I taksaah hian copper i nei tam lutuk em? Wilson Disease chungchang hi i zir chiang ang u!

A châng chuan i chau mai mai em? A nih loh leh, chhan awm lovin chak lohna, pum nuam lo, leh rilru buai ang chi thilte i tawng em? Natna mak tak tak, kan ngaihtuah phak loh khawpa taksa khawihtu a awm a. Vawiin hian natna chi khat, a tlem deuh thu kan sawi dawn a, mahse uluk taka hriattir i nih chuan i hre hmasa thei a, i nun a tihchhiat theih dan tur chu i ti tlem thei ang. Hei hi Wilson Disease an ti a .

Wilson Disease hi eng nge ni?

A awlsam zawngin sawi ila, Wilson’s Disease hi genetic condition hmuh tur awm lo tak a ni a, kan taksa hian a mamawh aia tam copper a khawlkhawm thin. He copper hi kan thin leh thluak ah a awm khawm thin. Kan taksa hian hrisel taka a awm theih nan ei tur atanga copper tlemte a mamawh tak zet a ni. Mahse, Wilson’s Disease vei tu emaw enkawl that loh chuan taksaa copper level chu hlauhawm takin a sang thei a, nunna atana hlauhawm taksa peng hrang hrang a tichhe thei a ni.

Wilson natna hian tute nge a tuar ber?

Wilson natna hi nu leh pa atanga fate rochun natna a ni . Chu chu a lo neih theih nan chuan naupang chuan nu leh pa pakhat zel hnen atangin gene pangngai lo pakhat a dawng tur a ni. Hei hi autosomal recessive inheritance an ti a. Tuin nge tihhmasawn ang tih chiang taka sawi a har hle. Hei hi a chhan chu a tam zawkah chuan nu leh pate hian an taksaah he gene danglam tak hi an nei tih hriatna engmah an lantir lo. Chuvangin he gene hi ‘carrier’ an ni tih an hre lo. Amaherawhchu, i chhungte hnai berin he natna hi a vei a nih chuan nang pawhin i hlauhawm thei bawk.

Wilson natna hi engtiang chiahin nge a hluar?

Hei hi natna hmuh tur awm lo tak a ni . Mi 30,000 zinga pakhat velin he natna hi an vei nia chhut a ni. Amaherawhchu, chhungkuaa tu emaw hian he natna hi a neih chuan midangte vei theihna chance a sang zawk thin. Mi tam zawk pawhin gene dik lo pakhat chauh an nei a, chu chu carrier an ti a ni. A tlangpuiin symptoms an nei lo a, mahse an fate hnenah natna hi an kai thei a ni. Carrier-te hian symptom an neih loh avangin mipui zingah hian mi engzat nge gene dik lo nei tih chiang taka sawi a harsa hle.

Wilson natna hian engtin nge ka taksa a nghawng?

Wilson natna hi hriat hmasak leh enkawl a nih loh chuan nunna atana hlauhawm harsatna a thlen thei a ni . Copper hian taksaah toxic level thlengin a pungkhawm a, a bik takin liver leh thluak-ah a pungkhawm nasa hle. Hei hian heng taksa peng hrang hrangte hi a tichhe thei a ni. Taksa a copper level a san chuan i rilru put hmang chu a danglam thei a ni. I chau hle a, i chak lo hle a, natna leh natna i nei thei bawk. Chak lohna, hahna, emaw, natna nei reng reng i neih chuan doctor thurawn lak a tha ber.

Wilson natna lan chhuah dan chu engte nge ni?

Wilson natna lan chhuah dan hi mi hrang hrangah a inang lo thei hle.Hei hi pianpui natna ni mah se, a lan chhuahna chu liver, thluak, mit emaw, taksa peng dang emawa copper a lo awm hnuah a lang tan a ni. Wilson natna vei te hian kum 5 leh 40 inkar ah symptoms an nei tlangpui a, mahse mi thenkhat chuan kum naupang zawk emaw, kum upa zawk emaw ah symptoms an nei thei.

Hriat reng tur chu, a chang chuan heng symptoms te hi thin natna dang emaw, rilru lam harsatna emaw nen a inang a, chuvangin miten thil dangah an ngai sual thei a ni. I copper level test i neih hma chuan Wilson natna a ni em tih chiang taka hriat a harsa thei.

Liver nena inzawm natna lan chhuah dan

Wilson natna vei tam tak chuan hepatitis symptoms an nei thin. Tin, sudden liver failure an nei thei bawk. A lan chhuah dan chu:

• Chau reng reng.
• Nausea leh luak chhuak.
• Chaw chu a thlum lo.
• Pum dinglam, liver chungah chiah natna.
• Zun dum.
• Stool rawng hring hring.
• Mit leh vun dum (jaundice) a rawn sen (jaundice).

Mi thenkhat chuan Wilson natna hian chronic liver disease leh cirrhosis ang chi complication a neih chuan symptoms an nei thei chauh a ni. Chung zingah chuan:

• Chauhna leh chak lohna reng reng.
• Ngaihtuah phak loh khawpa taksa rihna tihtlem.
• Pum chhunga a hring (ascites).
• Ke hnuai lam, ke ruh leh ke ruh (edema) te a hring (edema).
• Vun a na.
• Jaundice na tak a awm.

Central nervous system nena inzawm symptoms te

Taksaah copper a lo punkhawm chuan Wilson natna vei te hian central nervous system tichhe thei symptom an nei thei a, chu chuan rilru hriselna a nghawng thei a ni. Hengte hi puitlingah tam zawk mah se, naupangah pawh a awm thei.

Nervous system nena inzawm symptoms te chu:

• Thusawi, ei leh in, taksa inrem lohna lama harsatna.
• Muscle a nghet lo.
• Tremors emaw, control loh taka chetna (restlessness).

Wilson natna lan chhuah dan, rilru hriselna tichhe thei:

• Hlauhthawnna.
• Mod, mize, a nih loh leh nungchang inthlak danglamna.
• Lungngaihna.
• Ngaihtuahna leh rilru put hmang tihbuai a, thudik leh thu dik lo thliar hran harsa tak dinhmun (psychosis).

Mit nena inzawm natna lan chhuah dan

Wilson natna vei tam tak chuan mitmeng (mit dum) kil vel ah hian green, gold, brown rings (Kayser-Fleischer rings) an nei thin. Heng ``Kayser-Fleischer rings'' te hi mit chhunga copper awmkhawm vang a ni. I doctor chuan heng ring te hi mit exam bik (slit-lamp exam) neih laiin a hmu thei a ni.

Mi tam tak chuan nervous system tichhe thei symptom nei leh Wilson natna vei nia hriatte hian heng ``Kayser-Fleischer rings'' te hi an nei a ni.

Wilson natna lan chhuah dan dangte

Wilson natna hian i taksa peng dang a nghawng thei bawk a, a natna lan chhuahna te chu:

• Hemolytic anemia hi thisen cell a chhiat vang a ni.
• Ruh leh ruh natna (arthritis emaw osteoporosis emaw).
• Lung ruh natna `(cardiomyopathy)`.
• Kidney lama harsatna (renal tubular acidosis emaw kidney stone emaw) a awm thei.

Wilson natna hi eng nge thlen?

Wilson natna thlentu ber chu ATP7B an tih gene a mutation a ni. He gene hian kan taksa atanga copper tam lutuk paih chhuaktu a ni.

A tlangpuiin liver hian extra copper chu fluid (bile) ah a chhuah tir thin. He bile hi gallbladder-ah a awm a. Chaw ei tur digest turin a pui thin. Bile hian chaw ei kawng kaltlangin taksa atanga copper bakah toxin leh bawlhhlawh dangte a paih chhuak thin. Wilson natna i neih chuan i thin hian bile chhungah copper a chhuah tlem zawk. Hei hian extra copper chu i taksaah a awm reng a ni.

Wilson natna hi ka rochun thei ang em?

Ni e, Wilson natna thlentu ATP7B gene-a mutation chu i rochun thei a ni. Chumi awmzia chu mutation chu nu leh pa pakhat atanga nu leh pa dang hnenah a kal zel tihna a ni. Wilson natna vei tur chuan mi pakhatin gene chhia pahnih a rochun a ngai a – pakhat chu a nu hnen atangin leh pakhat chu a pa hnen atangin (autosomal recessive) .

Mite chuan ATP7B gene pakhat mutation nei lo leh pakhat zawk mutation nei te chuan Wilson natna hi an vei lo. Mahse natna phurtu an ni. Chumi awmzia chu, an kawppui genetic status a zirin, gene hrisel tak, carrier state emaw, natna chu an fate hnenah an pe chhuak thei tihna a ni.

Wilson natna hi engtin nge an hriat theih? (Diagnosis) tih a ni.

Wilson natna hriat chian nan i doctor chuan i chhungkaw damdawi lam chanchin leh i mimal damdawi lam chanchin a zawt ang a, i natna lan chhuah dan hi he natna avanga lo awm thei a nih leh nih loh hriat tumin a bei ang.

Doctor-te chuan i thin, thluak, emaw mit lama harsatna i neihzia tilangtu chhinchhiahna dang a awm leh awm loh an enfiah ang che. Mit exam lai hian doctor chuan ``slit-lamp exam.`` a ti ang.He test hi light bik hmanga ``Kayser-Fleischer rings.`` zawn nan a ni

Wilson natna rinhlelh a nih chuan i doctor chuan thisen leh zun test turin thu a pe ang.

Wilson natna hi eng test nge hman thin?

Wilson natna hi thisen test emaw, zun test emaw, genetic test emaw, liver biopsy emaw hmanga hriat theih a ni.

Thisen test a ni

Thisen test hian i thisenah thil tam tak a check thei a:

• Ceruloplasmin: Ceruloplasmin hi thisenah copper phurtu protein a ni. Wilson natna vei te hian ceruloplasmin an nei tlem hle.
• Copper: Wilson natna vei te hian an thisenah copper a tam zawk emaw, a hniam zawk emaw a awm thei.
• Alanine transaminase (ALT) leh aspartate transaminase (AST): ALT leh AST hi thin enzyme a ni a, liver a chhiat chuan a pung a ni.
• Thisen sen (Red blood cells): Wilson natna vei te hian thisen sen (anemia) an nei tlem thei hle.

Medical test dangin Wilson natna a finfiah theih loh emaw, a hnial theih loh emaw chuan i doctor chuan Wilson natna thlentu genetic mutation a awm leh awm loh enfiah turin thisen test a ti thei ang.

Darkar 24 chhung zun lakkhawm test a ni

Darkar 24 chhungin i zun chu in lamah i la khawm ang a, i doctor-in a pek copper tel lo bawm bik ah i la khawm ang. Laboratory-ah i zun a copper awm zat a test ang. Wilson natna vei te hian an zun-ah hian copper level pangngai aia tam an nei a.

Liver biopsy a ni

Thisen leh zun test-in Wilson natna hi a finfiah theih loh emaw, a finfiah theih loh emaw chuan i doctor chuan liver biopsy a ti thei ang. Hetiang tih danah hian i doctor chuan i liver atanga tissue sample tlemte a la thin. Pathologist chuan microscope hmangin tissue chu a enfiah a, Wilson’s disease, liver chhiatna leh cirrhosis te ang chi liver natna bik chhinchhiahna a zawng thin. Liver tissue pakhat chu lab-ah thawn a, copper a awm leh awm loh enfiah a ni.

Imaging test te pawh a awm

I nervous system nena inzawm symptom i neih chuan i doctor chuan imaging test hmangin Wilson natna emaw thluaka natna dang emaw chhinchhiahna a zawng thei ang. Chûng zîngah chuan:

• MRI (Magnetic Resonance Imaging) hmanga tihchhuah a ni.
• `X-ray` hmanga tih a ni
• `CT scan` (Computer hmanga Tomography Scan) hmanga tih a ni.

Wilson natna hi engtin nge enkawl a nih?

Wilson natna enkawlna atana thil pawimawh ber chu i taksaa toxic copper level tihtlem te, taksa peng hrang hrang tihchhiat loh te, leh taksa peng hrang hrangte hnathawh that loh vanga symptoms lo awm thin te ven hi a ni Enkawlna atan hian:

• Taksa atanga copper paih chhuak thei damdawi (chelating agents - e.g. D-penicillamine, Trientine, Tetrathiomolybdate) ei.
• Zinc ei hian intestine atanga copper a lakluh loh nan.
• Copper tlakchhamna diet ei.

Wilson natna vei te hian dam chhung enkawlna an mamawh a ni. Enkawlna tihtawp a nih chuan acute liver failure a awm thei. I doctor chuan thisen leh zun test a nei fo ang a, enkawlna a thawh that dan a enfiah ang.

Wilson natna damdawi te chu engte nge ni?

I doctor chuan i taksaa copper awm zat tihtlem nan damdawi chi hrang hrang a rawn rawt thei ang.

Chelating agents te pawh a awm

Chelating agents hian i taksa atanga copper paih chhuakin hna a thawk thin. Chungte entirnan:

• Penicillamine hmanga siam a ni
• Trientine a ni

Enkawlna an tan tirh chuan doctor-te chuan chelating agents dose an tipung zauh zauh thin. Taksa chhunga copper tam lutuk chu paih chhuah a nih hma chuan dose sang zawk a ngai a ni. Wilson natna lan chhuah dan a lo ṭhat tawh a, test-nain copper chu a him tawk tih a finfiah hnuah doctor chuan maintenance treatment atan chelating agents dose hniam zawk a pe thei ang. Lifelong maintenance treatment hian copper a khawlkhawm leh tawh loh nan a veng a ni.

Heng damdawi te hian side effect a nei thei a, chuvangin i doctor zawh a pawimawh a, supplement eng emaw zat, vitamin te ei a ngai em tih emaw, operation hmaa fimkhur tur a awm em tih emaw zawh a pawimawh.

Zinc a ni

Zinc hian intestine atanga copper lakluh a veng a ni. I doctor chuan chelating agents hmanga copper tam lutuk i paih hnuah maintenance treatment atan zinc a pe thei ang. Wilson natna i vei a, mahse a lan chhuah loh chuan i doctor chuan zinc a la pe thei tho.

Naupai lai hian Wilson natna hi engtin nge enkawl a nih?

Naupai i nih chuan i naupai chhung zawnga Wilson natna hi engtin nge i enkawl ang tih doctor i zawt tur a ni. Nausen hian copper tlemte a mamawh avangin i doctor chuan chelating agents dose tlemte a pe thei a ni. Wilson natna hre chiang tak obstetrician neih hi a \angkai thei hle.

Tin, Wilson natna enkawl lai hian hnute pek a him em tih doctor zawt bawk ang che.

Wilson natna i vei chuan eng ei tur nge i ei loh tur?

Wilson natna i vei chuan i doctor chuan ei tur thenkhat, copper tamna ei loh turin a fuih thei che a ni. A bik takin, i pumpelh tur a ni:

• Shellfish, clams leh oyster te ang chi te
• Thin

Copper tamna ei tur dang:

• Chocolate
• Thlai hring
• Bean leh peas hring
• Mushroom a ni
• Nuts (cashew, peanuts ang chi) 1.1.

Treatment hmanga i copper level tihhniam a nih hnuah leh maintenance treatment a tan hnuah, heng ei tur thenkhat hi a tlem berah i ei theih leh theih loh chungchang hi i doctor nen sawiho rawh.

I tap tui hi tuikhuah atanga lo chhuak emaw, copper pipe atanga lo chhuak emaw a nih chuan i tui chhunga copper level enfiah rawh. I tap tui atanga copper lakchhuah nan water filter i hmang thei bawk.

Vitamin ang chi supplement ei i ngaihtuah a nih chuan i ei hmain doctor nen inbiakna neih hmasa phawt ang che. Supplement thenkhatah chuan copper a awm thei bawk.

Enkawlnaah hian harsatna a awm em?

Wilson natna hian harsatna a thlen thei a, mahse a hmaa hriatchhuah leh enkawl a nih chuan side effect a awm theihna a ti tlem thei a ni. Wilson natna enkawlna atana hman damdawi te hian side effect a nei thei bawk a, chuvangin i doctor hnenah eng nge fimkhur tur tih zawt rawh.

Acute liver a chak loh vang a ni

Wilson natna hian acute liver failure a thlen thei a ni. Hei hi vaukhânna tel lova liver-in a hnathawh a hloh thut lai a ni. Wilson natna vei zinga 5% vel chuan an hmuhchhuah lai hian acute liver failure an nei a ni. Hetiang dinhmun hi liver transplant a ngai mai thei.

Wilson natna avanga acute liver failure nei te pawhin acute kidney failure leh anemia chi khat hemolytic anemia an tih te pawh an nei fo thin.

Cirrhosis natna (Cirrhosis) a awm

Cirrhosis hi liver tissue hrisel tak takte chu scar tissue-in a thlak a, chu chuan liver chu a thawk tha thei lo. Scar tissue hian liver kal tlanga thisen kal chu eng emaw chen a tikhawlo bawk. Cirrhosis a lo zual zel chuan liver chu a thawk tha lo tan thin.

Wilson natna vei hmuhchhuah zinga 35% leh 45% inkar chuan an hmuhchhuah lai hian cirrhosis an nei a ni.

Cirrhosis hian liver cancer vei theihna a tipung a ni. Mahse, doctor-te chuan Wilson natna avanga cirrhosis vei te chu thil dang avanga cirrhosis nei te aiin liver cancer an vei tlem zawk tih an hmuchhuak.

Liver a tha lo

Cirrhosis hian a tawpah chuan liver failure a thlen thei a ni. Liver failure chu i liver a chhiat em avangin a thawk tawh lo. Hei hi end-stage liver disease an ti bawk. Hetiang dinhmun hi liver transplant a ngai mai thei.

Wilson natna avanga harsatna awmte chu engtin nge enkawl an nih?

Wilson natna hian cirrhosis a thlen chuan i doctor chuan i harsatna chu damdawi emaw, operation emaw hmangin a enkawl thei mai thei.

Wilson natna hian cirrhosis avanga acute liver failure emaw chronic liver failure emaw a thlen chuan liver transplant a ngai mai thei. Mi thenkhat liver transplant nei te chuan Wilson natna atang hian an dam kim vek thin. Mahse i doctor chuan transplant a hlawhtlin leh hlawhtlin loh nan uluk takin a enkawl ang che.

Engtiang chiahin nge enkawl hnuah ka dam leh ang?

Wilson natna enkawl hi dam chhung zawnga tih tur a ni . I rilru a hahdam theih nan hun a duh rei zawng chu i natna lan chhuah dan nasat danah a innghat a ni. Mahse, thla li atanga thla ruk chhung enkawl hnuah, regular maintenance treatment i neih hnuah chuan i natna lan chhuah dan chu nasa takin a tlahniam thei a ni. I doctor chuan i Wilson natna enkawlna chu engtiang chiahin nge a thawh tih enfiah turin test a nei fo ang. I taksa mamawh ang zela i damdawi ei tur chu a siamrem bawk ang.

Wilson natna ka neih chuan eng nge ka beisei ang?

I natna hmuhchhuah dan azirin Wilson natna nena inzawm symptoms i nei thei a, i nei lo thei bawk. Symptoms i neih tak tak chuan enkawlna i hmuh loh chuan nunna atana hlauhawm tak a ni thei. A pawimawh ber chu i taksa atanga toxic copper paih chhuak tur leh taksa peng hrang hrang tihchhiat loh nan i doctor-in a enkawl dan tur dik taka zawm hi a ni.

Wilson natna hi dam chhung zawnga natna a ni a, a damdawi a awm lo. Enkawl loh Wilson natna hian dam rei a ti rei lo. Wilson natna vei nia hriat a, hlawhtling taka liver transplant nei te chuan dam rei zawng an nei tha hle. Amaherawhchu transplant an neih hnuah pawh an dam chhung zawng doctor enkawlna hnuaiah an awm a ngai dawn a ni.

Wilson natna hi ven theih a ni em?

Wilson natna hi inherited genetic mutation avanga lo awm a nih avangin ven theih a ni lo. Wilson natna vei chhungkaw history i neih chuan, Wilson natna vei theihna emaw, he genetic condition nei fa neih theihna tur emaw hriatthiam nan i doctor nen genetic testing chungchang sawipui rawh.

Engtin nge ka in enkawl ang?

Wilson natna i vei chuan i doctor-in a tih angin i damdawi ei a, i ei leh in atanga copper tamna ei tur paih chhuakin i in enkawl thei ang. Enkawlna hmang hian i natna lan chhuah dan i tihziaawm thei a, nunna atana hlauhawm harsatna pawh i veng thei bawk.

Engtikah nge ka doctor ka hmuh ang?

I Wilson natna lan chhuah dan a na zual a nih chuan, a bik takin a hmaa a lo ṭhat tawh chuan doctor i pan tur a ni. Hei hian i damdawi ei tur zat thlak a ngai tihna a ni thei.

I Wilson natna lan chhuah danin nitin hnathawh pangngai i tih theih loh chuan, a bik takin ei theih loh i nih chuan, i luak chhuak chhunzawm zel a nih chuan, rilru lam natna (psychosis) i nei a, i vun a sen (jaundice), emaw, i pum na tak i nei a nih chuan, emergency room-ah kal nghal la emaw, 911 call rawh.

Doctor hnenah eng zawhna nge ka zawh ang?

• Liver transplant ka mamawh dawn em?
• Wilson natna enkawlna atana damdawi an pekte hian eng side effect nge a neih?
• Nau ka pai a nih chuan ka naute hian Wilson natna a vei dawn em ni?
• Nau ka pai chuan ka damdawi hi ka ei thei ang em?
• I damdawi min pek kha eng chen nge ka ei a ngaih?

A tawp berah chuan (Take-Home Message) hi hre reng ang che.

Wilson natna hi a hmaa hriatchhuah leh enkawl hi he dam chhung zawnga genetic condition enkawl dan tha ber a ni. I ei leh in a copper tihtlem nan nunphung i thlak a ngai mai thei. I damdawiin i taksaa copper awm zat tihtlem nan hna a thawk em tih hriat chian nan i doctor hmu ngei ang che. Enkawlna dik tak chuan i dam leh thei a, nunna atana hlauhawm harsatna i neih loh nan a pui thei bawk. Hlauthawng suh, a pawimawh ber chu fimkhur a, doctor thurawn zawm hi a ni.

` Wilson natna, copper, thin, thluak, genetic natna, cirrhosis, Keiser-Fleischer rings te