Dubin Johnson vs Biliary Atresia

[spartan564]

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Why does biliary atresia cause cirrhosis but dubin johnson does not? In both, aren't you not able to excrete conjugated bilirubin?

 

[surgok]

Why does biliary atresia cause cirrhosis but dubin johnson does not? In both, aren't you not able to excrete conjugated bilirubin?

You can still excrete some in dubin johnson, so there's no total backup like in biliary atresia.

 

[spartan564]

Well in both cases don't you end up excreting most of it through your kidneys anyways as conjugated bilirubin is water soluble?

 

[surgok]

Well in both cases don't you end up excreting most of it through your kidneys anyways as conjugated bilirubin is water soluble?

only so much kidney can handle I would think.

 

[spartan564]

Yeah Im not sure about that either but it still doesn't make sense to me why you get cirrhosis in one case but not the other.

 

[Handinhand]

Because biliary atresia is going to block more than just conjugated bilirubin excretion; you are going to have no cholesterol excretion, no bile acid/salt excretion, no toxin secretion -- nothing.

Dubin-johnson is just a defunct transporter for your conjugated bilirubin, which is water soluble and can be excreted in your urine relatively harm-free.

 

[thehundredthone]

Short answer:

Because biliary atresia is going to block more than just conjugated bilirubin excretion; you are going to have no cholesterol excretion, no bile acid/salt excretion, no toxin secretion -- nothing.

Long answer: Ductular reaction. Damage to the blocked biliary ducts causes inflammation and reactive ductule formation along with fibrosis. A very interesting article outlines why Alagille syndrome, which also has ductopenia and cholestasis rarely progresses to cirrhosis.

... ductular reactive cells possess distinctive features with respect to “quiescent cholangiocytes” and actively participate to portal inflammation, producing a vast array of cytokines and chemokines, growth factors, and inflammatory mediators...