Isifo seDubin-Johnson

Isifo i-Dubin-Johnson syndrome (i-DJS) sisifo esidluliswa ziintsapho (ezizuzwe njengelifa). Kule meko, unokuba ne-jaundice emnene ebomini.

I-DJS sisifo esinqabe kunene semfuzo. Ukuze ufumane le meko, umntwana kufuneka afumane ikopi yesakhi esineziphene kubo bobabini abazali.

Isifo siphazamisa amandla omzimba okuhambisa i-bilirubin ngesibindi siye kwi-bile. Xa isibindi kunye ne-spleen ziqhekeza iiseli ezibomvu zegazi, i-bilirubin iveliswa. I-Bilirubin ihlala ihamba kwi-bile, eveliswa sisibindi. Emva koko ingena kwimibhobho ye-bile, idlule i-gallbladder, kunye nenkqubo yokwetyisa.

Xa i-bilirubin ingahanjiswanga ngokufanelekileyo kwi-bile, iyakha kwigazi. Oku kubangela ukuba ulusu kunye nabamhlophe bamehlo bajike batyheli. Oku kubizwa ngokuba yi-jaundice. Amanqanaba aphezulu e-bilirubin angonakalisa ingqondo kunye namanye amalungu.

Abantu abane-DJS bane-jaundice yobomi obungapheliyo enokuthi yenziwe mandundu ngoku:

• Utywala
• Iipilisi zokulawula inzala
• Izinto ezisingqongileyo ezichaphazela isibindi
• Usulelo
• Ukukhulelwa

I-jaundice emnene, engabonakali kude kube kwinqanaba lokufikisa okanye ebudaleni, yeyona nto iphambili kwi-DJS.

Olu vavanyo lulandelayo lunokunceda ukufumanisa esi sifo:

• Isibindi se-biopsy
• Amanqanaba e-enzyme yesibindi (uvavanyo lwegazi)
• Isirum bilirubin
• Amanqanaba e-uroproporphyrin e-Urinary, kubandakanya inqanaba le-coproporphyrin I

Akukho unyango oluthile luyafuneka.

Imbonakalo intle kakhulu. Ngokuqhelekileyo ii-DJS azinciphisi ixesha lokuphila lomntu.

Iingxaki aziqhelekanga, kodwa zinokubandakanya oku kulandelayo:

• Intlungu zesisu
• I-jaundice enzima

Shayela umboneleli wakho wezempilo ukuba kukho oku kulandelayo:

• I-jaundice inzima
• I-jaundice iya isiba mandundu ngokuhamba kwexesha
• Unentlungu yesisu okanye ezinye iimpawu (ezinokuba ngumqondiso wokuba enye ingxaki ibangela i-jaundice)

Ukuba unembali yosapho ye-DJS, ingcebiso ngemfuzo ingaluncedo xa uceba ukuba nabantwana.

• Inkqubo yokugaya ukutya

I-Korenblat KM, iBerk PD. Indlela eya kwisigulana esine-jaundice okanye iimvavanyo zesibindi ezingaqhelekanga. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahl. 138.

ILidofsky SD. I-jaundice. Ku: UFeldman M, uFriedman LS, uBrandt LJ, ii-eds. Sleisenger kunye neFordtran's Gastrointestinal and Liver Disease. Ngomhla we-11. IPhiladelphia, PA: Elsevier; 2021: Isahluko 21.

URoy-Chowdhury J, uRoy-Chowdhury N. Bilirubin metabolism kunye nokuphazamiseka kwawo. Ku: Sanyal AJ, Terrault N, ii-eds. I-Zakim kunye neBoyer's Hepatology: Incwadi yesifo seSibindi. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 58.