Ingaba ulusu lwakho kunye namehlo akho amhlophe ajika abe tyheli? Masifunde ngakumbi ngeDubin-Johnson Syndrome!

Ngaba wakha waphawula ukuba umbala wolusu lwakho okanye amhlophe emehlweni akho abonakala ngathi atyheli kancinci? Okanye wakha waphawula ukuba umchamo wakho utyheli mnyama kunesiqhelo? Ukuba uhlangabezana nezi zinto, enye imbangela enokwenzeka yimeko engaqhelekanga kodwa engeyongozi kakhulu ebizwa ngokuba yiDubin-Johnson syndrome . Ngoko ke, masithethe ngale nto ngokweenkcukacha, ngokulula.

Yintoni iDubin-Johnson Syndrome?

Ngamafutshane, i-Dubin-Johnson syndrome yimeko yemfuza echaphazela isibindi sethu. Okwenzekayo koku kukuba into etyheli ebizwa ngokuba yi-bilirubin, eveliswa emzimbeni wethu, iqokelelana endaweni yokuba ikhutshwe emzimbeni ngokufanelekileyo. Ngoku usenokuba uzibuza ukuba yintoni i-bilirubin. Emva kwexesha elithile, xa ixesha labo lobomi liphela, iiseli ezibomvu zegazi emzimbeni wethu ziyaqhekeka. Kulapho ke le bilirubin iveliswa khona. Ngokwesiqhelo, le bilirubin kufuneka ikhutshwe kwisibindi sethu kunye ne-bile kwaye ikhutshwe emzimbeni njengenkunkuma. Nangona kunjalo, le nkqubo ayenzeki ngokufanelekileyo emzimbeni womntu one-Dubin-Johnson syndrome. Ke ngoko, i-bilirubin iqokelelana kwisibindi nasegazini, ibangela iimpawu ezifana ne -jaundice . Kulapho ulusu kunye namhlophe emehlweni ajika abe tyheli.

Ngubani ochaphazeleka kakhulu yile meko?

Isifo iDubin-Johnson sisifo semfuza , ngoko ke sinokuchaphazela nabani na, nangaliphi na ixesha. Ukuze uzuze esi sifo, bobabini abazali bakho kufuneka badlulisele i-gene kuwe. Oku kubizwa ngokuba yi- autosomal recessive inheritance. Umzekelo, ukuba uzuze i-gene kumzali wakho omnye kuphela, awuyi kubonisa zimpawu, kodwa uya kuba ngumthwali we-gene. Oku kuthetha ukuba ungadlulisela i-gene kubantwana bakho.

Ixhaphake kangakanani le meko?

Akukho zibalo zichanekileyo zokuba oku kuqheleke kangakanani, kodwa kuqheleke kakhulu kubantu abahlala kumazwe afana ne-Iran, i-Iraq, iMorocco, ne-Japan. Kuqikelelwa ukuba malunga nomntu omnye kwi-1,300 kuloo mazwe unokuba nale meko.

I-Dubin-Johnson syndrome iyichaphazela njani umzimba wam?

Isifo sikaDubin-Johnson sibangelwa kukuqokelelwa kwe-bilirubin etyheli esibindini sakho. Yile nto ibangela ukuba ulusu lwakho kunye namehlo amhlophe abonakale emthubi. Ngamanye amaxesha, xa ungaphakathi kwaye ungenakukhanya kuncinci, usenokungawuboni lo mthubi. Nangona kunjalo, kwindawo ekhanyiswe kakuhle, njengaselangeni, lo mthubi ubonakala ngakumbi. Rhoqo, into yokuqala oyiphawulayo ngalo mthubi bubuso.Emva koko ingasasazeka nakwezinye iindawo zomzimba, njengesifuba nesisu. Xa uzijonga esipilini, ukuba uvula umlomo wakho uze ujonge phantsi kolwimi lwakho, ungabona ukuba ujike waba mthubi nalapho.

Zithini iimpawu zoku?

Uninzi lwabantu abafunyaniswe benesi sifo iDubin-Johnson syndrome baneempawu eziphambili ze-jaundice, ezibangelwa kukuqokelelana kwe-bilirubin esibindini. Oku kuthetha oku:

• Ukutyheli kolusu lwakho kunye namehlo akho amhlophe.
• Utshintsho kumbala womchamo wakho (ujika ube mthubi omnyama okanye umdaka).

Ngamanye amaxesha, xa umzimba uphantsi koxinzelelo oluthile, umzekelo, xa unesinye isifo, ngexesha lokukhulelwa, okanye ukuba usebenzisa iipilisi zokulawula ukukhulelwa (izithinteli zokukhulelwa ezithathwa ngomlomo), lo mbala umthubi unokwanda.

Ukongeza kwezi, ezinye iimpawu ezincinci zinokubakho, kodwa azisoloko zimbi kangako:

• Ukuziva udiniwe `(Ukudinwa)`.
• Intlungu encinci esiswini esisezantsi (Intlungu Yesisu).

Into ebalulekileyo kukuba i-Dubin-Johnson syndrome yimeko engeyongozi. Oku kuthetha ukuba ayizukubangela monakalo mkhulu ebomini bakho.

Ukuba wenza uvavanyo oluthile, ungabona izinto ezinje:

• Isibindi sakho sinezinto ezithile eziqokeleleneyo, ezenza sibonakale simnyama (oku kuhlolwa ngokuthatha iqhekeza elincinci lesicwili esibindini - sibizwa ngokuba yi -'Liver Biopsy ').
• Isibindi sinokwandiswa kancinci ( iHepatomegaly ).

Yintoni ebangela iDubin-Johnson syndrome?

Eyona nto ibangela iDubin-Johnson syndrome kukuguquka kwejini ye-'ABCC2'. Le jini ye-'ABCC2' inoxanduva lokwenza iproteni enceda iiseli zethu zisuse inkunkuma. Ngokukodwa, le protein inceda iiseli zesibindi zisuse i-bilirubin (into emthubi eseleyo ekuqhekekeni kweeseli ezibomvu zegazi) kwaye iyikhuphe njenge-bile (ulwelo olunceda ekugayeni ukutya).

Ngoko ke, ukuba une-Dubin-Johnson syndrome, umzimba wakho awukwazi ukuyisusa ngokufanelekileyo le nkunkuma ebizwa ngokuba yi-bilirubin kwiiseli zakho. Ngenxa yoko, izinto ezifana ne-bilirubin ziyaqokelelana emzimbeni wakho (oku kubizwa ngokuba yi -hyperbilirubinemia ). Yiyo loo nto kuvela iimpawu ezifana ne-jaundice.

Ifunyanwa njani iDubin-Johnson syndrome?

Ugqirha wakho uza kuqala akuhlole, akubuze ngeempawu zakho, aze afumanise ukuba kukho nabani na kusapho lwakho okhe waba nezi meko zifanayo ngaphambili (imbali yezonyango yosapho).

Emva koko, baya kuyalela ezinye iimvavanyo ezininzi ukuqinisekisa ukuxilongwa. Ezi zingabandakanya:

• Uvavanyo lwemifanekiso (umzekelo , i-X-ray , i- ultrasound scan ) ukujonga isibindi sakho.
• Uvavanyo lwegazi (ingakumbi uvavanyo lwe-bilirubin )Nye).
• Uvavanyo lomchamo .
• Mhlawumbi uvavanyo apho kuthathwa khona iqhekeza elincinci lesibindi ( `Liver Biopsy` ).

Ukuba uvavanyo lwe-bilirubin lubonisa ukuba inqanaba le-bilirubin egazini lakho liphezulu kakhulu, oko kuthetha ukuba unesifo esibizwa ngokuba yi -hyperbilirubinemia (i-bilirubin ephezulu egazini). Ukuba iimpawu zakho ziyaqhubeka, ugqirha wakho unokuyalela uvavanyo lwegazi lwemfuza ukuze kufunyanwe i-gene ebangela iimpawu zakho. Le yindlela yokuqinisekisa ukuba unesifo se-Dubin-Johnson.

Zithini iindlela zonyango zoku?

Iindaba ezimnandi zezokuba uninzi lwabantu abafunyaniswe benesi sifo seDubin-Johnson abadingi naluphi na unyango. Oku kungenxa yokuba asiyongxaki inzima. Nangona kunjalo, ukuba kufuneka unyango, ludla ngokuba lolunye uhlobo okanye inkqubo ebangela ukuba amanqanaba e-bilirubin anyuke. Umzekelo, ukuba unomkhuhlane, ugqirha wakho angakuxelela ukuba usele ulwelo oluninzi kwaye uphumle. Okanye, ukuba amayeza owasebenzisayo enza iimpawu zakho zibe mandundu, ugqirha wakho angakucebisa ukuba uyeke ukuwasebenzisa loo mayeza.

Yintoni enokwenza iimpawu zesifo sikaDubin-Johnson zibe mbi ngakumbi?

Iimpawu ze-jaundice ezibangelwa yi-Dubin-Johnson syndrome zinokuhlala ubomi bonke. Nangona kunjalo, ezi mpawu zinokuba mandundu ukuba:

• Ukuba usela utywala.
• Ukuba uthatha iipilisi zokulawula ukukhulelwa.
• Ukuba kukho usulelo.
• Ukuba ukhulelwe.

Ukuba uziva ngathi iimpawu zakho ziya zisiba mandundu, thetha nogqirha wakho malunga neendlela zonyango ezinokukunceda ufumane isiqabu.

Ngaba i-Dubin-Johnson syndrome ingathintelwa?

Ngenxa yokuba sisifo semfuza, asinakuyithintela iDubin-Johnson syndrome ukuba ivele. Nangona kunjalo, ukuba uceba ukuba nomntwana kwaye ufuna ukwazi umngcipheko wokuba umntwana wakho abe nesi sifo semfuza, ungathetha nogqirha wakho malunga novavanyo lwemfuza okanye iingcebiso ngemfuza .

Ukuba ndinesifo iDubin-Johnson syndrome, ndimele ndilindele ntoni?

Imbono yabantu abafunyaniswe benesi sifo ilungile kakhulu. Akukho nyango, kwaye unyango alufuneki, kwaye iimpawu ziyalawuleka. Okubaluleke kakhulu, le meko ayichaphazeli ubomi bomntu.

Ndifanele ndibonane nogqirha wam nini?

Ukuba iimpawu zakho zesifo seDubin-Johnson ziya zisiba mandundu, ingakumbi ukuba isifo sakho seswekile sibangela iimpawu ezifana nomkhuhlane, bona ugqirha wakho. Umzekelo:

• Intlungu zesisu .
• Ifiva .
• Ukudinwa .
• Isicaphucaphu okanye ukuhlanza .

Ndingayibuza yiphi imibuzo ugqirha wam?

Ungabuza ugqirha wakho imibuzo efana nale:

• Ngaba iimpawu zam zifuna unyango?
• Ngaba umntwana wam uza kulizuza eli lungelo?
• Ndingenza ntoni ukuba andiziva ndiphilile ngenxa yeempawu zam?

Yintoni umahluko phakathi kweDubin-Johnson syndrome kunye neRotor syndrome?

I-Dubin-Johnson syndrome kunye ne-Rotor syndrome zombini ziimeko zemfuza. Zombini zineempawu ezifanayo, ezifana ne-jaundice. Nangona kunjalo, umahluko omkhulu phakathi kwezi meko zimbini ngunobangela wazo.

I-Dubin-Johnson syndrome ibangelwa kukuguquka kwejini ye-`ABCC2`. Nangona kunjalo, i-Rotor syndrome ibangelwa kukuguquka kwejini ye-`SLCO1B1` kunye ne-`SLCO1B3` .

Enye into ebalulekileyo ekufuneka uyiqaphele kukuba iRotor Syndrome inokuchaphazela kakhulu indlela umzimba womntu ophatha ngayo amayeza athile. Ngoko ke, ukuba wena okanye umntwana wakho uneRotor Syndrome, kubaluleke kakhulu ukwazisa ugqirha wakho ngaphambi kokuba umnike amayeza athile.

Ukongeza, iRotor Syndrome ayibangeli kuguquka kwesibindi. Nangona kunjalo, isibindi sabantu abaneDubin-Johnson Syndrome sibonakala simnyama .

Kuqhelekile ukuba nexhala xa ulusu lwakho okanye lomntwana wakho lujika lube tyheli. Nangona i-jaundice ixhaphakile kwiintsana ezisandul’ ukuzalwa, i-jaundice ebangelwa yi-Dubin-Johnson syndrome yimeko eqhubekayo, ehlala ixesha elide.

Okokugqibela, izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Ngoko ke, ngoku uyazi okuninzi nge-Dubin-Johnson syndrome. Ungakhathazeki, ayisiyongxaki imbi kakhulu. Ugqirha wakho uza kukuchazela ukuba loluphi unyango olufunekayo ukunciphisa ukutyheli kwamehlo nolusu lwakho, okanye ukuba uvela nezinye iimpawu. Eyona nto ibalulekileyo kukuthetha nogqirha wakho malunga neempawu zakho rhoqo kwaye uqinisekise ukuba usempilweni. Ungaphila ubomi obuqhelekileyo, obonwabisayo ngale meko.

Isifo sikaDubin -Johnson, umkhuhlane otyheli, isibindi, i-bilirubin, izifo zemfuza, ukutyheli kolusu, i-jaundice