Essential Fructosemia and Hereditary Fructose Intolerance

[@tayyabanaz150]

Sir you've bcm my most fav biochem teacher. Your lectures are really really helpful. Thanku from PAKISTAN.

[@lilly-lk3mp]

Thank you for this great video!

[@dr.ruthsheeba3087]

Sir I'm a medicine student..Though i top other subjects..Biochem has never gone into my head..Last mnth i was jus struggling to read fr my cumulative exams of 2 yrs.Thts when suddenly i found one of ur video on youtube..Frm den i started getting thngs very easily..Tqsm Sir u hv made thngs better..!!! Hope u continue to make many more videos as well..God bless😊

[@DrMungli]

You are most welcome. I'm glad that you have found this channel. Consider SUBSCRIBING to this channel for regular updates. Please share with your friends and classmates.

[@dr.ruthsheeba3087]

Sure Sir..

[@kassa6870]

HELLO Dr. Mungli I have fructose lactose gluten free Intolerance and I have acidic body(can never eat or drink acidic foods. So what is your suggestion for your study I mean what kind of food should I have eat??? Thank you very much.

[@sagarboss2004]

Great, as usual

[@yomarie09]

Dr. Mungli, do you see lactic acidosis in HFI due to PDH complex activity decrease, accumulation of pyruvate and lactate production or am I wrong. I just have a small note of it and I wanted to clear that out.

[@DrMungli]

Yes there will be lactic acidosis (I forgot to say this in the video, now I have added annotation about that at 9:01 minutes). Lactic acidosis is due to slowing ETC as lack of Pi to support ATP synthase, because of slow ETC, NADH build-up in matrix of mitochondria leading to negative effect on PDH complex. This causes elevation of pyruvate and flow of pyruvate into cytoplasm where it is diverted to lactate leading to lactic acidosis.

[@williamarmstrong7199]

​@@DrMungli if our diet is that poor (inclusion of vegetables in our diet) then this may be an occasional issue. However most of use live a very healthy life without any vegetables and fruit in our diets. Because we eat red meat we do not suffer any deficiencies except for a shortage of vitamin C. Any amount of Fructose acts as a posion that may cause a "reaction" if there is too much of it. Brain fog and digestive issue will occure on fructose levels between 4g per day and 0g. The lower the better diet wise for fructose or other sugar alcohols like sorbitol.

[@williamarmstrong7199]

Several Key missing things in this video. 1/. Fructose in vegetables can be just as damaging as fruits! 2/. The clinical safe level of Fructose for an HFI person is 4g as an adult! Or 0.05g per kilo body weight. Above that damage will occure. However life on 4g a day as an adult will still be uncomfortable. Fructose is a posion to us in all that this means. 3/. Given as low a Fructose diet as possible HFI people live out a full normal lifespan with generally far less health issues that none HFI people suffer from. Indicating that diet is the cause of most people's ailments. Particularly the ultra processed foods that almost all have added sugar in them. This is to make people eat more of the product and therefore become obese. Or have other symptoms resulting from their diet. 4/. Hfi people rarely if ever get tooth decay unless they are given glucose as an unnessacerry suppliment

[@atlboy2002]

Great video. Thanks Dr. Mungli.

[@DrMungli]

You are most welcome

[@jameyatesmauriat6116]

Thank you for presentation.Can I suggest that you improve the videos by using mnemonics to help in memorization of all pathways? Also, if possible, add questions and explain them, this would greatly help in navigating all the names ,without this in my humble opinion, less would care about videos because students learned that long speeches won’t help them for the long term.

[@akashbhamwani9759]

sir can you explain why fructose in tolerance causes hyperurecemia??

[@teamammcri2224]

Dr_ Otaku26 phosphyration of fructose by fructokinade depletes cell pf ATP.pi levels decrease this will disinhibit ADA so more uric acid is produced.Hence hyperurecemia is seen.Hope this helps.

[@michaeljamesmartin]

I am dealing with cancer and had my dna test say I had HFI. I don't have symptoms listed. I wanted to ask... i am currently on a diet that has high apple and carrot juicing. I feel i am tolerating it however I am not sure if this is detrimental to my intent to heal with this diet. you mentioned it's a decrease in production of Adolase B .. Is there a way to measure how much of a problem this might be for me? also should I do another test to confirm what the DNA test found? I really need some clarity on how to know if this is causing homeostasis in my body for healing Cancer.

[@DrMungli]

I'm not really expert in medical treatment of this rare disease other than knowing its metabolic effects. One thing that could help is to restrict foods that contain sucrose to keep fructose levels low.

[@umesh3077]

U may have fruktokinase deficiency as told in lecture that's asymptomatic and harmless

[@frankenz66]

I have one of the two variants missing they test for. Supposed to be missing both to have an active case of HFI it says.

[@user-ic4is1ej5x]

I hope you're better now🙏❤️

[@Ankitpaswan378]

@@user-ic4is1ej5x 😂

[@alimz4891]

Hi, I'm suffering from hereditary fructose intolerance, is there a cure for it?

[@williamarmstrong7199]

Yes a 100% fructose free diet and you will soon start to feel better than you have ever felt in your life!

[@rmu1486]

Mungli good job

[@MuhammadIrfan16973]

nice description

[@DrMungli]

+Muhammad Muslim : thank you, I'm glad this video has helped you.

[@surenthar6032]

Sir why is the same intolerance and distaste for fructose not happens in fructosuria?? Fructosuria too increases the fructose level... It's odd to believe that it will not cause any symptoms... Kindly clear this doubt...

[@williamarmstrong7199]

Fructosuria has no negative side effects like vomiting or symptoms of posioning that is why. The avoidance of sweet flavours and fruits is a pavlovian reaction that is learnt if children with HFI are given glucose as sweets this can be broken leaving their life outcome very much damaged as a result. It is only the avoidance of even tiny amounts of sugars that keeps us safe.

[@younusgujjarladla403]

why jaundice occuring in this case sir?

[@williamarmstrong7199]

If it is occurring then the person (most likly a child) will be close to total liver failure and death. He misses out vegetables which all contain fructose to some degree. We have to avoid ALL sources of Fructose to be safe. If we do then living a productive normal life we'll into our late 80's is normal even for men.

[@Darkgospel]

It was kind of hard to understand your english, hahaha Even though this was a very good video. Thank you C:

[@Darkgospel]

Greetings from Mexico.

[@DrMungli]

Thank you. I'm glad you liked the video despite little difficulty in following me.