Why Jaundice is Seen in Classic Galactosemia?

[@franciscobenitez1158]

I haven´t seen a better explanation of galactosemia an jaundice than this, greetings from Mexico. 😃😃

[@shaziafarooq7804]

Thanks for useful information

[@chinosamson2689]

Very nice explanation. Just one question sir. In your video you said that galactosemia causes UNCONJUGATED BILIRUBINEMIA. However most sources would say that galactosemia causes CONJUGATED HYPERBILIRUBINEMIA. What do you think is the explanation for that sir? Thank you.

[@dn6170]

Came here for this exact same reason and had the same question lol.... thanks for posting!

[@omarsanduka818]

After the increase in unconjugated bilirubin, there is no defect in its conjugation. This is why classic galactosemia is thought to primarily cause direct hyperbilirubinemia. However, further accumulation of unconjugated bilirubin may exceed the liver's conjugating ability (especially in a newborn), leading to a rise in both conjugated & unconjugated bilirubin. This is why UpToDate says that classic galactosemia can present with direct AND/OR indirect hyperbilirubinemia.

[@perrycox9889]

Thank You very much Professor Mungli. I would want do know how galactosemia determ fat degenaration, thank you!

[@mohammadadnan6374]

May God bless u

[@suyashsinghtomar301]

very nicely explained

[@DrMungli]

Thank you.

[@mhditb7817]

Great lecture sir

[@sohamghosh9657]

nicely explained...thanx sir

[@triptikapoor9575]

Thank you for the video sir. It was very helpful.

[@malenejensen2995]

Very informative. But why does the raised unconjugated bilirubin lead to jaundice?

[@DrMungli]

Unconjugated bilirubin is hydrophobic and diffuse into tissues to stain, specially elastic tissue.

[@nehakeshri933]

explained well tq

[@DrMungli]

Thank you.

[@mingmingandmikki]

thank you so much!

[@drafiyathaha5292]

Thankyou! Helped a lot!♥️😁 ...just happened to notice the comments...what is the hm park guy going on about sir? Could you please explain what he is trying to say?

[@premkiran4502]

Thank you so.much sir

[@DrMungli]

You are most welcome.

[@AWESEOMELMO]

thank u so much this helped so much :))))

[@mujtabaalawadi]

Thank you so much, that was really helpful 👍🏻👍🏻

[@DrMungli]

You are most welcome. I'm glad this video was helpful to you.

[@Jahangiralam-qg9dq]

Thanks sir..

[@DrMungli]

You are welcome.

[@postmelon645]

Thank you very much Dr. Mungli 👌🏻❤

[@pavelboiko4649]

year, nice. Except speed. This information can fit to 0.30 min video with 100 words/min rate

[@DrMungli]

True, but it could have been much faster. Nevertheless, thank you for the feedback.

[@hmpark9530]

LOL This cannot be more wrong! Although galactosemia can cause both indirect and direct hyperbilirubinemia, it most commonly present as direct hyperbilirubinemia, not indirect hyperbilirubinemia as mentioned in this video. You can never explain this with your concept.

[@DrMungli]

I'm explaining the mechanism of initial elevation of unconjugated bilirubin in classic galactosemia. Once the liver pathology sets in, both types of bilirubin are elevated. You can watch my video on pre-hepatic, hepatic and post-hepatic jaundice to why do you see elevated levels of both types of bilirubin in hepatic jaundice. Kindly read the excerpt from one of the article from Journal of perinatology. Early hyperbilirubinemia of galactosemia may be predominantly unconjugated, and can become conjugated. A hepatic origin is still plausible, as bilirubin conjugation may be inhibited by galactose-1-phosphate or other toxic metabolites that accumulate in galactosemia. www.nature.com/articles/jp2009136 Hope this helps.

[@hmpark9530]

What I want to point out is that yours is just a hypothesis, not a well-established theory. Accumulation of any toxic material in the liver can cause hepatic dysfunction which may lead to both indirect and direct hyperbilirubinemia. In this concept, the inhibiton of UGT becomes an unnecessary step. The article you mentioned above is a case-report, which in turn indicates that galactosemia rarely present as indirect hyperbilirubinemia in clinical situations. As I know, there are numerous different hypotheses for the pathophysiology of jaundice in galactosemia. I wish I could see the article/textbook you used for reference.

[@DrMungli]

I generally do not assume things while I teach. I have either referred to text or reference book or a indexed peer reviewed journal. Since you insist on giving the text reference, here is the reference from a book Advances in Clinical Chemistry the link I have given below. I will paste the link in separate comment. Please check your spam folder if KZbin detects it as spam. I'm also sharing links from research articles too.

[@DrMungli]

link for textbook Advances in Clinical Chemistry books.google.com/books?id=g7bgewdepRcC&pg=PA21&lpg=PA21&dq=galactose+1+phosphate+inhibition+on+phosphoglucomutase&source=bl&ots=JVKFb_zpAw&sig=DsJ4D-02Tl5CsrQDCmNC2XdKR1Y&hl=en&sa=X&ved=0ahUKEwjugcyxm8nZAhUCCawKHZukDcUQ6AEIYTAI#v=onepage&q=galactose%201%20phosphate%20inhibition%20on%20phosphoglucomutase&f=false

[@DrMungli]

www.ncbi.nlm.nih.gov/pubmed/18421797

[@atteeqfarooq]

Bkwaas

[@PresenceSanctuary-DrOkema]

Omg your voice really drags, so boring and monotone, I struggled to stay awake!

[@DrMungli]

Sorry about that, it is probably because I used iPad to make this video rather white board. Thank you for the feedback.