I naute hmui chu a hring chhuak niin a lang a, a hmui chu a lian hle niin a lang em? Cherubism pawh a ni thei. Kan sawi dawn nia!

I naupang tê hmel chu a danglam deuh a, an hmui chu a hring chhuak ang maiin a lang tih i ngaihtuah deuh mai thei. A nih loh leh doctor in an hmui a inthlak thu a hrilh che em? Hetiang dinhmun hi (Cherubism) kan ti a ni. A hming hi a mak deuhin a lang thei a, mahse a awlsam zawngin i dah ang u. Hei hi a awm lo hle a, mahse hriat chian a pawimawh hle.

Cherub sakhua chu eng nge ni?

A awlsam zawngin sawi ila, Cherubism hi genetic condition hmuh tur awm lo tak a ni. I fate ruh hrisel ni lovin a hmui ruh (abnormal tissue) a lo thang lian chuan a thleng thin. He tissue pangngai lo hi cancer a ni lo va, a tlangpuiin a na lo. Mahse, naupang hmui chu a sir lehlamah a zau thei a, an hmui chu a zungbun leh a hring chhuak thei a ni . Naupang thenkhat chuan ha an hloh emaw, ha thang mumal lo emaw an nei thei bawk. Cherubism hian taksa ruh dang a nghawng lo va, hmui ruh chauh a nghawng a ni .

Heng symptoms te hi pian hnuah a lang nghal tan lo. Kum 2 leh 7 inkar vel an lo lang tlangpui a, an natna nasat dan hi mi hrang hrangah a inang lo thei. Naupang thenkhat chuan natna lan chhuah dan an nei lo thei a, a nih loh leh natna lan chhuah dan chu a fiah lo hle a, hriat theih loh khawpin a lang thei bawk. Mahse, a nasat zawkah chuan naupang chuan tawng a harsat thei a, a ei a harsa thei a, thawk pawh a harsa thei bawk.

A tha ber chu cherubism hian puitlin hnuah enkawlna tel lovin a mah chauhin a reh fo thin hi a ni. Doctor-te chuan he natna hi an thlir reng a, a natna lan chhuah dan a nasat emaw, naupang nunphung nasa taka nghawng a neih emaw chauhvin enkawl an rawt a ni.

Cherub sakhua hi engtiang chiahin nge a hluar?

Cherub sakhua hi a tam vak lo. Researcher-te chuan he natna hi mi engzat nge vei tih chiang takin an hre lo a, mahse medical article tihchhuah tawhte hmangin estimate engemaw zat an siam a ni. Kum 2019-a zirchianna pakhatah chuan damdawi lam lehkhabuah Cherubism case 513 report a awm tih hmuhchhuah a ni. Chuvangin a tlem zia chu i hmu thei ang.

Cherub sakhua chhinchhiahnate chu engte nge ni?

Cherub sakhua chhinchhiahna langsar tak takte chu hengte hi an ni:

• A hnuai lam ruh (manible) chu beisei aia zau a ni.
• Naupang jaw area -a cyst ang chi a lo thang lian.
• A hmui round, puffy tak a ni.
• Ha pianzia inthlak danglamna, ha bo emaw, ha nghawng emaw.

Naupang thenkhat pawhin heng symptoms te hi an nei thei bawk:

• Mit chu a rawn chhuak a, chunglam hawi ang maiin a lang. Mit a var (sclera) chu black iris hnuaiah a lang a.
• Upper jaw-a extra tissue hian mit leh a kaihhnawih nerve te a nghawng a, mit hmuhna a hloh emaw, mit hmuhna a hloh zauh zauh emaw a ni.
• Kawngkhar thawk chhuak.
• A bengchheng hle.
• Mut theih lohna (obstructive sleep apnea) a awm.
• Thusawi emaw, ei harsa emaw.

Cherub sakhua lo awm chhante chu engte nge ni?

Cherubism vei tam zawk (90% aia tam) hi `SH3BP2` an tih gene-a mutations vang a ni.

He `SH3BP2` gene hian kan taksa chu `SH3BP2` protein siam dan tur a zirtir a. He protein hi ruh tissue hlui lakchhuahna leh ruh tissue thar siamna (bone remodeling) ah a inrawlh a ni. Genetic mutations hian taksain he `SH3BP2` protein hi a siam tam lutuk a.

He protein hi a tam lutuk chuan jawbone-ah inflammation a awm thin. Tin , osteoclasts an tih cell siamchhuahna a tipung bawk . Osteoclasts hi cell chi khat a ni a, taksain a mamawh tawh loh ruh tissue tihchhiatna kawngah hmun pawimawh tak a chang a ni. Mahse, heng cell te hi an tam lutuk chuan a tul loh hunah jawbone chhunga ruh tissue te chu an tichhia tan thin. Hetiang ruh hloh dan pangngai lo hi SH3BP2 tihpun avanga inflammation awm nen hian jawbone-ah cyst ang chi a lo piang chhuak thin. Heng a lo thang lian te hi a lian zauh zauh a, chu chu he natna (Cherubism) ziarang (characteristic symptoms) thlentu a ni.

Mahse, Cherubism vei naupang thenkhat chuan SH3BP2 gene mutation an nei lo. Chutiang hunah chuan genetic mutations dang pawh a mawhphurtu a ni thei. Mahse zirchiangtute chuan chu mutations chu eng nge ni tih chiang takin an la hre chiang lo.

(Cherubism) hi thlah khat atanga thlah khata lo chhuak thil a ni em?

Ni e, naupang tam tak chuan Cherubism thlentu genetic mutation chu an rochun a ni. He condition hi autosomal dominant pattern of inheritance-ah a kal tlang a ni. A awlsam zawngin, nu leh pa pakhatin genetic mutation a neih chuan naupang pawhin a vei theihna chance a nei a ni.

Amaherawhchu, naupang thenkhat chuan chhungkuaa tu mahin an la nei lo a nih pawhin he natna hi an nei thei a ni. Zirchiangtute chuan hei hi ‘de novo mutation’ tiin an sawi. Chu chu mi pakhata, chhungkaw history engmah nei lo, genetic change thar lo thleng tihna a ni.

Chuvangin, Cherub sakhua hi rochun a nih tlangpui laiin, chutiang chu a ni ngai lo. Chhungkaw zingah tumahin he natna hi nei lo mah se, doctor-te chuan Cherubism hi natna hriat theihnaah an ngai a ni.

Cherub sakhua nena inzawm dinhmun dang a awm em?

Naupang thenkhatah chuan Cherubism hi genetic condition dang pakhat angin a awm thei bawk. Heng a kaihhnawih dinhmun thenkhat chu:

• `(Fragile X syndrome)` tih a ni
• `(Jaffe-Campanacci natna)`
• `(Neurofibromatosis chi 1)`
• `(Noonan syndrome)` tih a ni
• `(Ramon natna)`

Chutiang hunah chuan Cherubism awm chhan chu SH3BP2 gene mutation vang a ni lo. Chu ai chuan, he syndrome nena inzawm genetic change dangte hian a mawhphurtu a ni.

Doctor-te hian engtin nge Cherubism hi an hriat chhuah?

Doctor-te chuan Cherubism an hriat theih nan hengte hi an zawm a ni:

• Taksa enfiahna: Hei hian naupang chu a natna lan chhuah dan, a danglam bik taka a hmui a zau, a ha bo, a nih loh leh ha lama harsatna (Cherubism) a neih leh neih loh enfiah a ni.
• Imaging test order: Heng zingah hian X-ray emaw CT scan (computed tomography scan) ang chi test te pawh a tel a ni. Hengte hian jawbone dinhmun tehna tha zawk a siam thei a ni.
• Ordering genetic testing: Heng test te hi genetic mutations awm leh awm loh enfiah nan an ti thin.

Doctor-te pawhin natna dang, a lan chhuahna inang nei, fibrous dysplasia emaw aneurysmal bone cysts , te chu an paih chhuak a ngai a, Cherubism hi genetic syndrome-a tel a nih leh nih loh an hriat a ngai bawk.

Cherub sakhua enkawl dante chu engte nge ni?

Cherubism vei naupang tam zawk hian enkawlna bik an mamawh lo. Naupang enkawltu doctor-te chuan "nghah leh en" tih dan an hmang fo thin. Chu chu, an dinhmun an en a, a lo awm dan an en tihna a ni. A tam zawkah chuan heng tissue pangngai lote hi naupangin puitlin hun a thlen thlengin an thang lian a, kum engemaw zat chu an awm reng a, chutah chuan zawi zawiin an lo tlem tan ta a ni. Cherubism chhinchhiahna hi puitlin hma lamah a bo tlangpui.

Mahse, i fain ei leh in lama harsatna a neih chuan, doctor chuan zai a rawt thei a ni. Mahse, doctor-te chuan tissue than dan pangngai lo chu a reh hnuah operation an rawt tlangpui. Miin natna lan chhuah dan a la nei a, a hmel lan dan thlak danglam a duh chuan operation tih a ngaihtuah theih a ni. Reconstructive surgery hian mi hmel chu a duh angin a siam danglam thei a ni.

Supportive treatment te pawh a awm bawk

Cherubism hian naupang nun kawng tam tak a nghawng thei a, a bik takin a natna a nasat chuan a nghawng thei a ni. Chuvangin naupang chuan an mamawh ang zela enkawlna a mamawh mai thei. Enkawlna kawng hrang hrangte chu:

• Ha enkawlna: Cherubism vei naupangte chuan ha enkawl emaw, ha dah emaw ang chi ha enkawlna an mamawh thei a ni . Vawikhat chu a hmui chhunga cyst ang chi lo thang lian te chu a lo tlem tan chuan naupang chuan braces (orthodontics) pawh a mamawh thei bawk .
• Visual support: Cherubism natna khirh takah chuan naupang mit hmuhna a nghawng thei a ni. Mit lam doctor chuan naupang mit hmuh dan a enfiah thei a, an mamawh tur solution tha ber a zawng thei a ni.
• Thusawi leh ei leh in lama tanpui: Naupangin tawng a harsat emaw, a ei emaw a harsat chuan, .Speech-language pathologist (SLP) chuan a pui thei a ni.
• Psychological support: Cherubism hian naupang mahni inngaihhlutna a nghawng thei a. I fate chu an rilru put hmang leh he dinhmun hian an self-concept a nghawng dan chungchang sawipui a pawimawh hle. I fa chu naupang rilru lam thiamte nena an rilru put hmang chungchang sawipui chuan hlawkna a hmu thei ang.

Cherub sakhua neite tan eng thlirna nge ni?

Mi tam zawk chuan kum 30 an tlin chuan Cherubism symptoms an nei tawh lovang a, chutih lai chuan tissue pangngai lo chu ruh tissue pangngaiin a thlak ta a ni.

A tlem hle a, cherubism chhinchhiahna hi puitling thlengin a awm reng thei. Chutiang a nih chuan doctor chuan a tul angin kaihhruaina leh puihna a pe ang.

Cherub sakhua hi ven theih a ni em?

Doctor-te chuan he natna hi ven dan tur an hre lo. I chhungkuaa mi pakhatin Cherubism a neih chuan naupai hmain genetic counselling lak hmasak phawt a tha mai thei.

Engtin nge ka fa ka enkawl ang?

I fain Cherub sakhua a neih chuan hengte hi hre reng ang che:

• I fa chu naupang enkawltu hnenah hruai chhunzawm zel ang che. Doctor chuan engzat nge lo haw tur tih a hrilh ang che.
• Doctor-in a rawt angin i fa mit chu enfiah tir rawh.
• Naupang chu rilru lamah thlawp rawh. Anmahni fuih la, sawipui rawh.

Engtikah nge doctor ka koh ang?

I fain a hnuaia mite hi a neih chuan i pediatrician ko rawh:

• Thâwk lama harsatna, bengchheng emaw, mut lama thawk hah emaw .
• Chaw ei harsa.
• Thusawi harsa.
• Mit hmuhna lama harsatna a awm.

Engtin nge hming (Cherubism) hi a lo awm?

He natna hi kum 1933 khan Dr. William A. Jones chuan a hming a vuah a ni . "Cherubism" tih hming hi a thlan chhan chu he natna lan chhuah danah hian naupang ang maia hmai hring leh a chang chuan mit inthlak danglam te a tel a ni. Heng thilte hi "cherub" ang mai a ni tih a ring a, chu chu art leh sakhaw kalphung hrang hranga hmuh theih, vantirhkoh hmeltha tak a ni . Chumi hnuah chuan he hming hi hman a ni ta a ni.

I fa hian natna hmuh tur awm lo tak a nei tih i hriat chuan thil mak tak a ni thei. Cherubism chungchângah chuan pian tirh lamah a lan chhuah dan a lang lo va, chuvangin i fa hmaiah danglamna i hmuh emaw, doctor-in a sawi emaw hian i buai hle mai thei. Mahse, Cherub sakhua tam zawk chu naupang puitling a nih meuh chuan a mah chauhin a reh ta tih hre reng ang che.A nasat chuan doctor ten an natna (Cherubism) avanga nghawng a neih chu an enkawl thei a ni. I fate chu hmangaihna leh rilru lama ṭanpuina tam tak pêk chuan an naupan lai leh an tleirawl hun ṭha ber hmang tûra an mamawh enkawlna zawng zawng an hmuh theih nân a ṭanpui ang.

Take-Home Thuchah a ni

Cherubism hi genetic condition hmuh tur awm lo tak a ni a, jawbone a nghawng a ni. Hei hian naupang hmui a tibuai thei a, a hmui pawh a zau thei hle. Hei hi naupang a lo puitlin chuan a tha zawk tlangpui. Amaherawhchu, thaw harsa emaw, ei harsa emaw an neih chuan Doctor pan tur a ni. A pawimawh ber chu i fate hmangaihna leh thlawpna pe chhunzawm zel hi a ni. A pawimawh ber chu i fa chu damdawi lam thurawn zawm chungin a chak reng theih nan tanpui hi a ni. Lungngai suh, he zinkawngah hian nangmah chauh i ni lo.

` Cherubism, genetic diseases, jawbone, bulging cheeks, naupang natna, ha harsatna, SH3BP2 gene