Von Gierke's Disease (Type 1 Glycogen Storage Disease)

[@Anonymous-007]

No one can teach von girke disease better than u

[@DrMungli]

I'm glad that you liked this video.

[@patriciamaciel6507]

Very clear explanation. I will recommend this to my medical students!

[@shreyaskanthadi3263]

Perfect Recap of VG's Dr. Mungli! Thank you!

[@DrMungli]

+Shreyas Kanthadi Thank You Shreyas. I'm glad you found the video useful.

[@beaniforst7021]

Thank you Dr. Mungli ! Reminded me just of your class; feeling better already!

[@DrMungli]

You are most welcome Beani. I'm glad you found these videos useful.

[@DrSD-lu5lv]

Awesome lecture sir!!! thanks a ton!!

[@mumugugu8033]

Easily the best explanation on KZbin.! thank you

[@aaogee]

Thanks for detailed definition, here I Wana share my own experiences : I am suffering in Glycogen storage disease by birth. Now my age is 50 years, my pa, grand Pa and or previous all my family was not having this disease but I am, however one of my son out of 3 is same like me having this disease by birth. I passed all my life with compromising with this disease because I couldn't get any medical help through the life so couldn't find any medicinal treatment. The exercise makes me feel better and the only way to survive. Now I m applying exercise therapy on my child now aged 9 and he is passing more better and active life than me as I passed.

[@ziyumonjasimuni72]

Will you pls explain exercise

[@rakshitkulkarni8510]

Tnqqq sir for such easy and understand able explaination

[@DrMungli]

Rakshit, I'm glad this video has helped you. Make sure to SUBSCRIBE to this channel for more such videos and SHARE with your friends.

[@Lola-fb3hk]

Thank you Dr. Mungli! This video is a great review after class.

[@DrMungli]

Thank You Linyer. I'm glad you found the video useful.

[@Stocks_4]

wonderfully explained !!👍👍 I am so thankful Sir. so many concepts got cleared in 19 minutes. 😊

[@DrMungli]

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

[@100.aishwaryakonda2]

Thanks for the very clear explanation

[@sarojkhanal708]

sir, i have one question, can you please tell me age and sex characteristic of Von Gierke's disease,

[@DrMungli]

This disease follow autosomal recessive condition that means this disease will be seen equally in both male and female. Age of onset of the signs and symptoms is in childhood by two years of age.

[@AdamAdam-te6ye]

Amazing explanation. Many thanks.

[@ankanpal3521]

Awesome 👍

[@juancarlosgierkegac4120]

Saludos a todos , buena información,

[@sarojkhanal708]

liked and subscribed, Your videos are very informative sir, thanks for uploading

[@DrMungli]

Thank you Saroj Khanal. I'm glad these videos have been helpful to you. Kindly share with your friends.

[@sarojkhanal708]

yes sir, already shared with friends,

[@manishkharel895]

Can fatty acid synthesis and beta oxidation of fatty acid synthesisis can occur at same time

[@sudhanshusuman618]

Very nice explanation sir' thanks for great lecture 👌

[@DrMungli]

Thank you Sudhanshu Suman, let me know if you have any specific video request which could be helpful to many others too.

[@Tashfeenali1]

Respected Dr. What is the diet guide for GSD type 3, as my kid is GSD patient and is 1.5 years old and is only being fed banana and corn starch. Please send me some diet plan for my kid.

[@ziyumonjasimuni72]

Sir pls give dietitian chart for this disease

[@susudiwa7101]

thank you so much sir

[@DrMungli]

🙏 you are welcome

[@salmantaheri2748]

hello sir i was just wondering how would it be possible to have both increases ketone body synthesis and hyperlipidemia how is that possible?

[@DrMungli]

Salman Taheri: Good question. This happens in the liver. Under fasting condition, as G6Pase is deficient in these patients, G6P is diverted into different pathways, that includes glycolysis to yield pyruvate and then acetyl CoA. Now this acetyl CoA can get into ketone bodies specially under fasting and can produce ketone bodies. Now in the same when TCA cycle is saturated leading to accumulation of citrate could take this citrate out of the mitochondrion into cytoplasm to divert it into fatty acid synthesis although not at high rate. But under well fed condition in these patients almost entire acetyl CoA can be diverted into fatty acid synthesis leading to accumulation of fatty acids in the form of TAGs. Note that this type of biochemical change could take months to be seen clinically significant.

[@salmantaheri2748]

Thank you doctor

[@brijeshbachpalle98]

Thank you very much sir. it was very helpful.

[@DrMungli]

You are most welcome. I'm glad this video helped you. You could consider SUBSCRIBING to the channel for regular updates and also SHARE the video with your friends.

[@nomiyousaf1235]

Great explanation 👍🏻

[@ziyumonjasimuni72]

Pls reply my child suffering from GSD type 1 . can I give milk And biscut to him

[@bibijan9790]

Sir my child is suffering from gsd 9th b ....im giving my child cornfour ....nd now what should be done....sir im giving diet food also sir

[@fueelcalvo]

Thank you

[@nehakeshri933]

tq very well explained

[@step_zero]

great video , you are awesome !

[@ranonepal6851]

wonderful lecture.thank you very very much sir

[@DrMungli]

You are most welcome.

[@rovirat45]

Perfect sir👍👍👍👍

[@rawan7870]

Thanks for this helpful video . I have two questions : 1- what cause deficiency of G-6-Pase ? It is hereditary ? . 2- Are -all- these diseases you Mentioned appear in patient who suffering from (GSD1) disease ,or just some of them ? ( can you tell me what are your references, because I'll write a review about this disease and I need good references ).

[@DrMungli]

Thank you. I'm glad this video has helped you. This is an autosomal recessive disease and all the signs and symptoms explained here is for this disease only. I refer Mark's textbook of biochemistry, Devlin's textbook of biochemistry, Harrison's textbook of internal medicine. Kindly SUBSCRIBE for regular updates and SHARE with your friends.

[@globalvision9036]

What if the person is in a ketogenic state. There body isn't using glucose as the main fuel source.

[@nawalmariki6744]

Fantastic

[@Bbbbbb-qn5nq]

Very comprehensive

[@shrishtisharma8318]

Thanku so much sir

[@frigtartsxp]

What is a good diet for this disease besides fish?? What type of protein??

[@DrMungli]

Frequent carbohydrate meal

[@frigtartsxp]

Dr.Mungli what does that mean? Can you give some examples? Thank you doctor

[@chloeporporo653]

frigtartsxp cornstarch mixed with water every few hours around regular meals (wish I was kidding but I’m not lol). I have Type 1A 😊

[@frigtartsxp]

Chloe Porporo ohh trust me I know, my brother has it too :( .. do you also get massive nosebleeds that take forever to stop?

[@chloeporporo653]

frigtartsxp YES lol they were horrific. But I’ve gotten both sides of my nose cauterized after about 4 times lol so that helped that a lot. I used to have nosebleeds that would last hours and really heavy. One time it bled so hard it came out of my tear ducts (terrifying lol) In my research I saw it’s common to have Von Willebrand disorders with GSD so not sure if that’s why. My one friend who also has GSD 1A said she never had a nosebleed ever (I was so jealous haha) so I guess it’s only some patients

[@softtonyj]

thnk u sir..

[@DrMungli]

+Tony Joseph Thank You. I'm glad you found the video useful.

[@arikeomowunmi2711]

I want know the treatment for this

[@chloeporporo653]

Cornstarch mixed with water every few hours (literally lol). I have Type 1A 😊

[@arikeomowunmi2711]

Chloe Porporo ok

[@arikeomowunmi2711]

Like how many hours

[@chloeporporo653]

arike omowunmi for me it’s 3 or 4. But it varies patient to patient based on their metabolism and weight

[@arikeomowunmi2711]

Mine 2 years old has it, type 9

[@laveena425]

❤️❤️❤️