I taksaah hian blackhead leh pimples i nei em? Peutz-Jeghers Syndrome (PJS) chungchang hi han sawi ila!

I fa tê tê hian a hmui vêlah te, a kâ chhûngah te, a kutah te, a dum te te a nei em? Spot mai mai an ni tih i ngaihtuah mai thei. Mahse heng spot te hi taksa chhunga, a bik takin intestine-a lo awm tumor chi khat nen a inzawm thei tih i hriat chuan mak i ti mai thei. Vawiin hian hriat tur awm lo mahse pawimawh tak kan sawi dawn a ni. Hei hi Peutz-Jeghers Syndrome, a tawi zawngin PJS an ti a.

A awlsam zawngin, Peutz-Jeghers Syndrome (PJS) hi eng nge ni?

Peutz-Jeghers Syndrome (PJS) hi genetic condition a ni a, kan taksa chhungah hian thinghnah te te (polyps) a lo piang a, kan vun leh kan ka chhungah hian hmun dum a lo lang thin. A tha ber chu heng than leh spot te hi cancer (benign) a ni lo tih hi a ni. Mahse buaina chu PJS vei te hian midang aiin nakin lawkah cancer vei theihna an nei sang zawk a ni.

Heng hmun dum (damdawi lamah chuan `mucocutaneous hyperpigmentation` an tih) te hi naupan laiah hmuh tur a awm tlangpui. Mahse, kan lo upat deuh deuh chuan an bo zauh zauh a. Tumor chi ka sawi tak (`hamartomatous polyps`) hi kan chaw ei dan (`gastrointestinal tract`) ah a lo awm tam ber. Chu chu, small intestine, stomach, leh colon ang hmunah te a ni . Mahse a chang chuan kal, bladder, lung, leh hnar ang hmunah te pawh an lo thang lian thei bawk. Heng tumor te hian harsatna chi hrang hrang a thlen thei a, a chang chuan cancer ah a chang thei bawk.

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

He natna hi engtiang chiahin nge a hluar?

Hei hi thil awm lo tak a ni. Mi engzat nge vei tih chiang taka hriat ni lo mah se, zirchiangtute chuan mi 300,000 zinga pakhat leh mi 25,000 zinga pakhat inkar a nghawng niin an chhut .

PJS natna lan chhuah dan chu engte nge ni?

PJS hian dark spot (naupan lai) leh polyp (naupang leh puitlingah) a thlen ber a ni. Heng symptoms te hi a hranin han en ila.

Engvangin nge he PJS hi a lo thanglian? Eng nge a chhan?

PJS vei tam zawk chuan STK11 an tih gene-ah mutation an nei a. STK11 hi tumor suppressor gene a ni a . A awlsam zawngin sawi ila, he gene hnathawh hi kan taksaa cell-te thunun theih loha lo thanglian zel tur control hi a ni.

PJS vei zinga 80% vel chuan he gene mutation hi an nu emaw an pa emaw hnen atangin an rochun thin. A bak 20% chuan chhungkaw history-ah natna hi an nei lo mai thei a, mahse anmahni ngeiin gene mutation hi an nei thei a ni. Mi thenkhat chuan STK11 gene ah danglamna awm lovin PJS an nei thin. Scientist te hian a chhan dik tak an la hre lo.

Engtin nge he natna hi rochun a nih?

A tlangpuiin, naupang chuan he gene danglam tak hi nu leh pa pakhat aṭangin a rochun a ni. ``Autosomal dominant`` pattern-a rochun a ni. Hei hian a awmzia chu nu emaw pa emaw hian he ``STK11`` gene danglam tak hi rochun mahse, naupang hian PJS symptoms leh complications a neih theihna chance a sang zawk tihna a ni. He gene mutation hi i neih chuan i fa hian 50% in a rochun theihna chance a nei a ni.

PJS avanga harsatna awm thei te chu engte nge ni?

A harsatna lian ber chu cancer a ni .Zirchiangtute chuan PJS vei chuan a dam chhungin cancer vei theihna chance 93% thleng a nei tih an sawi . Chuvangin doctor-te chuan cancer hi an screen fo thin a, a hmaa hriatchhuah an tum thin.

Harsatna dangte chu:

• Intussusception: Hei hi small intestine-a polyp lian tak pakhatin a rawn nawr lut a, intestine thenkhat chu chhung lam hawia a ben tir hian a thleng thin. Hetiang dinhmun hian PJS vei zinga 69% thleng a nghawng thei a ni.
• Small bowel obstruction: Tumor hian small intestine a block thei. PJS vei zinga 50% vel chuan intestinal obstruction na tak an nei a, kum 20 an tlin hmain operation neih a ngai a ni.
• Digestive tract atanga thisen chhuak: Tumor hian intestinal tissue ah pressure a siam thei a, thisen a chhuah tir thei bawk.
• Iron-deficiency anemia: Thisen chhuah chhunzawm zel hian taksaa iron a tlahniam a, thisen tlakchhamna a thlen thin.

Hmeichhia leh mipa tan harsatna bik

• Hmeichhia: Ovarian tumor (sex-cord tumor) leh cancer chi khat, a hlauhawm tak, adenoma malignum an tih, serh chhungah a lo awm thin. Hei hian thlasik hun mumal lo emaw, puitlin hma emaw a thlen thei a ni.
• Mipa: An testicles-ah tumor (Sertoli-cell tumors) an nei thei. Hei hian hnute a tipung thei (gynecomastia), puitlin hma a thlen thei a, an ruhte chu a tlangpui aia rang zawkin a thang lian thei a, a tawpah chuan an sei tawi zawk thei bawk.

PJS leh cancer hlauhawmna

PJS hian taksa chhunga chaw ei leh taksa peng dangte cancer vei theihna a tipung hle. PJS vei pakhatin cancer a vei tih hmuhchhuah kum zat chu kum 42 vel a ni.

Engtin nge PJS hi hmuhchhuah a nih?

Mi tam tak chuan doctor an hmuh hnuah PJS an vei tih hmuhchhuah a ni a, hei hi intestinal obstruction emaw intussusception emaw ang chi symptoms vang a ni. Diagnosis kum zat chu kum 23 vel a ni. Diagnosis siam tur chuan doctor-te chuan a hnuaia mite hi an zawng thin:

• Chhungkaw zingah PJS nei an awm em?
• Vunah hmun dum a awm.
• Digestive system-ah polyp a awm leh awm loh.

Tin, `STK11` gene mutation i neih leh neih loh enfiah nan genetic test pawh tih theih a ni bawk.

Natna hriat chian nan test neih thin a ni

I doctor chuan i intestine-a tumor awm leh awm loh enfiah nan test hrang hrang neih a rawt thei ang. Chung zinga ṭhenkhat chu:

• Colonoscopy: Camera hmanga tube hmanga intestine lian enfiah.
• Upper endoscopy: Chaw ei kawng, pum, leh small intestine chunglam enfiah.
• Capsule endoscopy: Camera te tak te awmna capsule ei. He camera hian chaw ei kawng (digestive tract) atanga a kal laiin thlalak a la thin.

Iron tlakchhamna avanga thisen tlakchhamna (anemia) i neih leh neih loh enfiah nan thisen sample i la thei bawk.

PJS enkawlna tur engte nge ni?

PJS hi a dam kim thei lo va, chuvangin enkawlna tum ber chu cancer hlauhawmna enfiah leh tumor avanga harsatna awm thei ven hi a ni.

Doctor-te chuan colonoscopy an tih hian intestine lian chhunga tumor awmte chu an paih chhuak thei a ni. Tin, a tul chuan pum chhung leh small intestine chunglam a tumor te pawh an paih chhuak thei bawk. A châng chuan, balloon-assisted enteroscopy ang chi special procedure hmangin small intestine chhunga lut lehzual tumor te chu paih chhuah a ni.

A then phei chuan cyst lakchhuah nan operation neih a ngai thei bawk.

Eng screening nge ka neih fo tur?

PJS i neih chuan cancer leh harsatna dangte hriat hmasak nan i dam chhungin screening test neih fo a ngai dawn a ni. Hei hi a tithinur deuh mai thei a, mahse i nunna humhim nan a pawimawh hle.

He natna hi ven theih a ni em?

PJS hi a tlangpuiin rochun a nih avangin a lo thang zel tur venna tur engmah kan ti thei lo.

Mahse PJS i neih chuan i tih theih pawimawh ber chu i chhungte hnenah hriattir a, genetic counseling atan refer hi a ni . Tichuan he gene mutation hi test theih a ni a, cancer laka invenna atana an mamawh thurawn an dawng thei bawk.

PJS i neih chuan i fain a vei theihna chance 50% a nei a ni. Mahse tunah chuan chu hlauhawmna tihtlem dan tur a awm ta. Entirnan, in vitro fertilization (IVF) hmanga naute i neih chuan, preimplantation genetic diagnosis (PGD) tih technique hmangin embryo-a gene mutation awm leh awm loh enfiah theih a ni. Hengte hi thil buaithlak leh man to tak a nih avangin he thil hi doctor nen inbiakna neih a pawimawh hle.