I vun rawng emaw, i mit var emaw chu a sen deuh niin i hre ngai em? A nih loh leh i zun chu a hma aia a dum zawk tih i hmu tawh em? Heng thilte hi i tawng a nih chuan a chhan awm thei pakhat chu natna awm lo, mahse a na lutuk lo , Dubin-Johnson syndrome tih hi a ni. Chuvangin, he thu hi chipchiar takin, awlsam takin han sawi ila.
Dubin-Johnson Syndrome hi eng nge ni?
A awlsam zawngin, Dubin-Johnson syndrome hi kan liver tichhe thei genetic condition a ni . Hemiah hian thil thleng chu kan taksa atanga lo chhuak, bilirubin an tih, yellow substance pakhat chu taksa atanga chhuak dik lovin a pungkhawm thin a ni. Tunah chuan bilirubin hi eng nge a nih i ngaihtuah mai thei. Hun engemaw chen hnuah an dam chhung a tawp chuan kan taksaa thisen sen (red blood cells) te chu a chhe vek a. Chutah chuan he bilirubin hi a lo chhuak ta a ni. A tlangpuiin he bilirubin hi kan thin atang hian bile nen a chhuak tur a ni a, taksa atangin bawlhhlawh angin a chhuak tur a ni. Mahse, he kalphung hi Dubin-Johnson syndrome vei taksaah a thleng dik lo. Chuvangin, bilirubin hi liver leh thisenah a pungkhawm a, jaundice ang chi symptoms a thlen thin . Chutah chuan vun leh mit var te chu a sen a lo ni ta a ni.
He dinhmun hian tute nge a nghawng ber?
Dubin-Johnson syndrome hi genetic condition , chuvangin tu pawh, kum eng pawh nise a nghawng thei. He natna hi i rochun theih nan i nu leh pa pahnih hian chu gene chu i hnenah an hlan chhawng tur a ni. Hei hi autosomal recessive inheritance an ti a. Entirnan, i nu leh pa pakhat chauh hnen atanga gene i rochun chuan symptoms i lantir lovang, mahse gene carrier i ni ang. Chumi awmzia chu i fate hnenah gene chu i pass thei tihna a ni.
Hetiang dinhmun hi engtiang chiahin nge a awm?
Hei hi engtiang chiahin nge a hluar tih hi statistics dik tak a la awm lo a, mahse Iran, Iraq, Morocco, Japan ang ram a chengte zingah a tam zawk. Chu ramah chuan mi 1,300 zinga pakhat velin he natna hi an vei thei niin an chhut.
Dubin-Johnson syndrome hian engtin nge ka taksa a nghawng?
Dubin-Johnson syndrome hi i liver-a yellow substance bilirubin a awm khawm vang a ni. Hei vang hian i vun leh i mit var te chu a sen vek a ni. A châng chuan, in chhûnga i awm lai leh êng tlem takah i awm laiin, he sen hring hi i hre lo mai thei. Mahse, hmun eng tha takah, ni chhuahna hmunah chuan he sen hring hi a langsar zual thin. Vawi tam tak chu he yellowness chungchangah hian i hriat hmasak ber chu hmai hi a ni.. Tichuan taksa peng dang, ril leh pum ang chiah a darh thei a ni. Darthlalang i en chuan i hmui i hawng a, i lei hnuai i en chuan chutah pawh chuan a sen tawh tih i hmu thei ang.
Hei hi eng symptoms nge ni?
Dubin-Johnson syndrome vei hmuhchhuah tam zawk hi jaundice symptoms ber an nei a, hei hi liver-a bilirubin awmkhawm vang a ni. Chumi awmzia chu:
- I vun sen leh i mit var.
- I zun rawng a inthlak (dark yellow emaw brown emaw a inthlak).
A châng chuan taksain harsatna engemaw a neih laiin, entirnan, natna dang i neih hunah, naupai lai emaw, naupai venna damdawi (oral contraceptives) i hman a nih chuan, he rawng sen hi a pung thei a ni.
Heng bakah hian natna lan chhuahna tenau dang pawh a awm thei a, mahse a tlangpuiin a na lo hle:
- Chau tih hriat hi `(Fatigue)` a ni.
- Pum hnuai lam a na deuh (Abdominal Pain).
A pawimawh ber chu Dubin-Johnson syndrome hi natna tha lo tak a ni. Chumi awmzia chu i nunah chhiatna lian tham a thlen dawn lo tihna a ni.
Test thenkhat i tih chuan hetiang hi i hmu mai thei:
- I liver hian thil engemaw zat a buildup a, chu chuan a dum angin a lang (hei hi liver atanga tissue te tak te lakchhuah hmanga enfiah thin a ni - chu chu `Liver Biopsy` an ti a ni ).
- Liver hi a lian deuh thei ( Hepatomegaly ).
Dubin-Johnson syndrome hi eng thilin nge a thlen?
Dubin-Johnson syndrome thlentu ber chu `ABCC2` gene a mutation a ni. He `ABCC2` gene hian kan cell-te bawlhhlawh paih chhuahna tura puitu protein a siam a ni. A bik takin, he protein hian liver cells te chu bilirubin (thisen sen te tihchhiat atanga thil sen la awm) te chu paih chhuakin bile (chaw ei tur digest tu fluid) angin a paih chhuak thin.
Chuvangin, Dubin-Johnson syndrome i neih chuan i taksa hian he bawlhhlawh bilirubin an tih hi i cell atang hian a paih chhuak tha thei lo. Chuvang chuan i taksaah bilirubin ang chi thil a lo awm khawm a (hei hi hyperbilirubinemia an ti a ni ). Chuvangin jaundice ang chi symptoms a lo lang thin.
Engtin nge Dubin-Johnson syndrome hi an hriat theih?
I doctor chuan a enfiah hmasa ang che, i symptoms te a zawt ang che, i chhungkuaah hian hetiang dinhmun hi an lo nei tawh em tih a zawt ang che (family medical history).
Tichuan, an diagnosis tih finfiah nan test eng emaw zat an order leh ang. Chûng zîngah chuan:
- Imaging test (entir nan , X-ray , ultrasound scan ) hmangin i liver enfiah thin.
- Thisen test (a bik takin bilirubin test ) .pakhat).
- Urine test neih a ni .
- Test a ni mai thei, chutah chuan liver hmun te tak te ( `Liver Biopsy` ) lak a ni.
Bilirubin test-ah i thisen bilirubin level a sang lutuk tih a hriat chuan hyperbilirubinemia (thisen chhunga bilirubin sang) tih natna i nei tihna a ni. I symptoms a awm reng chuan i doctor chuan i symptoms thlentu gene zawng turin genetic blood test a ti thei ang. Hei hi Dubin-Johnson syndrome i vei tih finfiahna awmchhun a ni.
Hei hi eng enkawlna nge ni?
Thu lawmawm tak chu Dubin-Johnson syndrome vei nia hriat tam zawk chuan enkawlna engmah an mamawh lo. Hei hi a chhan chu natna khirh tak a nih loh vang a ni. Mahse, enkawl a ngai a nih chuan i bilirubin level tisangtu condition emaw process dang emaw vang a ni tlangpui. Entirnan, khawsik i neih chuan i doctor chuan tui tam tak in tur leh chawlh hahdam turin a ti thei che a ni. A nih loh leh, i damdawi ei hian i natna lan chhuahna a tizual a nih chuan, i doctor chuan chu damdawi chu ei tawh lo turin a fuih thei che a ni.
Dubin-Johnson syndrome symptoms hi eng thilin nge ti na thei?
Dubin-Johnson syndrome vanga jaundice symptoms hi dam chhungin a awm thei a ni. Mahse, heng symptoms te hi a na zual thei a, i:
- Zu i in chuan.
- Birth control pill i ei a nih chuan.
- Infection a awm chuan.
- Naupai i nih chuan.
I natna lan chhuah dan chu a na zual zel nia i hriat chuan, i doctor nen hahdamna i hmuh theihna tura enkawl dan tur chungchang sawipui rawh.
Dubin-Johnson syndrome hi ven theih a ni em?
Genetic condition a nih avangin Dubin-Johnson syndrome lo awm tur hi kan veng thei lo. Mahse, fa neih i tum a, i fain he genetic condition hi a neih theih dan tur i hriat duh chuan i doctor nen genetic testing emaw genetic counseling emaw chungchang i sawi thei ang
Dubin-Johnson syndrome ka neih chuan eng nge ka beisei ang?
Dubin-Johnson syndrome vei nia hriatte hmalam hun chu a tha hle. Damdawi a awm lo va, enkawl a ngai lo tlangpui a, a natna lan chhuah dan pawh enkawl a ni. A pawimawh ber chu, he natna hian mi dam chhung a nghawng lo.
Engtik hunah nge ka doctor ka hmuh ang?
I Dubin-Johnson syndrome symptoms a na zual a nih chuan, a bik takin i jaundice hian flu -like symptoms a thlen a nih chuan doctor pan rawh. Entir nan:
- Pum natna .
- Khawsik .
- Chau .
- Nausea emaw luak chhuak emaw .
Doctor hnenah eng zawhna nge ka zawh ang?
I doctor hnenah hetiang zawhna hi i zawt thei ang:
- Ka symptoms te hian enkawl a ngai em?
- Ka fa hian he dinhmun hi a rochun dawn em ni?
- Ka natna lanchhuahna avanga ka dam loh chuan eng nge ka tih ang?
Dubin-Johnson syndrome leh Rotor syndrome hi eng nge an danglamna?
Dubin-Johnson syndrome leh Rotor syndrome te hi genetic condition an ni vek a. An pahnih hian symptom inang an nei a, jaundice ang chi hi an nei ve ve a ni. Mahse, an dinhmun pahnih inthlauhna ber chu an chhan a ni.
Dubin-Johnson syndrome hi `ABCC2` gene a mutation vang a ni. Mahse, Rotor syndrome hi `SLCO1B1` leh `SLCO1B3` genes a mutation vang a ni .
Hriat tur pawimawh dang chu Rotor Syndrome hian mi taksain damdawi thenkhat a enkawl danah nasa takin nghawng a nei thei a ni. Chuvangin, nangmah emaw, i fa emaw hian Rotor Syndrome i nei a nih chuan damdawi thenkhat i pek hmain i doctor hnenah hriattir hmasak a pawimawh hle.
Tin, Rotor Syndrome hian liver a tidanglam lo bawk. Mahse, Dubin-Johnson Syndrome vei te liver chu a dum angin a lang .
Nangmah emaw, i naute emaw vun a sen chuan lungkhamna i neih hi thil pangngai a ni. Nausen piang tharte zingah jaundice hi a awm fo laiin, Dubin-Johnson syndrome avanga jaundice hi a awm reng a, dam chhung zawng a ni.
A tawp berah chuan thil hriat reng tur (Take-Home Message) .
Chuvangin, tunah chuan Dubin-Johnson syndrome chungchang hi i hre tawh hle. Lungngai suh, natna khirh tak a ni lo. I mit leh vun sen hring tihziaawmna tur emaw, symptom dang i neih chuan eng enkawlna nge mamawh tih doctor chuan a hrilhfiah ang che. A pawimawh ber chu i doctor nen i symptoms chungchang sawipui fo la, i hrisel tih chian a ni. Hetiang dinhmun nen hian nun pangngai, hlim takin i nung thei a ni.
` Dubin-Johnson syndrome, yellow fever, thin, bilirubin, genetic diseases, vun sen, jaundice











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