Naupang hrisel tak, tlan kual vel leh infiam lai te, zawi zawiin kea kal emaw, thusawi emaw harsat, emaw, an nungchang pawh zawi zawiin a inthlak danglam tih i hmu tawh mai thei. Nu leh pa i nih angin, a tir lamah an che tha hle a, chumi hnua an fate theihna chu hun kal zelah a tlahniam zel ang tih an hriat lai khan, i hlauhthawnna leh lungkhamna i neih chu tawngkam hmanga sawi chhuah a harsa hle. Hetianga a lo awm chhan hi natna hmuh tur awm lo, tumahin kan la hriat ngai loh a ni mai thei. Vawiin hian chutiang natna pawl khat kan sawi dawn a ni. Chu chu leukodystrophy a ni .
A awlsam zawngin, leukodystrophy chu eng nge ni?
Leukodystrophy hi natna pakhat a ni lo. Kan central nervous system (chu chu thluak leh ruhro) tichhe thei natna pawl a ni. Doctor-te chuan a chungchangah thil thar an hmuchhuak reng a ni. Tunah hian he natna chi hrang hrang 52 vel hmuhchhuah a ni tawh a.
Heng leukodystrophies chi hrang hrang tam tak hi genetic a ni . Chu chu nu leh pa atanga fate rochun an ni tihna a ni. A châng chuan naupang hian a pian hnu lawkah natna lan chhuahna a lantîr thei a ni. Mahse, naupang thenkhat chu natna kaihhnawih gene defect neiin an piang thei a, mahse a tir lamah chuan an hrisel vek angin an lang thei.
A pawimawh ber chu heng dinhmunte hi a kal zel a, chu chu naupang a tir lama hrisel tak chuan a hnuah symptoms a nei thei tihna a ni.
He natna lan chhuah dan engte nge ni?
Heng leukodystrophy chi hrang hrangte hian thil pakhat an inang vek a, chu chu thluaka myelin sheath a tichhe vek a ni.
Kan in chhunga electric wire awm chu han mitthla teh. A chhunga copper wire vel chu plastic sheath a awm a ni lawm ni? Chu chu electric chu a kalna tur hmun dik takah a kal thei a, a leak lo. Chutiang bawkin kan nerve te hian an chhehvelah hian ‘myelin’ an tih, thau var (white fatty sheath) an nei a. He myelin sheath hian nerve message dik tak leh rang takin thluaka a phur a, thluak atanga taksa peng hrang hrangah a phur bawk. He myelin sheath hi a chhiat chuan kan nervous system hian hna a thawk tha thei lo.
Leukodystrophy chi tin hian he myelin sheath hi kawng hrang hrangin a tichhia a, chuvangin naupangte’n harsatna an tawh theih dan pawh a inang lo thei hle.
| Area of symptoms awmna hmun | Harsatna tlanglawn hmuh tur awm |
|---|---|
| Taksa inthlauhna leh che vel | Kea kal harsa, tlu fo, taksa thunun theih lohna. |
| Nungchang leh zirlai | Zirna lama harsatna, hriatrengna hloh, nungchang inthlak danglamna. |
| Hmasawnna leh hmasawnna | Kum nena inmil hmasawnna lantir loh (e.g., thusawi tlai, kea kal). |
| Hriatthiamna (senses) hnathawh dan | Zawi zawiin hriatna, mit hmuhna leh tawng thiamna tlahniam. |
| Nerve nena inzawm harsatna dangte | Thâwk harsa, zun control lama harsatna, seizures. |
Entirnan, Canavan disease , tih chi khatah chuan naupang kawr ruhte chu a tichak lo va, mahse an kut leh ke chu a nghet tlat thung. Tin, an mit a hloh thei a, seizures an nei thei bawk.
A chang chuan pian tirh atangin symptoms a lang thin. Mahse Refsum Disease ang chi type ah chuan kum 20 vel ah hian symptoms hi a lang thin a, a chang chuan kum 50 vel atang pawhin a lang thei a, hei hi retina chak lohna, hriatna hloh, leh rim hloh te ang chi te hi a lang thei a ni.
A natna lan chhuah dan a danglam em avangin he natna hi hriat chian a harsa deuh thei.
Engvangin nge hetiang natna hi a lo awm? Eng nge a chhan?
Kan sawi tawh ang khan heng natna tam tak hi genetic condition a ni. Chumi awmzia chu nu emaw, pa emaw, a pahnih emaw atanga lo chhuak genes chhiatna avanga lo awm an ni tihna a ni.
Mahse chi thenkhat chu rochun a ni lo. Genetic mutation thar avanga lo awm an ni. Entirnan, Alexander natna hi rochun a ni lo niin a lang. Chumi awmzia chu nu leh pa pahnih zingah hian gene chhia chu nei lo mah se, naupang chuan a la nei thei tho tihna a ni.
Chuvangin chhungkaw khata naupang pakhatin leukodystrophy a vei theih laiin naupang dangte erawh chuan an nei lo mai thei.
I fate zinga pakhatin leukodystrophy a vei a, fa dang i beisei a nih chuan genetic counseling lak a pawimawh hle . Hei hian hei hi leh nakin lawka fa neih theihna tur hlauhawmzia i hriatthiam theih nan a pui ang che. Hemi chungchang hi i doctor nen inbia ang che.
Engtin nge doctor-te hian he natna hi dik taka an hriat theih?
He natna hi hriatchhuah hi a buaithlak deuh a, a chhan chu a natna lan chhuah dan a inang lo hle. Chuvangin Doctor ten test chi hrang hrang an tih a ngai a ni.
| Test chi hrang hrang | A awlsam zawngin... |
|---|---|
| Thisen leh zun test a ni | Taksa chemical process-a thil dik lo awmte hriatchhuah. |
| MRI scan a ni | Myelin sheath a chhiat leh chhiat loh enfiah nan thluak leh nervous system thlalak chiang tak, chipchiar tak tak lak. |
| CT scan hmanga tih a ni | Thluak structure inthlak danglamna hriat theihna tur imaging test dang. |
| Genetic testing neih a ni | Natna thlentu genetic defect bik hriatchhuah. |
| Rilru leh finna fiahna | Naupang ngaihtuahna, zirlai leh hriatrengna te fiahna. |
Hei hi enkawl dan tur a awm em?
Dik tak chuan leukodystrophy chi tam zawk hi damdawi chiang tak a la awm lo . Amaherawhchu, tih theih a awm lo tihna erawh a ni lo.
Enkawl lai hian doctor-te chuan natna lan chhuah dan control leh naupang nunphung a tha thei ang bera vawn that hi an ngaih pawimawh ber a ni. Enkawlna chu naupangin leukodystrophy a neih chi hrang hrangah a innghat a ni.
Enkawlna hman tlanglawn tak takte chu:
- Symptoms control nan damdawi pek (e.g., seizures control nan).
- Enkawlna bik, physical therapy, occupational therapy, leh speech therapy , te hian naupang chu a kal theihna te, nitin hna a thawh theihna te, leh a thusawi te a tichak thei a ni.
- Naupang thenkhat chuan zirlai leh ei leh in lama tanpuina dang an mamawh mai thei .
A then phei chuan ruhro (bone marrow transplant) hian natna zual zel chu a tikhawlo thei a, a titawp thei bawk. Scientist-te pawhin enkawlna thar, gene therapy -te chu an zirchiang mek bawk.
Heng zawng zawng nen hian a pawimawh ber chu i doctor nen inbiak reng a, a thupek zawm hi a ni.
Take-Home Thuchah a ni
- Leukodystrophy hi genetic diseases rare group a ni a, thluaka nerve covering (myelin sheath) tichhe thei a ni.
- Naupang chu a tir lamah a hrisel a nih pawhin hun kal zelah a natna lan chhuahna a lo lang zauh zauh thei a, a natna chu a zual zel thei bawk.
- I fa kal dan, tawngkam, mit hmuh leh nungchangah chhanna awm lova a chhe tih i hriat chuan doctor pan nghal vat rawh.
- A chi tam zawk tan chuan damdawi chiang tak awm lo mah se, a natna lan chhuah dan enkawl dan tur leh naupang nunphung tihchangtlunna tur enkawlna a awm a ni.
- Hlauhna, rinhlelhna, zawhna i neih apiang chu i doctor nen zalen takin sawipui rawh.
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