, kut leh ke kephah ah te dum hring hring a lo awm bawk. Heng growth leh spot pahnih hi cancer tak tak an ni lo va, benign an ni tihna a ni . Mahse, PJS neih hian cancer vei theihna a tipung hle.

Heng dark spots (damdawi lamah chuan mucocutaneous hyperpigmentation'' tia koh) te hi PJS vei naupang te zingah hmuh tur a awm a, mahse hun kal zelah a bo zauh zauh thin. Ka sawi tawh ang chi (hamartomatous polyps'' tia koh) hi i chaw ei kawngah ((GI tract)'') a lo awm tlangpui. I ril, i pum, leh i ril lian ((colon)'')-ah te an lang tam ber. Mahse, chaw ei kawng pawnah pawh an lo thang lian thei a, chu chu i kal, i bladder, lung leh hnar-ah te pawh a ni thei. Heng thang lian te hi cancer a lo ni thei a, harsatna hrang hrang a thlen thei a, enkawl ngai a ni.

PJS i neih chuan i doctor chuan cancer leh high-risk tumor a awm leh awm loh a enfiah fo ang.

He PJS condition hi engtiang chiahin nge a awm?

Peutz-Jeghers Syndrome (PJS) hi engtiang chiahin nge a hluar tih chiang taka sawi a harsa a, a chhan chu zirchiangtute hian number dik tak an la nei lo. Mahse mi 300,000 zinga 1 atanga mi 25,000 zinga 1 thlengin khawi hmunah pawh a nghawng thei niin an ngai. Chuvangin, a ni, a awm lo hle .

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PJS natna lan chhuah dan chu engte nge ni? Engtin nge i hriat chian?

PJS hian dark spots (hei hi naupang tê têah hmuh tur a awm) leh hamartomatous polyps'' an tih (hei hi naupang tê leh puitlingah hmuh theih a ni) a thlen ber a ni.

Hmun thim tak tak:

Peutz-Jeghers Syndrome vei naupang tê tête chuan blue-gray emaw brown emaw an nei ṭhin. Hengte hi a chang chuan freckles tia sawi sual a ni. Heng spot te hi naupang kum 1-2 a nih atanga a lo lang tan tlangpui a, an tleirawl lai a thlen meuh chuan a bo zauh zauh thin. Heng spot te hi 1 mm (pencil tip hrual ang vel) atanga 5 mm (pencil eraser ang vel) thleng a ni thei. Taksa peng hrang hrangah an lang thei a:

• Ka hmui chungah emaw, a chhehvelah emaw (hei hi a tam ber) .
• Kawng chhungah
• Hnar
• Mit vel chu
• Kut zungtang
• Chelh
• A hnuai lam
• Anus vel a ni

Digestive tract-a tumor awm avanga natna lan chhuah dan:

Symptom dangte chu chaw ei kawng (a bik takin intestines emaw stomach)-a thanlenna (polyps) emaw, chutiang thanna avanga harsatna awm emaw nen a inzawm a ni. Heng symptoms te hi kum 10 leh 30 inkar i nih chuan a lang tlangpui a, heng symptoms te hi a lo thang lian thei a, chu chu:

• Pum natna ((Pum na)) .
• Nausea leh luak chhuak ((Nausea and vomiting)) .
• Gastrointestinal bleeding ((Chaw chhung thisen chhuak)) .
• I ek chhungah thisen ((Blood in your stool))
• Anemia (thisen chhuah tam vangin taksaa iron level a tlahniam thei) .

PJS hi eng thilin nge a thlen?

Peutz-Jeghers Syndrome vei tam zawk chuan STK11'' tih gene copy khatah mutation (or change) an nei a. He STK11'' hi tumor titawptu gene a ni. Phên dangin sawi ila, he gene hi cell thanna control tu switch ang a ni. He gene hian hna a thawk tha lo a nih chuan light eng reng reng, off tur nei lo ang mai a ni. Cell te chu an inthen darh zel a, an thang lian zel a, an inzawm khawm a, tumor an siam ta a ni.

PJS vei zinga 80% vel chuan an nu leh pate hnen atangin gene mutation hi an rochun a, chu chu an nu emaw an pa emaw atanga an rochun tihna a ni. A dang 20% ​​chuan mutation hi an nei a, mahse an chhungkuaah hian he natna hi an la nei lo a, a nih loh leh mi thenkhat chuan mutation hi an nei lo tak tak lo. Chhungkaw history nei lo (STK11) gene mutation nei te hian an dam chhungin engtik lai pawhin he mutation hi an nei tawh a rinawm. Mahse, scientist-te chuan (STK11) gene mutation tel lo chuan PJS hi engtin nge a lo thanglian tih hi chiang takin an la hre lo.

Engtin nge PJS hi lineage atanga lo chhuak?

A tlangpuiin naupang hian PJS gene hi nu leh pa, gene mutation nei a\angin a rochun \hin. PJS thlentu mutation hi autosomal dominant pattern-a inherit a ni.

Hetiang hian a thleng thin: Nu leh pa pahnih hian gene (STK11) copy pakhat chu an fa hnenah an pe chhuak thin. PJS symptoms leh complications i neih theihna chance a sang zawk a nih chuan he mutated gene hi nu leh pa pakhat chauh hnen atangin i rochun a ni ngei ang.

He gene mutation hi i neih chuan i fa pawhin a neih ve theihna chance 50% a awm.

PJS avanga harsatna awm thei te chu engte nge ni?

Hei hi a harsatna lian ber chu cancer a ni . Zirchiangtute chuan PJS i neih chuan i dam chhungin cancer vei theihna chance chu 93% vel a ni thei niin an sawi. Chuvang chuan i doctor chuan cancer a screen reng ang che. Chutiang chuan a hmaa hriatchhuah theih a ni a, enkawl theih a ni bawk.

Harsatna dangte chu:

• Intussusception of the small bowel : Hei hi i small intestine thenkhat chu chhung lam hawia a inzawm khawm lai a ni a, chu chu sock chhung lam hawia a inher ang maiin a ni. Hei hi thil lian tak (polyp) pakhatin intestine kal tlanga kal a tum hian a thleng thin. Hetiang dinhmun hian PJS vei zinga 69% thleng a nghawng thei a ni.
• Small bowel obstruction ((Thinlung te tak te inkharkhip)) .: Chu tumor chuan i small intestine a block thei. PJS vei zinga 50% vel chuan intestinal blockage an nei ang a, kum 20 an tlin hmain operation neih a ngai ang.
• Gastrointestinal bleeding : Tumor hian chaw ei kawng tissue-ah pressure a siam thei a, thisen a chhuak thei a ni.
• Iron-deficiency anemia: Thisen chhuah chhunzawm zel chuan taksaa iron level a ti tlahniam thei a, chu chuan thisen tlakchhamna a thlen thei a ni .

Hmeichhiaah chuan ovarian tumor chi khat sex-cord tumors'' an tih leh cervical cancer chi khat, na tak, adenoma malignum'' tih chu a lo awm thei a ni. Heng tumor te hian thlasik hun mumal lo leh puitlin hma a thlen thei a ni.

Mipa hian an testicles-ah sex-cord leh Sertoli-cell type tumor an nei thei a. Hei hian hnute (gynecomastia) a siam thei a, puitlin hun a paltlang hma thei a, an ruh chu a pangngai aia rang zawkin a thang thei (advanced skeletal age), a pangngai aia tawi zawk pawh a ni thei bawk.

PJS nena inzawm cancer hlauhawmna chu eng nge ni?

Peutz-Jeghers Syndrome (PJS) hian chaw ei leh taksa peng dang cancer vei theihna a tipung a ni. PJS vei mi pakhatin cancer a vei chuan kum 42 vel a ni tlangpui a, PJS vei zinga cancer chi hrang hrang awm tam ber leh an rates te chu hetiang hi a ni:

• Colorectal cancer: 40% thleng a ni.
• Hnute cancer: 30% atanga 50% thleng.
• Pancreatic cancer ((Pancreatic cancer)): 11% atanga 36% inkar a ni.
• Pum cancer: 30% thleng.
• Ovarian cancer ((Ovarian cancer)): 20% a ni.
• Lung cancer ((Lung cancer)): 15% a ni.
• Small bowel cancer ((Small bowel cancer)): 13% thleng a ni.
• Cervical cancer ((Cervical cancer)): 10% a ni.
• Uterine cancer: 10% aia tlem.
• Testicular cancer: 10% aia tlem lo.
• Chaw ei kawng cancer: 2%.

Heng risk percentage te hi i hmuh hian hlau suh. A pawimawh ber chu a hun taka test neih hi a ni. Tichuan, eng emaw a awm a nih chuan hriatchhuah leh enkawl thuai theih a ni.

PJS hi engtin nge kan hriat theih ang? (Diagnosis) tih a ni.

PJS vei nia hriat tam zawk chuan doctor an pan a, a chhan chu an rilru a buai (bowel obstruction) emaw intussusception (intestinal intussusception) emaw symptom an neih vang a ni. PJS hmuhchhuah kum zat chu kum 23 vel a ni. Doctor-te chuan PJS hmuhchhuah nan a hnuaia mite hi an zawng thin:

• Chhungkaw zingah PJS nei an awm leh awm loh history.
• Taksaah hmun dum a awm.
• Polyps chu chaw ei kawngah a awm.

Tin, (STK11) gene-ah hian mutation a awm em tih an enfiah mek bawk.

He natna hi hriat theih nan eng test nge tih thin?

I doctor chuan imaging procedure hrang hrang a ti thei a, a bik takin test-ah chuan camera (scope) nei tube hmanga i chaw kawng chhunga tumor awmte chu a zawng thei a ni. Heng test-ah hian:

• Colonoscopy : Hei hian i intestine lian tak a enfiah thin.
• Upper endoscopy : Hei hian i chaw kawng, i pum leh i rilru chhungril (small intestine) chunglam a enfiah thin.
• (Capsule endoscopy) : Hetah hian camera te tak te awmna capsule i ei thin. I digestive system a kal pelh rualin thlalak a la thin.

Thisen sample la in iron-deficiency anemia a awm leh awm loh i enfiah thei bawk. STK11 gene mutation i neih leh neih loh hriat nan i thisenah genetic test i ti thei bawk.

Peutz-Jeghers Syndrome (PJS) hi engtin nge enkawl a nih?

I doctor-te chuan i taksa peng hrang hrangte an enfiah ber ang a, chu chu cancer emaw, tumor avanga harsatna dang emaw a awm leh awm loh enfiah tihna a ni.

Colonoscopy hmang hian intestine lian chhunga tumor awmte chu a paih chhuak thei a ni. A tul chuan pum chhung leh small intestine chunglam (duodenum)-a tumor awmte pawh biopsy-in lakchhuah theih a ni bawk. A châng chuan, balloon-assisted enteroscopy tih test hmangin small intestine thuk zawka tumor awmte chu hmuh leh paih chhuah theih a ni.

A then phei chuan a tumor te chu lakchhuah nan operation neih a ngai thei bawk. Surgeon-te chuan tumor chu pakhat hnu pakhatin an la chhuak thei tlangpui a, intestine hmun thenkhat chu an la chhuak lo.

Eng ang screening nge ka neih a ngai dawn?

Hei hi a pawimawh ber a ni. PJS i neih chuan test hrang hrang neih fo a ngai dawn a ni. Hei hi tithinur deuh angin lang mah se, i hriselna humhim a pawimawh hle.

Test neih dan tlangpui chu hetiang hi a ni:

• (CT/MRI enterography) emaw (video capsule endoscopy) emaw a ni thei ang :
• Kum 8-10 inkar atanga tan tur a ni. Tumor a awm chuan kum 2-3 danah tih leh thin ang che.
• Tumor a awm loh chuan kum 18 i tlin hma nghak la, test tan leh la, kum 2-3 danah tih tur a ni.
• A tlai lutuk chuan kum 12 atanga tan tur a ni a, thei i neih chuan kum tin emaw, kum 2-3 danah emaw ti thin ang che.
• (Endoscopy chunglam) : 1.1.
• Kum 12 atanga tan tur a ni a, nuts i neih chuan kum tin, emaw kum 2-3 danah emaw ti thin ang che.
• (Magnetic resonance cholangiopancreatography (MRCP) emaw (endoscopic ultrasound) emaw a ni thei bawk .
• Kum 25-30 a\anga \anin kum 1-2 danah tih tur a ni.

Hmeichhia tan test bik:

• Kum 25 a nih atangin kum khatah vawi hnih Doctor hnenah hnute enfiah tir thin ang che.
• Kum 25 a\angin kum tin mammogram leh hnute MRI neih thin tur a ni.
• Kum 18 atanga 20 inkar, kum tin pelvic exam leh Pap smear neih thin tur a ni.
• Kum 18 atanga 20 inkarah kum tin (transvaginal ultrasound) (hei hi a pawimawh lo, damdawi lam thurawn angin).

Mipa tan test bik:

• Kum 10 a\angin kum tin testicular exam i nei tur a ni. Tin, hmeichhe lam thila inthlak danglamna eng pawh, hnute lo thanglian ang chi te pawh i hriat a ngai bawk.

Peutz-Jeghers Syndrome (PJS) hi ven theih a ni em?

PJS hi thlahtute atanga lo piang a nih tlangpui avangin a lo awm loh nan engmah tih theih a awm lo tak zet a ni.

Mahse, i tih theih thil a awm. PJS i neih chuan chhungkaw member dangte hnenah hriattir la, genetic counseling la turin fuih bawk ang che. He evaluation hian PJS gene mutation a awm leh awm loh a enfiah ang. STK11 gene mutation pawh an neih chuan cancer laka invenna leh hrisel taka awm theihna tura pui turin rawtna an dawng ang.

PJS i neih chuan i fa pawhin gene mutation a neih theihna chance 50% a awm. Mahse chu risk tihtlem nan chuan duhthlan tur thenkhat a awm.

Entirnan, naute neih nan in vitro fertilization (IVF) i hmang a nih chuan preimplantation genetic diagnosis (PGD) tih hi i lo enchhin ve mai thei. PGD ​​hian fertilized embryos te chu genetic mutations an neih leh neih loh test a ni.

Mahse, PGD hi thil buaithlak leh man to tak a nih avangin thutlukna siam hmain genetic counselor nen sawiho hmasak a tha ber.

Peutz-Jeghers Syndrome natna hi eng ang nge ni? (Hmuh dan tur) .

Peutz-Jeghers Syndrome (PJS) hi tihdam theih a ni lo. Nun chhung zawnga awm thei a ni a, i fate hnenah pawh kai darh theih a ni. PJS i neih chuan polyp awm leh awm loh enfiah fo tur a ni a, cancer a nih theih avangin emaw, intestinal obstruction a thlen thei a, operation a ngai a ni.

Ka doctor hnenah eng nge ka zawh ang?

PJS i neih chuan doctor hnenah heng zawhnate hi zawh theihnghilh suh:

• Eng ang screening nge ka neih a ngai ang? Engtianga zingin?
• Eng symptom nge a lo awm a nih chuan ka biak nghal tur che?
• Eng ang enkawlna nge ka mamawh?
• Eng ang specialist nge ka thawhpui a ngai dawn?
• PJS neih hian engtin nge ka fertility plan-ah nghawng a neih ang?

Peutz-Jeghers Syndrome (PJS) vei tan chuan an doctor nen thawhhona tha tak neiin an dinhmun enfiah a pawimawh hle .. Doctor appointment pangngaia kal hi a chang chuan a hahthlak thei a, a hahthlak thei bawk. Mahse i hriselna chungchânga awm reng a pawimawh. Cancer ang chi complication hriat hmasak hian hrisel zawk, dam rei zawk i neih theih nan kawng thui tak a zawh thei a ni. I natna hmuhchhuah hian i nunphung leh hun rei tak chhunga hriselna a nghawng dan tur chu i doctor nen sawiho rawh.