Ingabe isikhumba sakho kanye namehlo akho amhlophe aphenduka aphuzi? Ake sifunde kabanzi nge-Dubin-Johnson Syndrome!

Wake waphawula ukuthi umbala wesikhumba sakho noma amhlophe emehlweni akho abonakala ephuzi kancane? Noma wake waphawula ukuthi umchamo wakho uphuzi omnyama kunokuvamile? Uma uhlangabezana nalezi zinto, imbangela eyodwa engaba khona yisimo esingavamile kodwa esingesibi kakhulu esibizwa ngokuthi i-Dubin-Johnson syndrome . Ngakho-ke, ake sixoxe ngalokhu ngokuningiliziwe, kalula.

Iyini i-Dubin-Johnson Syndrome?

Kalula nje, i-Dubin-Johnson syndrome yisimo sofuzo esithinta isibindi sethu. Okwenzekayo kulokhu ukuthi into ephuzi ebizwa ngokuthi i-bilirubin, ekhiqizwa emzimbeni wethu, iyaqongelela esikhundleni sokukhishwa emzimbeni ngendlela efanele. Manje ungase uzibuze ukuthi iyini i-bilirubin. Ngemva kwesikhathi esithile, lapho isikhathi sokuphila kwawo siphela, amangqamuzana abomvu egazi emzimbeni wethu ayawohloka. Yilapho le bilirubin ikhiqizwa khona. Ngokuvamile, le bilirubin kufanele ikhishwe esibindini sethu kanye ne-bile bese ikhishwa emzimbeni njengemfucuza. Kodwa-ke, le nqubo ayenzeki kahle emzimbeni womuntu one-Dubin-Johnson syndrome. Ngakho-ke, i-bilirubin iqongelela esibindini nasegazini, okubangela izimpawu ezifana ne -jaundice . Yilapho isikhumba kanye namehlo amhlophe ephenduka aphuzi.

Ubani othinteka kakhulu yilesi simo?

I-Dubin-Johnson syndrome iyisimo sofuzo , ngakho-ke ingathinta noma ubani, nganoma yisiphi isikhathi sobudala. Ukuze uzuze lesi simo, bobabili abazali bakho kumele badlulisele i-gene kuwe. Lokhu kubizwa ngokuthi i- autosomal recessive inheritance. Isibonelo, uma uzuza i-gene kumzali wakho oyedwa kuphela, ngeke ubonise izimpawu, kodwa uzoba ngumthwali we-gene. Lokhu kusho ukuthi ungadlulisela i-gene ezinganeni zakho.

Sivame kangakanani lesi simo?

Azikho izibalo eziqondile zokuthi lokhu kuvame kangakanani, kodwa kuvame kakhulu kubantu abahlala emazweni afana ne-Iran, i-Iraq, iMorocco, ne-Japan. Kulinganiselwa ukuthi cishe umuntu oyedwa kwabayi-1,300 kulawo mazwe angaba nalesi simo.

I-Dubin-Johnson syndrome iwuthinta kanjani umzimba wami?

I-Dubin-Johnson syndrome ibangelwa ukunqwabelana kwe-bilirubin ephuzi esibindini sakho. Yilokhu okubangela ukuthi isikhumba sakho kanye namehlo amhlophe abukeke ephuzi. Ngezinye izikhathi, uma ungaphakathi endlini futhi ekukhanyeni okuncane, ungase ungakuboni lokhu kuphuzi. Kodwa-ke, endaweni ekhanyiswe kahle, njengaselangeni, lokhu kuphuzi kubonakala kakhulu. Ngokuvamile, into yokuqala oyiphawulayo ngalokhu kuphuzi ubuso.. Ngemuva kwalokho ingasakazekela kwezinye izingxenye zomzimba, njengesifuba nesisu. Uma uzibuka esibukweni, uma uvula umlomo wakho ubheke ngaphansi kolimi lwakho, ungabona ukuthi usuphenduke waba phuzi nalapho.

Ziyini izimpawu zalokhu?

Iningi labantu abatholakale benesifo i-Dubin-Johnson syndrome banezimpawu eziyinhloko ze-jaundice, ebangelwa ukunqwabelana kwe-bilirubin esibindini. Lokhu kusho ukuthi:

• Ukuphuzi kwesikhumba sakho kanye nokumhlophe kwamehlo akho.
• Ukushintsha kombala womchamo wakho (uphendukela kophuzi omnyama noma onsundu).

Ngezinye izikhathi, lapho umzimba ungaphansi kokucindezeleka okuthile, isibonelo, uma unesinye isifo, ngesikhathi sokukhulelwa, noma uma usebenzisa amaphilisi okuvimbela inzalo (izithibela-nzalo eziphuzwa ngomlomo), lo mbala ophuzi unganda.

Ngaphezu kwalokhu, kungase kube nezinye izimpawu ezincane, kodwa ngokuvamile azibi zimbi kakhulu:

• Ukuzizwa ukhathele `(Ukukhathala)`.
• Ubuhlungu obuncane esiswini esingezansi (Ubuhlungu Besisu).

Into ebalulekile ukuthi i-Dubin-Johnson syndrome iyisimo esingenabungozi. Lokhu kusho ukuthi ngeke ibangele umonakalo omkhulu empilweni yakho.

Uma wenza izivivinyo ezithile, ungase ubone izinto ezinjengalezi:

• Isibindi sakho sinezinto ezithile eziqongelelekile, okwenza sibonakale simnyama (lokhu kuhlolwa ngokuthatha ingxenye encane yezicubu esibindini - kubizwa ngokuthi i -'Liver Biopsy ').
• Isibindi singakhula kancane ( i-Hepatomegaly ).

Yini ebangela i-Dubin-Johnson syndrome?

Imbangela eyinhloko ye-Dubin-Johnson syndrome ukuguquka kwezakhi zofuzo ze-'ABCC2'. Le zakhi zofuzo ze-'ABCC2' inesibopho sokwenza iphrotheni esiza amangqamuzana ethu ukususa imfucuza. Ngokukhethekile, le phrotheni isiza amangqamuzana esibindi ukususa i-bilirubin (into ephuzi esele ekuqhekekeni kwamangqamuzana abomvu egazi) bese iyikhipha njenge-bile (uketshezi olusiza ekugayeni ukudla).

Ngakho-ke, uma une-Dubin-Johnson syndrome, umzimba wakho awukwazi ukususa kahle lo mkhiqizo ongcolile obizwa ngokuthi i-bilirubin emaseli akho. Ngenxa yalokho, izinto ezifana ne-bilirubin ziyanqwabelana emzimbeni wakho (lokhu kubizwa ngokuthi i-hyperbilirubinemia ). Yingakho kuvela izimpawu ezifana ne-jaundice.

I-Dubin-Johnson syndrome itholakala kanjani?

Udokotela wakho uzoqala akuhlole, akubuze ngezimpawu zakho, bese ethola ukuthi kukhona yini emndenini wakho oke waba nezimo ezifanayo ngaphambili (umlando wezokwelapha womndeni).

Ngemuva kwalokho, bazo-oda ezinye izivivinyo eziningana ukuqinisekisa ukuxilongwa. Lokhu kungafaka:

• Ukuhlolwa kwezithombe (isibonelo , i-X-ray , i- ultrasound scan ) ukuze kubhekwe isibindi sakho.
• Ukuhlolwa kwegazi (ikakhulukazi ukuhlolwa kwe-bilirubin )eyodwa).
• Ukuhlolwa komchamo .
• Mhlawumbe ukuhlolwa lapho kuthathwa khona ingxenye encane yesibindi ( `I-Liver Biopsy` ).

Uma ukuhlolwa kwe-bilirubin kukhombisa ukuthi izinga le-bilirubin egazini lakho liphezulu kakhulu, kusho ukuthi unesifo esibizwa ngokuthi i-hyperbilirubinemia (i-bilirubin ephezulu egazini). Uma izimpawu zakho ziqhubeka, udokotela wakho angayala ukuhlolwa kwegazi ngofuzo ukuze athole i-gene ebangela izimpawu zakho. Lena ukuphela kwendlela yokuqinisekisa ukuthi unesifo i-Dubin-Johnson syndrome.

Yiziphi izindlela zokwelapha lokhu?

Izindaba ezinhle ukuthi abantu abaningi abatholakale benesifo i-Dubin-Johnson syndrome abadingi ukwelashwa. Lokhu kungenxa yokuthi akusona isimo esibucayi. Kodwa-ke, uma kudingeka ukwelashwa, ngokuvamile kungenxa yesinye isimo noma inqubo ebangela ukuba amazinga akho e-bilirubin akhuphuke. Isibonelo, uma unomkhuhlane, udokotela wakho angakutshela ukuthi uphuze uketshezi oluningi futhi uphumule. Noma, uma umuthi owuthathayo wenza izimpawu zakho zibe zimbi kakhulu, udokotela wakho angakweluleka ukuthi uyeke ukuthatha lowo muthi.

Yini engenza izimpawu ze-Dubin-Johnson syndrome zibe zimbi kakhulu?

Izimpawu ze-jaundice ezibangelwa yi-Dubin-Johnson syndrome zingahlala impilo yonke. Kodwa-ke, lezi zimpawu zingase zibe zimbi kakhulu uma:

• Uma uphuza utshwala.
• Uma uthatha amaphilisi okuvimbela inzalo.
• Uma kukhona ukutheleleka.
• Uma ukhulelwe.

Uma uzizwa sengathi izimpawu zakho ziya ziba zimbi kakhulu, khuluma nodokotela wakho ngezinketho zokwelapha ezingakusiza uthole impumuzo.

Ingabe i-Dubin-Johnson syndrome ingavinjelwa?

Ngenxa yokuthi kuyisimo sofuzo, asikwazi ukuvimbela i-Dubin-Johnson syndrome ukuthi ingakhuli. Kodwa-ke, uma uhlela ukuba nomntwana futhi ufuna ukwazi ingozi yokuba ingane yakho ibe nalesi simo sofuzo, ungakhuluma nodokotela wakho mayelana nokuhlolwa kofuzo noma ukwelulekwa ngofuzo .

Uma ngine-Dubin-Johnson syndrome, yini okufanele ngiyilindele?

Umbono wabantu abatholakale benesifo i-Dubin-Johnson syndrome muhle kakhulu. Akukho ukwelashwa, futhi ukwelashwa ngokuvamile akudingeki, futhi izimpawu ziyaphathwa. Okubaluleke kakhulu, lesi simo asithinti isikhathi sokuphila komuntu.

Ngingambona nini udokotela wami?

Uma izimpawu zakho ze-Dubin-Johnson syndrome ziba zimbi kakhulu, ikakhulukazi uma i-jaundice yakho ibangela izimpawu ezifana nomkhuhlane, bona udokotela wakho. Isibonelo:

• Ubuhlungu besisu .
• Imfiva .
• Ukukhathala .
• Ukucanuzela noma ukuhlanza .

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Ungabuza udokotela wakho imibuzo efana nale:

• Ingabe izimpawu zami zidinga ukwelashwa?
• Ingabe ingane yami izozuza lesi simo njengefa?
• Yini okufanele ngiyenze uma ngizizwa ngingaphilile kahle ngenxa yezimpawu zami?

Uyini umehluko phakathi kwe-Dubin-Johnson syndrome ne-Rotor syndrome?

I-Dubin-Johnson syndrome kanye ne-Rotor syndrome zombili ziyizimo zofuzo. Zombili zinezimpawu ezifanayo, njenge-jaundice. Kodwa-ke, umehluko omkhulu phakathi kwalezi zimo ezimbili yimbangela yazo.

I-Dubin-Johnson syndrome ibangelwa ukuguquka kwezakhi zofuzo ze-`ABCC2`. Kodwa-ke, i-Rotor syndrome ibangelwa ukuguquka kwezakhi zofuzo ze-`SLCO1B1` kanye ne-`SLCO1B3` .

Enye into ebalulekile okufanele uyiqaphele ukuthi i-Rotor Syndrome ingathinta kakhulu indlela umzimba womuntu ophatha ngayo imithi ethile. Ngakho-ke, uma wena noma ingane yakho nine-Rotor Syndrome, kubaluleke kakhulu ukwazisa udokotela wakho ngaphambi kokumnikeza imithi ethile.

Ngaphezu kwalokho, i-Rotor Syndrome ayibangeli ukushintsha kombala wesibindi. Kodwa-ke, isibindi sabantu abane-Dubin-Johnson Syndrome sibonakala simnyama .

Kuvamile ukuzizwa ukhathazekile lapho isikhumba sakho noma somntwana wakho siphenduka siphuzi. Nakuba i-jaundice ivame kakhulu ezinganeni ezisanda kuzalwa, i-jaundice ebangelwa yi-Dubin-Johnson syndrome iyisimo esiqhubekayo, esihlala isikhathi eside.

Okokugcina, izinto okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

Ngakho-ke, manje usuyazi okuningi nge-Dubin-Johnson syndrome. Ungakhathazeki, akusona isimo esibucayi. Udokotela wakho uzochaza ukuthi yiluphi ukwelashwa okudingekayo ukuze kuncishiswe ukuphuzi kwamehlo nesikhumba sakho, noma uma uba nezinye izimpawu. Into ebaluleke kakhulu ukukhuluma nodokotela wakho ngezimpawu zakho njalo futhi uqinisekise ukuthi uphilile. Ungaphila impilo evamile nejabulisayo ngalesi simo.

I- Dubin-Johnson syndrome, umkhuhlane ophuzi, isibindi, i-bilirubin, izifo zofuzo, ukuphuzi kwesikhumba, i-jaundice