I ruh leh vun tichhe thei natna hmuh tur awm lo tak chungchang i zir dawn em ni? (Maffucci Syndrome) tih hi a ni.

Natna mak tak mai, rang taka i ruh, leh a chang chuan i vunah harsatna thlentu i hre ngai em? I kut emaw, i ke emaw atanga ruh lo chhuak, i vunah sen emaw, sen emaw ang chi thil i hmu tawh mai thei. Chu chu vawiinah hian kan sawi dawn a, a buaithlak deuh a, a awm lo hle bawk. `(Maffucci Syndrome)` tih a ni. Lungngai suh, awlsam takin, i hriatthiam theih dan tur angin kan sawi dawn a ni.

Chuti a nih chuan, he Maffucci Syndrome hi eng nge ni?

A awlsam zawngin sawi ila, Maffucci Syndrome hi a awm lo hle a, i ruh leh vun a nghawng a ni. Thil pawimawh pathum a huam a:

1. I ruh chhungah cartilage a thang tam lutuk. He cartilage hi tissue khauh tak, flexible tak a ni a, i ruh ang chi hmun a khuh a ni. Chungte chu i ruh chhunga tumor-ah an lo thang lian chuan `enchondromas` kan ti thin. Hengte hi cancer (benign) an ni lova, a tir lamah chuan an hlauhawm lo tihna a ni. Mahse, heng `enchondromas` za tam tak, a nih loh leh sang tam tak pawh hi a lo awm thei a ni.

2. Heng `(enchondromas)` te hian ruh natna a thlen thin. Ngaihtuah teh, ruh chhungah thil ṭul lova a lo thang lian chuan chu ruh pianzia chu a danglam a ni lawm ni? Chutiang chu a ni. Kawng leh ke tawi te, inzawm khawm, leh ruh tliak ang chi thil a awm thei.

3. Hemangiomas chu thisen kalna kawng pangngai lo anga lang, vun chunglama inzawm khawm, lumps a ni. Chûngte chu a sen, a sen, a sen hring emaw a ni thei.

Heng cartilage growths, enchondromas an tih te hi i kut zungtang leh ke ruh ruh ah a awm tam ber. Mahse, a hnuaia mite hi an nghawng thei bawk.

• Arms a ni
• Ke te
• Ribs tih a ni
• Luruh a ni
• Vertebrae (vertebrae) 1. A rilru a hah lutuk chuan a rilru a buai em em a.

Pawimawh tak chu heng enchondromas te hi a tir lamah chuan cancer ni lo (benign) an ni a, mahse a chang chuan malignant (malignant) an ni thei bawk. Maffucci syndrome vei mi tam tak (zirchianna thenkhatah chuan 50% thleng) chu ruh cancer chi khat chondrosarcoma an tih, cartilage cell atanga intan an vei theihna a sang hle. Cancer chi dang vei theihna an nei sang hle bawk.

Maffucci Syndrome hi engtiang chiahin nge a awm?

Dik tak chuan hei hi thil awm lo tak a ni. Khawvel pumah hian medical record-ah hian case za tlemte chauh an awm a, chuvangin a hmaa i la hriat loh chuan thil mak a ni lo.

Maffucci Syndrome natna lan chhuah dan chu engte nge ni?

Maffucci syndrome symptoms hi mi hrang hrangah a inang lo thei. Mi thenkhat chuan symptoms na lo tak tak an nei thei a, thenkhat erawh chuan symptoms na zawk an nei thei thung. Heng symptoms te hi pian tirh atangin a awm thei a, naupan lai atang pawhin a lang tan thei bawk.

A lan chhuah dan ber chu kan sawi tawh cartilage tumor ``enchondromas'' an tih te hi a ni.

• Ruh natna
• Bone fractures – Ruh chu tlu tenau atang pawhin a chhe thei.
• Kut emaw ke emaw a bawkkhup
• Bulging bones – lumps angin a lang.
• Curved spine – scoliosis nen a inang.
• Osteolysis ( ruh tihchhiatna ) .
• A sei zawng tawi
• Kut emaw ke emaw inang lo – pakhat chu a dang aiin a sei emaw, a tawi emaw a lang.

Thisen kalna kawng tumor, hemangiomas tia hriat hian natna lan chhuahna a thlen thei a, chungte chu:

• Thisen a chhuak thin
• Lymphangiomas – Hei hi lymph fluid-a khat lump ang mai a ni.
• Vunah sen, purple, emaw blue bumps (hemangiomas) – kut ah hmuh tam ber. Hun kal zelah hengte hi a khauh thei a, a hring thei bawk.
• Muscles lo thang lo – a natna hmunah.

Eng thilin nge Maffucci Syndrome hi a thlen?

Maffucci syndrome hi genetic disorder a ni a. Chumi awmzia chu kan genes inthlak danglamna (mutation) avanga lo awm a ni tihna a ni. A bik takin, `IDH1` (a tam ber) emaw `IDH2` (a tlem ber) emaw an tih gene pahnih zinga pakhat random change avanga lo awm a ni. Zirchiangtute chuan he genetic mutation hi nausen pum chhunga a la awm lai, chu chu a pian hmaa lo awm niin an ngai. Heng `IDH` genes te hian kan taksa chhunga cell hnathawhna atana pawimawh `(enzymes)` siamchhuahnaah a pui thin. Mahse, scientist-te chuan heng `(enzymes)` leh Maffucci syndrome inzawmna dik tak hi an la hre thei lo.

A pawimawh ber chu hei hi thlahtu natna a ni lo tih hi a ni. Chu chu, nu leh pate’n an neih avangin thlah khat atanga thlah danga inthlunzawm theih thil a ni lo tihna a ni . Hei hi random genetic mutation a ni.

Maffucci Syndrome hi engtin nge an hriat theih?

Heng test te hian doctor chu Maffucci syndrome a hriat theih nan a pui thei a ni:

• Taksa enfiahna: Doctor chuan a enfiah ang che a, chhinchhiahna hmuh theih (lump emaw deformities emaw ang chi) a zawng ang. I natna lan chhuah dan leh i chhungte zingah hetiang dinhmun hi an nei tawh em tih an zawt bawk ang.
• X-ray emaw CT scan emaw: Hengte hian i ruhte thlalak chiang tak a la thei a ni. Chumi hnuah chuan enchondromas a awm em tih te, eng chen nge a darh tih te, ruh chhiatna a awm em tih te an hmu thei a ni.
• Surgery hmanga tissue sample lak leh enfiah (`Biopsy`):A châng chuan rinhlelhna hmun (ruh emaw vun hring emaw ang chi) aṭangin tissue te tak te chu operation hmangin lakchhuah a ni a, microscope hmangin an enfiah ṭhin. Hei hian a dikna (definitive) a siam a ni.

Eng ang specialist nge Maffucci Syndrome hi enkawl?

Maffucci syndrome i neih chuan specialist hrang hrang team an rawn kalkhawm thei a, an enkawl thei che a ni. Entir nan:

• Dermatologist: Vun lo thang lian tham tak tak, hemangiomas ang chite enkawl, tihtlem emaw, tihbo emaw a pui thin.
• Orthopaedic surgeon: Ruh ruh tliak a enkawl a, ruh tliak te chu surgery hmangin a siamthat a, enchondromas te pawh surgery hmangin a paih chhuak thei bawk.
• Orthopaedic oncologist: Kan sawi tawh ang khan chondrosarcoma ang cancer a lo awm a nih chuan heng doctor te hian a paih chhuak a, a dam chhung zawngin natna hi a lo lang leh em tih enfiah turin an pui thin.
• Radiologist: Medical imaging, X-ray leh CT scan-ah te an specialise a, hun kal zelah ruh inthlak danglamna tehnaah an pawimawh hle.

Maffucci Syndrome hi engtin nge enkawl a nih?

Dik tak chuan Maffucci syndrome hi tihdam kim theih dan tur a la awm lo. Chuvangin, enkawlna tum ber chu:

• Cancer hmuhchhuah hmasak leh enkawlna tha zawk pek.
• Ruh tliak ven emaw enkawl emaw.
• Natna ang chi symptoms tihtlem.

I medical team chuan i ruhte enfiah nan medical imaging test (X-ray ang chi) an order fo ang. Enchondromas emaw ruh lama harsatna emaw hian nitin hnathawhna a tibuai a nih chuan enkawlnaah hian:

• Brace vuah emaw, scoliosis siamthat nan operation neih emaw.
• Ruh tliak te chu cast, splint leh surgery te a ni.
• Ke sei zawng inang lo chu kekawr bik (kekawr chawi), damdawi emaw, surgery emaw hmangin tihtlem rawh.
• Kut a deformities siamthat nan surgery neih a ni.
• (Enchondromas) te hi surgery hmanga lakchhuah theih a ni. Mahse, hengte hi a lo thleng leh thei a ni.

I vunah hemangiomas te hian a tibuai a nih chuan i doctor chuan sclerotherapy tih damdawi a rawn rawt thei ang. Hei hian vunah solution inject a, chu chuan a lo thang lian te chu a tihtlem phah a ni. A tul chuan hemangiomas te hi surgery hmangin lakchhuah theih a ni.

Maffucci Syndrome hi ven theih a ni em?

Kan sawi tawh ang khan scientist-te chuan hetiang thil thlentu genetic mutation hi engtin nge a lo awm tih chiang takin an la hrethiam lo. Chuvangin tun dinhmunah chuan ven dan tur bik a awm lo.

Maffucci Syndrome vei hmalam hun chu eng nge ni?

Maffucci syndrome hi dam chhung zawnga natna awm thei a ni. Mi thenkhat chuan taksa lama harsatna tenau te chauh an nei thei a, chu chuan an nitin hnathawhnaah nghawng lian tham a nei lo mai thei. Mahse, mi dangte tân chuan ruh lama harsatna chu a na zual thei a, taksa chak lohna pawh a pung thei bawk.

Pawimawh tak chu he dinhmun hian finna a nghawng lo. Mahse, cancer a lo awm chuan dam rei theihna a ti tawi thei tihna a ni.

Maffucci Syndrome vei angin engtin nge ka in enkawl ang?

Maffucci syndrome vei te hi cancer vei theihna a sang zawk avangin i medical team te rawtna angin screening neih fo a pawimawh hle. Cancer hmuhchhuah hma poh leh enkawl a nih theihna a sang zawk.

Ollier Syndrome leh Maffucci Syndrome hi eng nge an danglamna?

Ollier syndrome leh Maffucci syndrome te hi cartilage tumor enchondroma an tih te lo awm thin a ni. Mahse, Maffucci syndrome-ah chuan thisen kalna kawnga tumor, hemangiomas an tih pawh hmuh tur a awm bawk. Ollier syndrome hian hemangiomas a nei lo. Chu chu a danglamna ber chu a ni.

Maffucci Syndrome tih hming dang a awm em?

A tlem em avangin a natna hi a chang chuan hming dangin an ko thin a, mahse chutiang chuan an awm lo. Entirna thenkhat chu hetiang hi a ni:

• `(Chondrodysplasia leh hemangioma)`
• `(Dyschondroplasia leh cavernous hemangioma)`
• `(Enchondromatosis leh hemangiomata)`
• `(Kast syndrome)` tih a ni

Hetiang hming hi tam tak awm mah se, a hming hman tam ber chu Maffucci Syndrome a ni.

A tawp berah chuan take-home message

Nangmah emaw i fa emaw hian Maffucci Syndrome i vei tih hmuhchhuah a nih chuan a buaithlak deuh thei. I dam chhung zawngin ruh tliak leh ruh cancer chungchangah i buai a ngai a ni. Specialist hrang hrang hmuh leh test neih fo pawh a hahthlak thei bawk.

Mahse, hetianga fimkhur hi, a bik takin cancer test hmasak hian i nunna chhanhimna emaw, tihzauh emaw kawngah kawng thui tak a zawh thei a ni. Cancer natna hi a hmaa hriatchhuah leh enkawl hi i hriselna tihchangtlun nan a tha ber a ni. I doctor chuan eng symptom nge fimkhur tur tih a hrilh ang che.

Lungngai suh. Nangmah chauh i ni lo. Heng natna hlauhawm tak nei te tanpui leh kaihruaitu tur doctor leh support group an awm a. A pawimawh ber chu information dik tak la chhuakin doctor thurawn zawm hi a ni.

` Maffucci syndrome, ruh natna, cartilage, hemangioma, cancer hlauhawmna, genetic natna, natna tlemte