I kut emaw i ke emaw chu a danglamin a thim em? He ruh natna (Melorheostosis) hmuh tur awm lo tak hi i zir dawn teh ang!

Nang emaw i fa emaw hian a chang chuan kut emaw, ke emaw, thinga siam mai mai emaw, a thuk emaw, a khauh emaw mak tak maiin i hria em? A nih loh leh, kut pakhat emaw, ke pakhat emaw chu a dang aiin a thau zawkin a lang em? Naupan lai atang tawh khan ruh pakhat chu i ben tha thei lo va, i dinglam thei lo va, natna i nei em? Hengte hi random thil mai a ni lo. Vawiin hian heng thil hnung lama awm thei ruh natna hmuh tur awm lo tak mai kan sawi dawn a ni.

He natna mak tak (Melorheostosis) hi eng nge ni?

A awlsam zawngin sawi ila, Melorheostosis hi ruh natna a ni a, a tlem hle. Chutah chuan kan ruh pawn lam layer-ah ruh tissue thar a lo piang tan a, chu chu cortical bones an ti a ni. Plaster layer dang dah belh ang maiin han ngaihtuah teh, kan ruh pakhat chungah ruh tam zawk a lo piang ta a ni. Chuti a nih chuan eng nge thleng ang? Chu ruh chu a thim zual sauh sauh a.

Taksa kil khat, kut emaw, ke emaw a nghawng tlangpui. Mahse, ruh dang, hip emaw sternum emaw a nghawng thei bawk. Naupan lai emaw tleirawl lai emaw atanga a lan chhuah dan hi a intan fo thin .

Hemi chungchanga thil danglam bik dang chu he ruh siam thar hi X-ray-a a lan dan hi a ni. Doctor te chuan candle atanga melted wax a leak chhuak a , a tla chhuak ang maiin an sawi . Chuvang chuan 'dripping candle wax' look an ti bawk. Mak tak mai a ni lawm ni?

Meloriostosis hi a takah chuan skeletal dysplasia chi khat a ni. Leri natna tia hriat a ni bawk.

Engtin nge "meloriostosis" tih hming hi a lo awm?

‘Melorheostosis’ tih thumal hi i hriat chuan a lei a ti na deuh mai thei, a dik em? Mahse he hming awmzia i hriat hnu chuan he natna chungchangah hian ngaihdan engemaw zat i nei thei ang. He hming hi Greek tawnga thumal pathum inzawmkhawm atanga siam a ni. Eng nge an nih i en ang u?

• ‘Melos’ tih thumal hian kut emaw ke emaw , chu chu kan taksa peng pakhat tihna a ni.
• ‘Rheos’ tih awmzia chu luang chhuak tihna a ni . Lui luang ang maiin.
• ‘Ostosis’ tih awmzia chu ruh siam tihna a ni .

Tunah chuan i hrethiam tawh a ni lawm ni? Heng thumal pathumte hi kan inzawm khawm chuan a hming hian ruh siam dan a hriattir a, a hmaa kan sawi tawh ang khan candle atanga wax tlanchhuak ang mai a ni. Hming tling tak a ni lawm ni?

Engtin nge meloriostosis hian taksa a nghawng?

Nang emaw i fa emaw hian meliorostosis i nei a nih chuan, ruh lian lo tak, lian zawk chuan i range of motion a tikhawtlai thei a ni. Entîr nân, kut i chawi ṭha thei lo emaw, i ke i ben ṭha thei lo emaw, i ding nghet thei lo emaw pawh a ni thei. Tin, ruh (joint deformities) (contractures) pawh i nei thei bawk. Chumi awmzia chu joint pakhat chu a hmunah a lock ta tihna a ni. Mi thenkhat chuan natna nasa tak an nei thei.A thleng thei a ni.

Heng ruh inthlak danglamna te nen hian vunah pawh inthlak danglamna i hmu thei bawk . Vun chu a thim deuh emaw, a hring hring emaw a awm thei a, a polih tawh ang maiin a lang thei bawk.

A tha ber chu meloriostosis hi ruh pakhat atanga ruh dangah a darh lo tih hi a ni. Chumi awmzia chu kut khata a awm chuan kut dangah a darh lo vang tihna a ni. Mahse, hun kal zelah a dinhmun hi a na zual deuh thei a ni. Chumi awmzia chu a natna a zual thei a, chetna pawh a tlem zawk thei tihna a ni.

He natna hi engtiang chiahin nge a hluar?

Meloriostosis hi a takah chuan natna hmuh tur awm lo tak a ni. Chutiang khawpa tlem a nih avangin mithiamte chuan khawvel puma mi maktaduai khat zinga pakhat velin he natna hi a vei niin an sawi. Chumi awmzia chu Sri Lanka-ah pawh heng damlote hi an tlem hle tihna a ni.

Engvangin nge he meloriostosis hi a lo awm? Eng nge a chhan?

He thu hi i hriat chuan mak i ti mai thei. Mi thenkhat tan chuan mi pahnih zinga pakhat vel, he natna vei te tan chuan a chhan chu genetic mutation a ni. Kan taksaah hian MAP2K1 tih gene kan nei a. He gene hian kan ruh cell te thanna control tu protein bik a siam a. Chuvangin, he MAP2K1 gene-ah hian inthlak danglamna, chu chu mutation a awm a nih chuan, he meloriostosis dinhmun hi a awm thei a ni.

A pawimawh ber chu he genetic variation hi sporadically-ah a thleng thin. Chu chu nu leh pate rochun thil a ni lo tihna a ni. Ngaihtuah teh, lottery ang maiin, he genetic variation hi mi pakhat chungah a thleng thei a ni.

Mahse, meloriostosis vei zawng zawng hian he MAP2K1 gene mutation hi an nei vek lo. He gene mutation nei lo mi dang pawh he natna vei an awm bawk. Chuvangin chutiang hunah chuan doctor-te chuan he natna thlentu dik tak hi an la hre thei lo. Chumi chungchangah chuan zirchianna dang neih belh zel a ni.

Meloriostosis natna lan chhuah dan chu engte nge ni?

Meloriostosis vei mi pahnih zinga pakhat vel chuan kum 20 an tlin hmain a lan chhuah tan a, a tam zawkah chuan heng natna hi naupan lai atanga tan a ni a, a châng chuan nau pian aṭanga ni engemaw zat chhungin a inṭan a ni . Hun a kal zel a, heng chhinchhiahnate hi a na zual thei a ni .

Heng symptoms te hi kut emaw ke emaw ah a langsar ber tlangpui. Mahse, a tlem hle a, nangmah emaw i fa emaw hian hmun dangah pawh heng natna hi i nei thei a, chu chu hip, rilru ruh lai tak (sternum), emaw, ribs emaw ah te pawh a ni thei. A lan chhuah dan tlangpui han thlir ila:

• Chronic pain: Hei hian natna rei tak awm thei tihna a ni. Hei hi mi tam tak tan chuan harsatna lian ber a ni.
• Vun thim emaw, eng emaw: Vun chu polish ang maiin a lang thei. A khawih pawhin a tight tih a hriat theih bawk.
• Kut emaw ke emaw a hring: He hliam hi tui inkhawl (edema) emaw, lymph node hring (lymphedema) emaw avanga lo awm thei a ni.
• Range of motion tihtlem a ni:Entirnan, elbow a ben kim theih loh emaw, khup a tizau kim thei lo emaw.
• Muscle atrophy: Kut emaw ke emaw a natna hmuna ruh te chu zawi zawiin a atrophy thei.
• Kut emaw ke sei zawng inang lo: Kut pakhat chu a dang aiin a tawi thei a, a ke pakhat chu a dang aiin a tawi thei bawk. Hei hian kea kal harsatna pawh a thlen thei a ni.

Engtin nge meloriostosis hi an hriat theih?

Doctor-te chuan meloriostosis hmuhchhuah nan special imaging test an hmang ber a ni.

Chung zingah chuan:

• Bone scans: Hei hi taksaah radioactive substance tlemte inject a, chu chu camera bik hmanga en a ni. Hei hian ruh chhunga thil dik lo awm apiang chu chiang zawkin a hmu thei a ni.
• X-ray: Hei hi i hriat chian a ni ngei ang. Radiation dose tlemte hmangin ruh leh soft tissue te a en thin. Meloriostosis vei mi pakhat X-ray i en chuan chu "dripping wax" hmel kan sawi tawh kha i hmu ang. Hei hi he natna hriatchhuahna atana chhinchhiahna pawimawh tak a ni.

Heng imaging test bakah hian, a châng chuan doctor-te chuan genetic testing pawh an rawt thei bawk . Hei hi thisen sample lak atanga tih a ni. Heng genetic test te hian a hmaa kan sawi tawh MAP2K1 gene mutation awm leh awm loh an zawng thin.

Hei hi eng enkawlna nge ni? Dam theih a ni em?

Dik tak chuan meloriostosis hi damdawi a la awm lo. Hei hi hriat chuan a lungchhiatthlak deuh mai thei. Mahse, lungngai suh. Nang leh i fate natna lan chhuah dan thunun a, nun tha zawka nunpui thei tur enkawlna chi hrang hrang a awm a.

Doctor-te chuan natna tihziaawmna tur leh taksa hnathawh that nan hetiang ang enkawlna hi an rawt tlangpui:

• Physical therapy: Hei hian taksa chakna tihpunna tur leh ruhkawrte chetna range tihpunna tur exercise leh treatment bik hman a ni. Entîr nân, i kut ben harsa i tih chuan, ṭanpui thei tûr exercise zirtîr i ni thei.
• Occupational therapy: Hei hian fine motor skills tihhmasawn nan a pui thin. Chu chu kut zungtang hmanga hna tenau tih te, button vuah te, ziak te, etc. Tin, naupang chu nitin hna hrang hrang, inbual leh inchei te, mahni inbualna leh incheina te tih theihna tura tanpui hi he enkawlnaah hian a tel a ni.
• Damdawi: 1.1.
• Painkillers: Natna tihziaawmna tur damdawi, non-steroidal anti-inflammatory drugs (NSAIDs) te hi pek thin a ni.
• Bisphosphonates: Hengte hi ruh tichak turin pek thin a ni.
• Inzai:Ṭhenkhatah chuan ruh dang lo piang thar chu lak chhuah emaw, ruh siam thar emaw nân operation neih a ngai mai thei. Amaherawhchu, hei hi enkawlna dangin hahdamna a pek loh phawt chuan tih a ni.

Pawimawh: Heng enkawlna zawng zawng hi mi zawng zawng tan a tha vek lo. I doctor chuan i dinhmun atan eng treatment nge tha ber tih a rel ang.

Meloriostosis lo awm tur ven dan tur a awm em?

Hei hi thu lungchhiatthlak deuh a ni. Because meloriostosis is a disease that occurs randomly, meaning it happens for no apparent reason , a lo awm loh nan engmah kan tih theih a awm lo tak zet a ni. Genetic mutation nena inzawm a nih avangin kan thunun theih loh thil a ni.

He natna hian hun rei tak chhunga nghawng a neihte chu engte nge ni?

Meloriostosis hi cancer a ni lo. Chuvangin ngaihtuahawm tur a awm lo. Tin, he natna hian i dam rei zawng a ti tawi lo bawk. Hei hi chanchin tha ber a ni.

Mahse, he natna hian taksa hnathawh leh che vel a tikhawtlai thei a ni. I ruh natna leh hliam ang chi thil nen i nunpui a ngai mai thei. Mahse, enkawlna ruahmanna dik tak a awm chuan meloriostosis vei te pawhin nunphung sang tak an hmang thei bawk. Chumi awmzia chu hlim takin an khawsa thei a, midang ang bawkin mahni hna an thawk thei tihna a ni.

Doctor hnenah eng nge zawh belh tur?

Nangmah emaw i fa emaw hian meloriostosis symptoms i neih chuan, emaw, he natna hi i vei tih hmuhchhuah a nih tawh chuan, i doctor hnenah hetiang zawhna hi zawh a pawimawh:

• "Meloriostosis natna lan chhuah hmasak berte chu engte nge ni?"
• "He natna hi dik taka hriat theih nan eng test nge ka tih ang ?"
• "Eng treatment option nge ka neih? Eng treatment nge ka tan a tha ber?"
• "He thil hi ka enkawl loh chuan eng nge thleng thei ang ?"
• "Meloriostosis natna avanga ka nunphung tihsan nan eng nge ka tih theih ang ?"

I dinhmun hriatthiamna tha zawk neih theih nan heng zawhnate hi zawt rawh. I doctor nen zalen takin inbiakna nei rawh.

Meloriostosis ang chi natna dang a awm em?

Ni e, meloriostosis natna lan chhuahna ang chi natna dang engemaw zat a awm bawk. I doctor chuan diagnosis a siam hunah heng conditions te hi a rule out ang. Chung dinhmun thenkhat chu:

• Buschke-Ollendorff natna a ni
• Desmoid tumor a ni
• Hemangioma natna (Hemangioma) a awm
• Osteopathy natna a ni
• Osteopoikilosis natna a ni
• Scleroderma a ni

Hei vang hian diagnosis dik tak siam a pawimawh em em a ni.

A tawp berah chuan thil hriat reng tur

Melorheostosis hi ruh natna hmuh tur awm lo tak a ni.Hei hi ruh awm tawh chungah ruh tissue thar a lo awm hian a ni. Kut pakhat emaw ke pakhat emaw a nghawng tlangpui. Ruh pakhat atanga ruh dangah darh lo mah se, hun kal zelah a natna hi a zual thei hle.

Meloriostosis i neih chuan hlauthawng suh. Enkawlna hrang hrang a awm a, chu chu damdawi, taksa lam enkawlna, hnathawhna lama enkawlna, emaw, surgery emaw te hi a ni. Heng enkawlnate hian natna a tihziaawm thei a, thil tih dan pangngaiah a kir leh thei a, nun tha zawk i nei thei bawk. A pawimawh ber chu symptom i neih chuan a rang thei ang bera doctor rawn la, i doctor thupek uluk taka zawm hi a ni.