Leber Hereditary Optic Neuropathy (LHON) chungchang hi a awlsam zawngin i zir dawn nia!

I mit a tlahniam zauh zauh a, engkim a fiah lo tan tawh em? Mit pakhatah a intan a, thla engemaw zat hnuah mit dang pawh he natna hi a lo ni ta mai thei em? Heng thilte hi i ngaihtuah nasa hle a ni ngei ang. Vawiin hian natna hlauhawm tak, heng natna lan chhuah tir thei, mahse Leber Hereditary Optic Neuropathy, emaw ``(LHON)`` an tih chungchang kan sawi dawn a ni.

Leber hereditary optic neuropathy (LHON) hi eng nge ni?

A awlsam zawngin sawi ila, Leber hereditary optic neuropathy, a tawi zawngin LHON tih hi genetic condition a ni a, thlah khat atanga thlah dangah a inthlak danglam zel a ni. Thil thleng ber chu i mit i hloh hi a ni. Mi tam zawkin an rochun chuan an mit a fiah tan a, thla ruk vel chhungin a chhe zauh zauh a ni. A chang chuan, mit pakhatah a intan a, thla engemaw zat hnuah mit dang a nghawng leh thin. A tlangpuiin naupan lai emaw, puitling naupang emaw atanga tan a ni. Vanduaithlak takin LHON vei tam tak chu dan anga mitdel an ni thei. Chumi awmzia chu dan anga mitdel anga ngaih an nihna thlengin an mit a tlahniam tihna a ni.

Leber natna an ti bawk. Chu chu a zirchiang hmasa bertu doctor chu Dr. Theodore Leber a nih vang a ni. "Hereditary" tih thumal hian i rochun thil a ni tihna a ni . "Optic neuropathy" tih hian i mit nerve tichhe thei natna a ni tihna a ni . I optic nerve hi camera atanga i thluak thlenga cable kal ang a ni. I mit hmanga i thil hmuh, chu chu visual signals te hi he optic nerve hian i thluak lamah a phur a ni. Chutah chuan thluak hian "a hmu" thin. Chuvangin, he optic nerve hi a chhiat chuan chu chu i mit hmuhna i hloh theihna kawng lian ber pakhat a ni.

LHON chi hrang hrang a awm em?

Ni e, LHON hian i mit nerve a nghawng ber a nih chuan, chu chu mit hmuh lohna chauh a ni tihna a nih chuan, chu chu standard LHON condition an ti a ni. Hei hi Leber natna vei tam zawkte dinhmun a ni.

Mahse, a tlem hle , mi thenkhat chuan LHON nen hian symptom dang an nei thei bawk. Chungte chuan an nervous system hmun dang a nghawng thei a, a chang chuan an thinlung pawh a nghawng thei bawk. Doctor-te chuan he natna hi "Leber plus" tiin an sawi. Hei hi a buaithlak deuh a, a chhan chu mit lama harsatna chauh ni lovin hriselna lama harsatna dang a thlen thei a ni.

LHON hi engtiang chiahin nge a awm?

LHON hi engtiang chiahin nge a hluar tih hriat a la ni lo. Mahse, chhut dan chuan mi 25,000 atanga 50,000 zinga pakhat chuan he natna hi a nei thei a ni. Tin, LHON vei zinga 80% atanga 90% vel chu mipa an ni tih pawh hriat tur a ni .

LHON (Leber natna) natna lan chhuah dan engte nge ni?

`(LHON)` hian mitdelna chu na lo leh zawi zawiin (subacutely) a thlen thin., chu chu thla engemaw zat chhungin zawi zawiin a thleng tihna a ni. I hriat hmasak ber tur inthlak danglamna chu i central vision ah a ni . Central vision hi hmalam i thlir dik chuan i hmu thin - lirthei khalh nan te, lehkhabu chhiar nan te, hmel hriat nan te i hman thin vision hi a ni. I central vision chu a fiah lo tan thei a, i field of vision lai takah chuan mitdel ang maiin hmun dum i nei thei bawk. Hei hi mit pakhatah a intan a, mit dangah a darh thei bawk.

Hetiang dinhmun hi thla engemaw zat chhungin a zual zel dawn a ni. I rawng hmuh theihna pawh i hloh tan thei bawk - chu chu rawng thenkhat i hmu lo emaw, i thliar hrang thei lo emaw tihna a ni. Mitdelna hi a lan chhuah hmasak ber atanga thla ruk atanga kum khat chhungin a nghet tlangpui. Hetih lai hian mi tam zawk mitdelna chu 20/200 emaw a aia na zawk emaw a ni thei a, chu chu dan anga mitdel nihna level a ni. Light perception engemaw zat i la nei mai thei a, mahse mitdelna neia nun i zir a ngai dawn a ni. Ngaihtuah teh, khawvel hi a rawn inher chhuak nghal mai ang maiin a lang.

"Leber's plus" symptoms te chu engte nge ni?

Kan sawi tawh ang khan "Leber's plus" vei te hian mitdelna bakah hian symptom dang chi hrang hrang an nei thei bawk. Chung zinga ṭhenkhat chu:

• Movement disorders , entirnan tremors.
• Balance leh coordination lama harsatna (ataxia) . Kal laiin staggering ang chi, emaw thil man thiam loh ang chi.
• Cardiac conduction problems , thinlung thawk mumal lo (arrhythmias) ang chi te.
• Multiple sclerosis (MS) natna lan chhuah dan , taksa ruh chak lohna leh hah lutuk te.

Leber hereditary optic neuropathy hi engte nge ni?

Leber-a hereditary optic neuropathy hi mitochondrial natna a ni a . I cell chhunga mitochondria kaltlangin i rochun genetic disease a ni. Tunah chuan mitochondria hi eng nge ni tih i ngaihtuah mai thei. A awlsam zawngin, mitochondria hi i cell chhunga power generator te tak te ang mai an ni. Oxygen leh nutrient te an la a, i cell te hnathawh theihna tura an mamawh chakna an siam chhuak thin. Kan in chakna pe thei generator ang mai a ni.

Chuvangin, mitochondrial natnaah chuan he chakna siamtu kalphung hi a tibuai a ni. Tichuan cell te hian an mamawh energy an hmu lo. Chumi avang chuan cell thenkhat chuan hna an thawk tha tawh lo thei a, an thi hial thei bawk.

I mitochondria hnathawh that loh chuan i taksa peng mitochondrial energy rinchhan berte hian a nghawng chu an hre hmasa ber thin. Chung zingah chuan i mit peng hrang hrang, a bik takin i mit nerve te hi a pawimawh ber a ni.. Mitochondria chhia i neih tlem chuan heng part te hian an hnathawh that theihna tura an mamawh energy an hmu lo. Hei hi Leber natnaah hian a thleng thin. Chumi rah chhuah chu i mit nerve chu a chhe zauh zauh (`a chhe`) . Hei hi `(LHON)` hmanga i mit i hloh dan chu a ni.

Engtin nge LHON hi rochun a nih?

LHON hi i mitochondria-a DNA-a gene mutation avanga lo awm a ni. A bik takin, gene MT-ND1, MT-ND4, MT-ND4L, emaw MT-ND6-a mutations hian LHON a thlen thin.

Pawimawh tak chu i mitochondria zawng zawng hi i pa hnen atangin ni lovin i nu hnen atangin i hmu vek a ni . Chuvangin nute chauh hian he mutation hi an fate hnenah an pe chhuak thei a ni. Pa berin natna a nei a nih pawhin he mutation hi a fate hnenah a pe chhuak lovang.

Mahse, he gene mutation kengtu zawng zawng hian `(LHON)` symptoms an nei vek dawn lo. Chuvangin mi thenkhat chuan he gene mutation hi an keng tel tih pawh an hre lo mai thei. Hei hi a buaithlak deuh a ni lawm ni? Chumi awmzia chu nuin he gene mutation hi nei mahse naupang hian symptoms a nei thei a, a nei lo thei bawk.

LHON vei theihna tur risk factor a awm em?

Hei pawh hi zawhna pawimawh tak a ni. Gene mutation nei mi thenkhatin `(LHON)` symptom an neih a, thenkhatin an neih loh chhan hi zirchiangtute chuan an la hre chiang lo.

Mahse, evidence thenkhat chuan physiological stress leh environmental toxins te hian symptoms lo awm tanna kawngah a pui thei tih a tarlang. Hei hian a awmzia chu heng pawn lam thilte hian genetic mutation avanga stress nei tawh system-te chu stress dang a belhchhah tihna a ni. Hun a kal zel a, ngaihdan chu heng stress te hi a pung zel thei a, a tawpah chuan symptoms a thlen thei a ni.

Risk factor ni thei thil thenkhat chu hetiang hi a ni:

• Meizial zuk.
• Zu in hman dan.
• Environment toxins (entir nan, pesticide thenkhat, industrial chemicals) nena inzawmna .
• Systemic illnesses dang neih te.
• Psychological stress nasa tak a awm .

Engtin nge LHON hi hmuhchhuah a nih?

I mit enkawltu specialist chuan standard eye exam hrang hrang a nei ang a, i mit hmuh dan a enfiah ang a, harsatna awm chhan a zawng ang. A tir lamah chuan i mit emaw, mit nerve emaw-ah thil dik lo an hmu thei lo mai thei a, chu chu natna lan chhuah tan atanga thla ruk chhungin a ni. A chhiatna hi a lan chhuah tirh atanga thla ruk hmasa berah a langsar zual thin.

I doctor chuan LHON a rinhlelh chuan genetic testing neih a rawt ang . Hei hi kan sawi tawh ang khan genetic mutations pakhat i nei em tih chiang taka hriat theihna awmchhun a ni. He test hi diagnosis finfiah theihna awmchhun a ni.

LHON hi enkawlna emaw, damdawi kimchang emaw a awm em?

Vanduaithlak takin tun dinhmunah LHON damdawi a la awm lo. Tuna FDA-in damdawi a pawmpui awmchhun chu Coenzyme Q10 synthetic form Idebenone tih a ni. Randomized controlled trial-ah chuan LHON vei te mit hmuh danah hmasawnna engemaw zat a awm tih hmuhchhuah a ni. Hetiang damdawi dang pawh hi research phase-ah a awm mek bawk.

Researcher-te chuan gene therapy , nakin lawka LHON enkawlna atana hman theih tur chu an zirchiang mek bawk. Mahse hengte hi research stage-ah an la awm a ni. Chuvangin, tun dinhmunah chuan damna famkim beisei a harsa hle. Mahse, a natna lan chhuah dan enkawl dan tur leh mit hmuhna tlahniam zel tur chu eng emaw chen chu thunun theih dan a awm.

LHON nen hian eng thlirna nge an neih?

LHON avanga mitdelna a awm chuan a rang thei a, a na hle thei a, a tam zawkah chuan a awm reng thei bawk . Mi tam tak chuan mitdelna neia nun an zir a ngai a ni. Mitdelna hi mitdelna (moderate to severe degree) a ni a, mahse mitdelna famkim tihna erawh a ni lo. Moderate low vision hi mitdelna test 20/70 a ni. Mitdelna na tak chu mitdelna test 20/200 aia tlem lo a ni. Hei hi a takah chuan a tawpah chuan i hmuh theih tur visual acuity range zau tak a ni.

He range-a moderate emaw severe emaw lam i nih leh nih loh hi vanneihna thil a ni fo. Enkawlna hian danglamna a thlen thei a, mahse gene mutation i neih hian danglamna lian ber a siam a ni. LHON vei thenkhat chuan kum khat vel zet an mit an hloh hnuah beisei loh takin an mit hmuhna thenkhat an nei leh thin. Hetiang mitdelna hi gene mutation hrang hrangah hian a danglam thei a ni. Mahse, mit hmuhna engemaw chen i nei leh a nih pawhin, mitdelna neiin i la nung reng a ngai ngei ang.

LHON laka inven dan tur a awm em?

LHON nena inzawm gene rochuntu tam zawk chuan mitdelna an nei ngai lo nain, tunah chuan ven dan tur hriat a la ni lo. Antioxidants ei leh zu leh zuk leh hmuam ang chi neurotoxins ei loh hian i hlauhawmna a ti tlem thei a, mahse hei hi finfiah a la ni lo.

Hmeichhia heng genes nei zawng zawng hian an fate hnenah an pe chhuak vek dawn tih hre reng ang che. Genetic counseling hian he hlauhawmna hi ngaihtuah turin a pui thei che a ni. I chhungkuaa tu emaw hian he natna hi a vei a nih chuan, emaw, he gene mutation hi i nei tih i hriat chuan chhungkaw din hmain genetic counselling lak hmasak a pawimawh hle.

LHON chhungkaw chanchin i hriat emaw, i tan beisei loh tak tak emaw pawh nise, i mit hmuhna hloh nghal mai chu thil hlauhawm leh thinlung ti na thei tak a ni thei. Nang leh i doctor hian a tir lamah chuan thil thleng chu i hrethiam lo mai thei. Mahse, i hriat hnu chuan i thil tak tak thar i insiamrem rualin zir tur tam tak i nei ang.

He zinkawngah hian nangmah chauh i ni lo tih hre reng ang che - i tanpui thei tur resources khawvel a awm. Mitdelna nei lote tan pawl, hmanrua leh rilru lam hriselna lama puihna a awm a.

A tawp berah chuan thil hriat reng tur

Okay, chuvangin kan sawi `(LHON)` chungchangah hian ngaihdan i neih ka beisei. Hei hi thupui buaithlak deuh a ni a, mahse hriat chian a pawimawh hle.

• (LHON) hi thlahtu natna a ni a, mit nerve a tichhe a, mit a tichhe thei a ni.
• Hei hi mitochondrial DNA-a genetic mutation avanga lo awm a ni a, chu chu nu atanga naute hnena rochun a ni .
• Naupan laia natna lan chhuah dan a lang tlangpui a, natna engmah awm lovin mit a tlahniam zauh zauh thin.
• Mit hmuh bakah hian nervous system lama harsatna dang pawh "Leber plus" an tih condition ah hian a awm thei bawk.
• Meizial leh zu ang chi thilte hi natna lan chhuahna atana risk factor a ni thei.
• Tunah hian damdawi chiang tak a la awm lo a, mahse `Idebenone` ang damdawi leh mitdelna nena insiamrem dan tur a awm.
• Hemi chungchangah hian rinhlelhna i neih chuan mit lam doctor pan ngei ngei tur a ni a, genetic test pawh ti ngei ang che.
• Hetiang dinhmun nena nun hi a harsa thei a, mahse nangmah chauh i ni lo, tanpuina la rawh.

He thu hi in tan a tangkai ngei ka beisei. Hemi chungchanga zawhna dang i neih chuan doctor biak hreh suh. Hrisel takin awm rawh!

` Leber hereditary optic neuropathy, LHON, mit hmuhna hloh, mit nerve, genetic mutations, mitochondrial natna, hereditary blindness