Rapp-Hodgkin syndrome emaw AEC syndrome emaw i hre tawh em? A ni lo mai thei. Hengte hi a tlem hle a, chu chu mi tam zawkin an nei lo tihna a ni. Genetic factors avanga lo awm an ni . A awlsam zawngin, tunah chuan heng dinhmun pahnih hi a inang hle nia ngaih a ni a, natna pawl khata tel ve an ni. Heng dinhmunte hian i sam, nail, vun, sweat glands , leh ha te a nghawng thei a ni. Chuvangin an chungchang hi chipchiar deuhin, awlsam takin han sawi ila.
Engvangin nge hetiang dinhmun hi a lo awm?
Awle, hei hi eng thil nge a thlen tih hi han en hmasa ila. Kan taksaa cell tin hian gene an tih hi an nei vek a. Hengte hi kan nu leh pa hnen atangin kan dawng thin. A dik tak chuan gene tin copy pahnih kan nei a, pakhat chu kan nu hnen atangin leh pakhat chu kan pa hnen atangin a ni.
AEC syndrome hi kan gene pakhat, a bik takin TP63 gene -a chhiatna avanga lo awm a ni. TP63 gene copy pahnih zinga pakhatin he chhiatna hi a neih chuan chu mi chuan AEC syndrome a nei thei a ni.
A châng chuan he dinhmun hi nu leh pa aṭanga fate rochun a ni ṭhin. Hei hian a awmzia chu he genetic defect nei nu leh pa hian an fate hnenah he natna hi an kai theihna chance 50% vel an nei tihna a ni. Mahse a tam zawkah chuan random condition a ni a, chu chu pian tirh atanga chhungkaw history nei lovin a thleng tihna a ni. Chuvangin he thil hi ngaihtuah buai a ngai lo.
He natna lan chhuah dan engte nge ni?
Hei hi a pawimawh ber a ni. AEC syndrome symptoms hi mi hrang hrangah a inang lo thei hle. Chhungkaw khata miten he natna hi nei mah se, an lan chhuah dan chu a inang lo thei. Chuvangin mi zawng zawng hian symptom set inang an nei vek dawn lo.
A tlangpuiin a lan chhuah dan lian ber ber thenkhat chu han thlir ila.
| Symptom a ni | Hrilhfiahna awlsam tak |
|---|---|
| Hmuh leh hmui a inthen | A hmui chunglam chu a insiam kim lo va, cleft a awm ta a ni. Kawngkhar chunglam (upper palate)-ah pawh cleft a awm thei bawk. |
| Thawmhnaw a tlahniam | Taksa hian thawmhnaw a nei tlem emaw, a nei lo emaw pawh a ni thei. Hei hian thawmhnaw a siam tlem hle. Chumi avang chuan taksa chu awlsam takin a lum lutuk thei a, a lum hle bawk. |
| Mitmeng fused a ni | Mitmeng chu a inzawm khawm thei a, a then emaw, a inkhar vek emaw pawh a ni thei. Mittui chhuahna kawngah pawh harsatna a awm thei a, chu chuan mit vawt leh mit pink ang chi dinhmun a thlen thei bawk. |
| Hmasawn harsatna | Naupang hian thanlenna leh thau lutuk chungchangah harsatna a nei thei. |
| Hriatna hloh | Hei hian naupang thusawi zir a tikhawtlai thei a ni. |
| Nail-a inthlak danglamna | Nail a bo emaw, a pianzia danglam tak a nei emaw pawh a ni thei. |
| Vun a tichhe thei | Hmun thenkhatah chuan vun chunglam chu a lo hring chhuak vek a. Hei hi nausen piang thar tan chuan nunna atana hlauhawm tak a ni thei. Chuvangin hei hi a bik takin fimkhur a ngai hle. |
| Ha lam harsatna a awm | Ha thenkhat chu a bo thei a, ha inkarah gap lian tak tak a awm thei a, cone ang maia ha a lo lang thei a, ha pawn lam khuhna, enamel an tih chu a hring thei bawk. |
| Sam lama harsatna nei | Lu leh hmaiah sam a tlem hle thei a, sam awm chu a hring leh a hring thei bawk. |
| Mipa naupang zun kawng lama harsatna nei | Urethra hawnna chu a awmna pangngai ni lovin penis hnuai lamah a awm thei. |
Engtin nge natna hi kan hriat theih ang?
I doctor chuan i natna lan chhuah dan, i damdawi lam chanchin , leh i taksa enfiahna a\angin he natna hi a hre thei tlangpui. Heng symptoms te hi a inzawm khawm chuan AEC syndrome hi rinhlelh theih a ni.
Chu bakah, TP63 gene awm leh awm loh enfiah nan genetic test pawh tih theih a ni bawk . Mahse, he test hi Sri Lanka-a lab tinah tih theih a ni lo. Test specialized deuh a ni.
Hetiang natna vei hlauhawm nu leh pate chuan an fa chu a pian hmain, naupai laiin test tir theihna hun remchang an nei thei a ni . Hemi chungchang hi i doctor nen in sawi dun thei a, i zir belh thei bawk.
Engtin nge enkawl a nih?
Hei hi dam chhung zawnga natna a nih avangin a dam kim thei lo. But don't worry , a symptom tam tak chu hlawhtling taka enkawl theih a ni. Chutiang ti tur chuan doctor team pakhat chauh ni lovin, doctor team nen thawhhona tha tak i neih a ngai dawn a ni. Entir nan:
- Surgeon te pawh an awm
- Dermatologist te chuan an
- Ha enkawltu te
- Mit lam doctor te
Thil chi hrang hranga mithiamte tanpuina i mamawh mai thei. Chutiang natna hmuh tur awm lo tak nena nunpui avanga lungkhamna i hmachhawn theih nan counselling i zawn hi nang leh i chhungte tan pawh a pawimawh hle bawk.
A lan chhuah dan tlangpui enkawlna thenkhat chu hetiang hi a ni:
| Tihdamna | Hrilhfiahna |
|---|---|
| Hmai leh hmui cleft a awm chuan operation tih a ni | Heng crack te hi surgery hmangin hlawhtling takin siamthat theih a ni. |
| Ha enkawl dan | Ha nghet tak chu ha dahna hmanga thlak theih a ni . Naupang tê tête chuan ha lem lakchhuah theih an hmang thei. |
| Mitmeng zai a ni | Mitmeng tangte chu then hran nan surgery an ti thin. A chang chuan mitmeng chu mitmengah a innghah chuan zai ngai lovin a inthen thei a ni. |
| Vun hring (peeling skin) enkawlna | Hliam vun atanga lo chhuak tawh chu zawi zawiin enkawl tur a ni. A châng chuan i doctor chuan hliam chu bleach solution chak lo , Dakins solution ang chi hmanga silfai tûrin a fuih thei che a, chu chuan hrik tihlum a ni. |
| Hriatna lama harsatna nei tan | Beng chhunga tui awmkhawm avanga hriatna hloh leh beng natna a lo awm chuan enkawlnaah chuan beng chhungah tube te te dah a ni. |
| Thusawi hmanga enkawlna | He enkawlna hi naupang hriatna lama harsatna emaw, cleft palate emaw avanga tawng lama harsatna nei tan chuan a \angkai hle. |
| A solution dang pawh a awm bawk | Mit vawt enkawlna atan eye drop i hmang thei a, sam tlakchham emaw, thinning emaw khuh nan wig i hmang thei bawk. |
Take-Home Thuchah a ni
- AEC syndrome hi genetic condition hmuh tur awm lo tak a ni.
- Hei hi natna hrik kai theih a ni lo. Chu chu khawih emaw, kawng dang emaw hmangin mi pakhat atanga mi dangah a darh lo tihna a ni.
- Mi tin hian symptom a inang lo thei hle a, chuvangin midang nen tehkhin suh.
- He natna hi tihdam kim theih ni lo mah se, a natna lan chhuahna tam tak enkawltu leh nun pangngai nei thei tur enkawlna tha tak tak a awm nual.
- Chumi atan chuan specialist doctor hrang hrang team te tanpuina lak a pawimawh hle.
- Nang emaw i fa emaw hian heng symptoms te hi i neih chuan emaw, hemi chungchanga zawhna i neih chuan doctor nen inbia ang che.
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