Defects in Urea Cycle and Hyperammonemia

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  • Опубликовано: 6 янв 2025

Комментарии • 67

  • @izzyawe5799
    @izzyawe5799 5 лет назад +18

    My baby sister, River has ASA she is doing well but can't have much protein at all. She is 11 months old and standing almost walking. Please wish her luck in life knowing she might not have the life we anticipated for her 💖

    • @arjunforest3097
      @arjunforest3097 3 года назад

      You probably dont care but if you guys are stoned like me during the covid times then you can watch pretty much all of the new series on instaflixxer. Have been streaming with my brother for the last couple of weeks =)

    • @uriahallen7212
      @uriahallen7212 3 года назад

      @Arjun Forest yup, have been using Instaflixxer for months myself =)

    • @izzyawe5799
      @izzyawe5799 3 года назад +5

      @@arjunforest3097 this comment is literally talking about my sister who could potentially die and you talk about getting high? Damn.
      But an update: she still has ASA but is doing very good, she is a healthy baby girl who is walking and lives to swim. She is 2 years old now and is starting to talk 💖

    • @quangthuongtran4364
      @quangthuongtran4364 3 года назад

      @@izzyawe5799 these guys are probably just spam, just ignore them. Also congrats on your sister's health!

  • @AswinSSoman
    @AswinSSoman 7 лет назад +11

    You breakdown complex reactions in such a way that it no longer fells complex.
    HATS OFF for the effort you put for that.👍👏

  • @patrickvanmeter2922
    @patrickvanmeter2922 5 лет назад +1

    Finally what I have been looking for. I can't thank you enough for this. Liked and subscribed.

  • @AnneSgambatoFox
    @AnneSgambatoFox 8 лет назад +5

    i wonder why he doesn't have a lot more views, but anyways i rlly liked the way that you explain things! Also, your drawings and organization makes me jealous hahaha Congrats and Thanks! :)

  • @franckynjantou8124
    @franckynjantou8124 4 года назад

    YOU ARE JUST THE BEST AND ONLY THE BEST IN EXPLAINATION

  • @hanahdetalla6257
    @hanahdetalla6257 8 лет назад +39

    Doctor's comment: excellent explanation, very comprehensive, and well organized.
    Fan girl comment: How do I marry you and your brain?! 😍😳😛

    • @khalidyar
      @khalidyar 7 лет назад +5

      try to become hyperammonemia patient than go to him for treatment and during the course of treatment you can get a proposal from him ... although its a risky route to take ... :)

    • @Byrial
      @Byrial 6 лет назад

      @@khalidyar I didnt laugh as i actualy excperienced hyperammonemia, but people are allowed to make fun of anything imo.

    • @khalidyar
      @khalidyar 6 лет назад +2

      @@Byrial it was not on a serious note .. frankly speaking my 3 years old child is going through the same condition and he was in hospital 2 days ago .. . but some time we have to laugh out our challenges . It gives us strength and motivation to to stay firm and keep fighting . if you are been heart I apologize from the depth of my heart.. I am really sorry ..

    • @annegonzalez6683
      @annegonzalez6683 5 лет назад

      Let her get your number 👩‍⚕️

    • @Rebecca-zn8cz
      @Rebecca-zn8cz 5 лет назад +1

      Midway through the lecture i was thinking; i am sure someone is thinking what im thinking and... sure enough! ( i’m two years late but still! Haha)

  • @haniachailover6590
    @haniachailover6590 4 года назад +1

    Best presentation, simple , concise and to the point..Thank you ❣

  • @sheeshsheesh1778
    @sheeshsheesh1778 2 года назад

    very well done,Alhumdullilah you explained it fully and indeed it is conceptual.

  • @iqbalmusthan287
    @iqbalmusthan287 5 лет назад

    you are excellent . we need people like you.

  • @heroho149
    @heroho149 8 лет назад +2

    Very beautiful explanation.
    Thank you very much.

  • @naveenbabu4057
    @naveenbabu4057 3 года назад

    Thank you sir for ur invaluably simple explanation.

  • @nabeelalipkhan23
    @nabeelalipkhan23 2 года назад

    You are making med studs life easier, can u pls make a video regarding microsomal oxidation?📹

  • @Jefsieb
    @Jefsieb 6 лет назад +2

    Excellent video !

  • @collobanks
    @collobanks 7 лет назад +1

    very nice...well expounded and comprehensive lecture.thumbs up 👍👏

  • @niusha3323
    @niusha3323 3 года назад

    I dont know how to thank you enough

  • @RyanF0800
    @RyanF0800 2 года назад

    Great vid keep up the stella work you star

  • @sebastiana3525
    @sebastiana3525 8 лет назад +3

    Beautiful explanation. Had fatigue and brainfog for 3 years sudden onset. Was deficient in Ornithine, Citrulline and BCAAs, but had hight Arginine. Amino Acid therapy helped a bit. Still have brain fog but docs cant figure out the cause since my organs look fine, my liver too. I dont have OTC deficiency (23and me showed that), so we don't know what caused the amino acid deficiency despite a high protein diet. Taking BCAA help me not to lose weight. Don't know if I should take glutamine, my docs know nothing about that.

    • @Byrial
      @Byrial 6 лет назад

      Do you have high ammonia? In that case i wouldnt take glutamine obviously. Im not sure if an ornithine deficiency is a strong indication of elevated ammonia.

    • @sineadehorgan
      @sineadehorgan 5 лет назад

      Also, 23 and me is not a sensitive enough genetic test for this kind of condition

  • @McFwoupson
    @McFwoupson 4 года назад

    I got hyperammonemia from depakote (valproic acid, seizure medication) and almost died from it. Epic experience.

  • @mswendypie
    @mswendypie 6 лет назад +1

    You are a genius!!! Thank you

  • @Byrial
    @Byrial 6 лет назад +1

    So what does it take to make, lets say the carbomoyl phosphate synthelase enzyme work? Any minerals etc that support this function?

    • @wendylyles2204
      @wendylyles2204 5 лет назад

      Probably not because its a genetic defect

  • @viswanadhpolisetty1362
    @viswanadhpolisetty1362 4 года назад

    Cool lecture. A doubt why are we getting rid of glyine> How does hyperammonemia lead to a rise in glycine levels.. is it just that are trying to get rid of the amino acid pool in the body or just that we have a mechanism of excretion of hippurate so we make use of glycine

  • @Bactino
    @Bactino 8 лет назад +3

    Nice sir!

  • @MikeFrame
    @MikeFrame 8 лет назад +1

    Great presentation and leaves me curious why nitric oxide wasn't part of this cycle. Also is Urea another possible way to treat a cycle defect and why is Glycine a problem? And what about Keto Acids binding to Ammonium for safe removal?

  • @abdullah.khan98
    @abdullah.khan98 4 года назад

    Sir I have a question related to clinical significance of urea cycle ! So can I add these disorder that you have added in this video ? Help me

  • @noname-dy4yv
    @noname-dy4yv 7 лет назад +1

    very nice sir......but u should zoom on the writhing so that we can see whats written......please write a bit larger......(sorry 4 my bad eng)

    • @booloob
      @booloob 7 лет назад +1

      just full screen and watch in hd

  • @benellison3546
    @benellison3546 6 лет назад +1

    how does the body get rid of excess arginosuccinate?

    • @Byrial
      @Byrial 6 лет назад

      By converting it to Arginine through the arginase succinate enzyme?

  • @wizardatmath
    @wizardatmath 2 года назад

    Adding exogenous arginine for arginosuccinase deficiency makes no sense. Given your explanation, adding ornithine would make sense. Furthermore, you must mention the vitamin, mineral cofactors.

  • @sumanrajbahak3540
    @sumanrajbahak3540 7 лет назад +1

    Why high level of glutamine is sought in bodybuilding industry then? It needs to be corrected.

  • @BossChronicles
    @BossChronicles 7 лет назад +1

    how do you reverse a fatty liver?

    • @AKLECTURES
      @AKLECTURES  7 лет назад

      stop the cause i.e stop drinking.

    • @BossChronicles
      @BossChronicles 7 лет назад +1

      i don't drink alcohol maybe a few times a year socially small amounts i think it's from red meat

    • @AKLECTURES
      @AKLECTURES  7 лет назад

      you should go speak with your primary care physician.

  • @bobohung1195
    @bobohung1195 7 лет назад

    For argininosuccinase deficiency, can we treat the patient by using ornithine replacement instead of arginine?

    • @wendylyles2204
      @wendylyles2204 5 лет назад

      Hey I know you posted this a year ago but I just had the same thought. You couldn't because arginine is needed to combine with water to form urea (from what I understand)

  • @sumanrajbahak3540
    @sumanrajbahak3540 7 лет назад +1

    Does this mean excess glutamine is adverse to our body?

    • @dmarlhole
      @dmarlhole 4 года назад

      Yes! "Excess" is the operative word.

  • @mir_kaminov
    @mir_kaminov 6 месяцев назад

    2:48 reaction

  • @mj-je7el
    @mj-je7el 4 года назад

    Thats my nightmare right now 💔😥😥😥😥😥😥😥😥

  • @khalidyar
    @khalidyar 7 лет назад

    is hyper ammonium fully curable if diagnosed in infants

  • @fadyalhosain9170
    @fadyalhosain9170 8 лет назад +2

    very graet

  • @kezzaman
    @kezzaman 3 года назад

    Arginine supplement does help with Ornithine Transcarbamylase Deficiency, apparently*

    • @Eye-it-azz
      @Eye-it-azz 2 года назад

      No, why do you need arginine supplement ?

    • @kezzaman
      @kezzaman 2 года назад

      @@Eye-it-azz ask Google

  • @mj-je7el
    @mj-je7el 4 года назад

    Could someone Translate this in danish???? 🙏🙏🙏💖

  • @mlgshariquekhan8326
    @mlgshariquekhan8326 8 лет назад

    Respect

  • @Alaghbari86
    @Alaghbari86 5 лет назад

    why I did not find you earlier 😭

  • @mrbob1959
    @mrbob1959 2 года назад

    🙏🙏🙏

  • @younottube9802
    @younottube9802 6 лет назад +3

    من اجل نزوه لا تتجاوز بضع لحظات يولد انسان ليشقى من ستين الى سبعين عام 😃الامور قد تبدو عبث ولكنها سنة الحياه ، فى بداية الامر حيوان منوى من الاب يندمج مع حيوانه منويه من الام 😁ثم تحدث عدة انقسامات وتحوارت شكليه وتمايزات جسديه فى رحم الام طيلة 9 اشهر ليولد طفلا ضعيفا هشا ثم يكتسب من بعد ضعف قوه واخيرا فى النهايه من بعد قوة ضعفا على النمط التالى طفل ضعيف >شاب مغرور بقوته >واخيرا كهل عجوز لو طبقنا هذا على واحده من اهم موجبات الحياه للإنسان لكانت مثالا مطابقا لحياتنا اولا الحيوان المنوى هو الCO2 والحيوانه هى الNH4 😁يندمجا معا ليتكون الطفل الCarbamoyl phosphate يتمايز ويكبر داخل رحم الام حتى يكتمل تمام يخرج الى الحياه وهى (cytoplasm ) على هيئه citrulline هذه المرحله كلها داخل رحم الام وهى ال mitochondrial matrex 😉اما بدايه الحياه تاخذ الام هذا الطفل الرضيع الضعيف ال citrulline وتغذيه بالaspartate حتى ينضج ويصبح فى سن ما قبل البلوغ اى قبل الاعتماد على النفس وهى الargininosuccinate حيث كلمة argi تعنى ما وكلمة nino قبل وsuccinate تعنى البلوغ وهذا من معجم اللغة الزومبيه الوجيز 😂يريد الطفل المميز اى القادر على تميز الاشياء ان يتحرر من سطوة والديه الى اكتشاف قدراته ومهاراته وهى فترة المراهقه فيفقد حنان والديه على هيئة fumarate ليصبح شابا ناضجا فى ريعان شبابه اسمهarginine ثم بعد الشباب يفقد قوته وهى الماء H2O ومن الماء الحياه فاذا فقد فقدت الحياه يدخل الماء على الشاب العفى Arginine ويتركه وهو عجوز كهل وهو ornithine ويعطيه ولدا اسمه الurea ليعيد دورة حياة ابيه هذا كله فى الcytoplasm ويقولون فى الامثال لفى بينا يا urea دوخينا يا urea خدينا يمين شويه خدينا شمال شويه وادينا عايشين يا urea 😂وشكرا 😃

    • @rya8042
      @rya8042 5 лет назад

      Saleem Abutaleb واضح انك شربان شي 😂🥃

  • @mairakhan1350
    @mairakhan1350 3 года назад

    Plx made this lecture in Urdu