Understanding Wilson's Disease
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- Опубліковано 5 вер 2024
- This video contains a detailed and simplified explanation about Wilson's disease. We discuss cause of Wilson's disease, the pathophysiology, presentation, investigations and management.
CORRECTION The motor symptoms in Wilsons disease are often ASYMMETRICAL. The video states the motor symptoms are typically symmetrical, which is inaccurate.
CORRECTION A LOW serum caeruloplasmin is indicative of Wilsons (not a raised level).
More written notes and diagrams about Wilson's Disease are available on the website at www.zerotofinals.com/wilsons.
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DISCLAIMER: This video is for education and entertainment only, and is not medical advice. This video should NOT be used for medical advice or to guide clinical practice. The Zero to Finals content should not be used in any way to guide medical decision making. Zero to Finals takes no responsibility for any actions taken or not taken based on the information provided. Local and national guidelines and senior clinicians are there to help you make decisions, not UA-cam videos. If you need medical advice or information, seek it from an appropriately trained and licenced doctor or healthcare provider that can address your individual needs. Zero to Finals cannot guarantee the accuracy of information in this video. Please highlight any errors you notice in the comments below - thank you.
I was extremely ill with Wilson's Disease in the late 1970s and all through the 1980s. My symptoms were neurological and psychiatric. I have been fine for decades and have a completely normal life.
I have Wilson disease Wilson disease
Can i talk to you?
@@user-ze1vn3fw4w Of course! You can contact me here or message me on Facebook.
@@loriespohn6109 hey
So happy for you Lorie. I wish you a long, happy and healthy life. My doctor suspects I might have Wilson’s disease and we are to find out on Monday/Tuesday. Not gonna have a very relaxing weekend 😞
My son has this disease he's late he has not walked for 1 year a go . Can I ask you about willson?
My aunt and uncle are first cousins and they married 25-30 years back. Today, their 3 out of 6 children have this disease. They take copper chelating medicine on a regular basis and that’s what helps them get rid of excessive copper in their body. To whomsoever this may concern, you can live with the disease and have a normal lifespan if you promptly treat the disease. Wishing you happiness and praying for you to have a long and prosperous life.
are they normal now? if yes then please suggest the medicines and treatment?
❤
As a person who has been diagnosed with the actual disease myself this video helps
I've had this desease since 2001. It almost killed me, but i'm doing great now. My liver is almost back to normal again. I still have some neurological things like cramps and muscle stiffness. I had all the symptoms, both neurological and liver. good video.
Plzzzz help me maam ...my younger sister suffering from this disease ...i want to talk u plzzzzzz mam help me or suggest wht should we do.her copper,serum is 5.18
Ceruloplasmin is 9.18
My gmail a/c is sweetishikasaxena@gmail.com
Plzzz contact me maam
Kristin Clem Could you, please, tell us how you manage your condition?💝🌺🌹🌹🌸
@@anilsaxena8494 hi anil please contact dr. Anu agarwal she is the best doctor to treat on this disease in India. She is operating in kokilaben Ambani hospital in Mumbai. Hope its helpful.
Hi, can I ask you a question about your treatment? Do you use Trientina or Penicillamina? I need this answer because I saw in my book that Penicillamina is not indicated with neurological problems...is that true?
@Kristin Clem Thank you for your post and can you please tell how you were able to manage all of your symptoms. Thank you in advance!
Not even a medico, still knows almost everything in brief 😅😅.. Credit goes to zero to finals.. ❤️😍😍😍😍😍.. Keep up the work brother.....
Thank you
- In Wilson's disease, ↑ Free serum copper, but ↓ total serum copper.
- Total body copper consists of ∼ 10% free copper and ∼ 90% ceruloplasmin-bound copper.
- As tests detect copper bound to ceruloplasmin, total serum copper concentrations decrease with decreased serum ceruloplasmin levels.
- Note that due to reduced incorporation of copper into apoceruloplasmin, less ceruloplasmin (copper transport protein) is formed.
I have started ayurvedic treatment for one patient and result is just a miracle
I was diagnosed with Wilson’s disease in 92 and I learned some stuff today
Can i talk to you?
I have Wilson disease
Can you contact me
Great presentation, very clear, consise and informative. Thanks
Zero to finals- please respond because my son is in the Memorial Hermann hospital in the Houston, Tx. Medical center due to cirrhosis of the liver caused by Wilson’s disease. The more I learn about this disease the more I realize how terrible it is. He is depressed, but I thought that was mostly due to the overall situation. He hasn’t shown neurological signs otherwise. He was perfectly normal and fine up until about two months ago. Fatigue, vomiting blood and other symptoms drove him to the ER. It still took several hospital visits before these “doctors” realized and correctly diagnosed what was wrong.
As a man of fifty-two years and extensive experience with hospitals and doctors, the best advice I could give to any medical student, nurse or doctor is this: NEVER PRESUME A DIAGNOSIS OR ASSUME THAT A PATIENTS LIKELY CONDITION IS THE CORRECT AND TRUE DIAGNOSIS!!! The doctor automatically assumed, and in fact accused my son of being an alcoholic because he had severe cirrhosis of the liver. At twenty four years old.
Sure, sounds logical to me.
Asshole. Like most “medical professionals” are. Don’t be that.
Amen! I was misdiagnosed by 7 neurologists before one got it right as Wilson's Disease. They all thought the shaking of my hands was an intention tremor, which is something far less
far less serious that my mother had. I lost the ability to walk and talk before someone finally figured it out.
My son has Wilson's disease with liver, neurological and psychiatric symptoms, been on penicillamine for 10 years, very poorly by the time he was diagnosed aged 26
Is penicillamin is working for him ...did u try any other medicine like trientine,syprine...my younger sister is also suffering from this disease..plz help me if u can
Contact me - sweetishikasaxena@gmail.com
I have Wilson disease and penicillamin is working for me
This is a very helpful video. I have a friend who has Wilson's Disease.
Excellent presentation and I hope your endeavors were successful. Very informative and you have a gift for presentations. Thanks again.
The genome was mapped and the Wilson's gene has been found. It is now known that the Wilson's disease defect is in the binding of copper. It is a defect in the ATP7B gene and there are 6 binding exons on the gene. There are over 400 mutations that have been identified and more are being added to the database each year. In most mutations, only part of the exons aren't binding to ceruloplasmin, which still allows the body to bind and evacuate some copper, so the free copper gradually builds over time. In the rare severe mutations none of the 6 exons are binding copper and the free copper builds up in childhood. The defect is not in the inability to produce ceruloplasmin, as once thought, but the disease is caused by defective ceruloplasmin that won't bind copper. There are 2 types of copper, bound and unbound. Bound copper is non toxic and actually used by the body, but the unbound is toxic and like a heavy metal. In a normal person 5-10% should be unbound copper. How do you estimate free, or unbound to ceruloplasmin copper? You can do it by 2 methods. The old method, which is not that accurate is called an indirect measurement of free copper. You have your Ceruloplasmin (Cp) tested, along with your serum copper. Then you multiply the Cp x 3 and subtract it from the total serum copper which will give you the amount of free copper. Remember, the serum copper by itself will not be a diagnostic tool. The serum copper is a measurement of the total copper both bound and unbound. You must estimate the ratio of bound to unbound to see if you have a binding problem which is Wilson's disease. The 2nd method is a direct measurement of free copper lab test and it's the most accurate. Only a few labs are doing this test in the US, and Lab Corp is one of them. The free copper test # at Lab Corp is 279071. This is the test number at Oklahoma Lab Corps, and they may differ at the Lab Corp in your state. As far as the 24 hour urine copper test, it is not worth a sh*t. Medical literature says that this is the most accurate test, but it's worthless. Control group studies have been done and come to find out, people with Wilson's disease actually secrete less copper out their urine that normal people do. The only way this test is any good is if it's used with the penicillamine challenge, where they administer the drug to mobilize copper from the tissue before the test. Penicillamine is so toxic and has so many side effects that it should not be used period. There are much safer drugs out there. Penicillamine has put people into a permanent vegetative state because it is too aggressive at mobilizing the free copper and floods the brain with free copper. Zinc is a safe but slow chelator of copper. The compound ammonium tetrathiomolybdate (TM) is also safe and much faster than zinc. If you have one of the mutations where the liver is storing the copper, you need to use trientine. New research is now showing that liver involvement is rare in Wilson's disease. It's still a mystery why some people's livers start storing the copper and others don't. Family members with the same mutation have been found and only one person in the family has copper liver storage. New research is also showing an elevated free copper is the cause of schizophrenia, Parkinson's, ALS, Alzheimer's and Tourettes. Anyway, you can't rely on the medical books when it comes to Wilson's disease because they have inaccurate outdated information. If you think you have it, just go get the direct measurement of free copper lab test done. If the free copper is high, this means you have a problem. If the free copper is below normal, this means you also may have a problem, because when the liver starts storing the copper, it causes it to be low in the blood. The body produces Ceruloplasmin by the amount of copper it detects in the blood and when the liver starts storing it and the blood becomes low, the Ceruloplasmin production will be turned off by the body and it will become low. Most people with Wilson's will have a normal serum copper and a normal Ceruloplasmin. As mentioned before, it's not an inability to produce Cp, but it's defective Cp that can't bind copper that causes the problem. Just get the free copper test and start taking zinc 50mg 3 times a day, one hour before or 2 hours after meals. Zinc is a primer for metallothionein in the intestines, which latches on to the copper. Since the intestinal cells turn over every 8 days and new ones are formed, the old metallothionein copper filled cells are sloughed off out the stool and the process starts all over. This is how zinc gets copper out of the bloodstream. Zinc also blocks its absorption. It takes 9-12 months for zinc to bring a person to a non toxic copper state, whereas TM can do it in 2 weeks. In 8 weeks TM can totally decopper the body whereas zinc will take several years. Once decoppered you still need to take zinc daily to prevent the free copper from building back up.
Hello Captain America, I have Wilsons Disease Zinc is NOT a Chelator its a blocker. Penicillimene and Trientine are both Chelators. You cannot use Zinc alone to treat initially only after successful chelation has happened. Also as you mention copper is related the genetic test is simple enough ATP7B is easy to find just over 400 mutations. I was diagnosed over 27years ago with 24hr and liver biopsie. Although other diseases use ATP7B its the liver where all the interesting stuff occurs.
Thank you so much for taking the time to inform so specifically. Appreciate it very much.
It's amazing how much has sprung from genome mapping.
Thank you for your very informative comments above!
Note to all! Above all else, please remember that healing is supercharged when we maintain a positive mental state.
Love and pleasantries ❤
Blood Ceruloplasmin level is decreased in case of wilson's disease.....
you are right!
@@ZeroToFinals how is with sexual intimacy with people who have Wilsons disease? Anything good to know?
Good point, this wasn't actually clarified in the video! Thanks!
True
Amazing video, as always. Entertaining, and above all informative!
Thanks!
@@ZeroToFinals you are a big inspiration for me! Can I ask how do you make the animations?
@@howtomedicate Thanks, what a compliment! You're channel looks great. Looks like you have recently got going. Keep up the good work and you will build a big audience. It might take time but keep uploading! I record the audio first. Then use an ipad to make the illustrations whilst my computer captures the screen. Then I edit the video so that the recording matches the audio. Hope that helps
Each video takes between 10-20 hours of work
@@ZeroToFinals Thank you for your answer! It means a lot to me! I am now trying to improve the quality of my videos by starting editing aswel, and improving my video/camera quality. It might help haha.
Maybe one day we can make a video together! That would be great!
i got this condition in DOCTOR HOUSE M.D SERIES S1E6 and got interested. thanks alot
I came from watching Dr House but I was looking up Hemacromatosis which is like Wilson's disease but with iron
KF rings aren’t usually visible to the naked eye. In most cases they are only visible via a slit lamp examination.
What are the home remedies
Thank you to make it easy
Hi, I have a question -
My notes say there should be:
reduced serum caeruloplasmin
reduced serum copper (counter-intuitive, but 95% of plasma copper is carried by ceruloplasmin)
increased 24hr urinary copper excretion
Presumably serum caerulosplasmin and copper are reduced because the copper is all deposited in the tissues.
But why is there Increased urinary excretion? I thought the whole thing is that it's trying to hold on to copper so there would be less excreted? Thank you!
I think it’s because of chronic hepatitis that causes decreased liver function, that in time damage the kidneys
Plus the tubular renal damage can cause increased urinary excretion, due to renal failure
Wilson disease ruined my brain, i used to be extremely violent. My old grandma tought i was posessed specially when the tremors came.
How did u get diagnosed? Blood urine tests?
wow. what a prolonged thing to have to suffer. Psychiatrists assume things and give drugs not needed that only masks real problems. Shut you up, and call you a hypochondriac. It can take years and several doctors to diagnose correctly. I gave up on all, knowing how rough it can be. May I ask if you ever had an MRI, and if so, did they find multiple "lesions" or any in white matter. No one has been able to tell me what those are from. I've had high iron and copper too. I feel for everyone going through a gamut of tests that don't get properly diagnosed, or called psychiatric.
Thanks for this video
Two family members died from Wilson disease
And now Two more fighting Wilson disease
May Allah bless them
Thank you my friend a lot .
All video is highlight
You have made a great video.
Thanks!
Hepsinbelirtilerivar ben de gözümde ķaybettim rabibim yardımcımız olsun wilson hastalığın yakalanbenimgibi olan arkadaşlar ında bütün hastalara şifa versin amin
I came here because if the episode of house. Interesting disease.
Thank you for the video!
Thank you
why would serum copper be low
Thanks alot,, very informative
Thanks!
Can Wilson's disease also cause adhd In some people?
My face changes shapes everyday and it prevents me from going out and I have Wilson’s disease and I’m wondering if that is the cause of it
I’m 54 and this has been happening my whole life
Great video
Verry good explanation
very helpful
Thank you❤
Sir when i was 15 years old my urinary cooper was 411ug /d is this normal or very high now I'm 18 years old I'm feeling better than that
So helpful
Nice and precise
thankyou, its so helpful
amazing video thank u
thank u soo much
Thank you sir!
Very pleasant voice
Dude! I have this!!
Excellent
Thanks for the info , Just watched house and searched for this lol
why serum copper low in Wilson disease when it should be high?
Normally, the serum contains ceruloplasmin which is the protein that carries copper in the blood. Due to the insult on the liver, the production of ceruloplasmin is decreased. The result is decreased TOTAL serum copper levels, however, due to the accumulation of copper in the body and lack of ceruloplasmin, the FREE serum copper is increased. It is the free serum copper that ends up depositing in tissues that causes the manifestations of Wilson’s disease.
@@TheTovin you are amazing!the first person that explains that in such a good way.I had never understood whats was wrong with the cerruloplasmin and the free cooper.Thank you so much!
Low ceruloplasmin indicates Wilson's not high. This is inaccurate information
They said that it could be FALSELY elevated in people with cancer and inflammation. Basically don't rely on ceruloplasmin solely in cancer patients and so on...?
My little sister died by this disease in 2018!
I'm so sorry for that horrific loss. So hard, especially with prolonged illness and suffering.
Why the serum copper is decreased ? I am lost.
Because, the copper is accumulated inside liver cells. And cp remains as apoenzyme(not active). So there is no way cu get out of cells. So cu cannot enter into blood.
Loved this ❤
HI my brother has suffering from Wilson disease ...plz tell me how to treat it...
High or low serum copper?
Very nice video
My doctor told me yesterday. I might have Wilson's disease.
I have one but I'm 13 I im scared
thank you alottt
Hello, i am from Bangladesh. I have wilson disease.
So i want to talk with you doctor.
Hello brother can i talk with you?
l also wilson disease. 0:52
Brillant Vid
Thanks Mansour!
they call it Hepatolenticular degen. now
I just read it in my book
1 person got their question on Wilson's Disease in their exam wrong
Good
Hi i am from India.. I have willsion disease 🙁
Hi tripathi what tests did you undergo for confirming this disease. ?
Penicillianine
Trientene
Serum Cu ?
My brother died from this disease and my sis has it😢
in the vid 2:10 "parkinsonism symmetrical" in notes "Motor symptoms are often asymmetrical in Wilson disease" - which one is it?
Well spotted. Please see the video description: The motor symptoms in Wilsons disease are often ASYMMETRICAL. The video states the motor symptoms are typically symmetrical, which is inaccurate.
انا واخي توأم مصابين بي مرض داء ويلسون
I bid a very good bye and safe journey to my dear most sweetheart😔 who has lost her life of the disease called Wilson's disease. I pray that Father in heaven shall lead me till I breadth last and we shall meet again in heaven
I have low copper serum even after supplementing 3 mg for a year. My ceruloplasmin was 22 but I have inflammation problems. How did you get your wife diagnosed? Liver biopsy? 24 hour urine?
Reading the books of the New Testament, we perhaps asked ourselves more than once: *"Why 2000 years we do not see those miracles that accompanied the Сhurch of Christ in the I century, as described in the New Testament?"* Why do the so-called preachers of Christ have to prove that Jesus really existed and atheists boldly deny the historicity or divine origin of Christ? Maybe because the Сhurch of Christ has not existed for 2000 years?
The Сhurch does not exist in the form in which it is presented in the books of the New Testament, but there are Catholic, Orthodox, Protestant and other christian sects claiming to be the place of the Church, but they not have the only thing that distinguishes the divine from the human and is characteristic of just the Сhurch of Christ -the reinforcement of the word with signs, that is, miracles (Mark 16:15-20). Therefore, some researchers doubt the historicity of Christ, and some of them are not opposed to declaring him a an ordinary philosopher, teacher. But even if Jesus were an ordinary philosopher, his disciples would be ordinary followers of Jesus. And they would not dare to write about the miracles that not only Jesus, but also his disciples, could perform. If there were the Church in our time as described by the authors of the New Testament books, where miracles are performed, the sick are healed, where prophesied, and the dead are raised, no one would doubt the historicity of Christ. Then there would be the same controversy throughout the world as in the first century - Jesus the Son of God or the false prophet who seduces the world by miracles. As a result, we can say that the emergence and development of christian sects and atheism was the result of the fact that over the 2000 years the Сhurch of Christ did not exist.
Find *"The Mystery about the Church of Christ"* video on UA-cam. The video reveals the prophecy of the disappearance and reappearance of the Church of Christ before the End of the World. Watching this video will give hope to all who sincerely seek God and will interest those who are not too lazy to think freely. Click on my name to watch the video (The video is in Russian, but English subtitles are included).
♥
💌
the white background is blinding af at night...c'mon now...
Hair test holy mother
Our last name!
This kid thinks he's a vampier he eats dark chocolate if you see his eyes they have copper in them he has broses on him and his girlfriend but no copper in her eyes 👁🧠🦴
.
Wilson deases ..is a bad luck..
dude i fell asleep
Honestly providing false information in your lessons even if there are only a couple of them is a big no-no in these kind of videos. You should double check the informations that you are providing!
If you think there is misinformation, then why don’t you put that in your comment? What false information?
o w ta ad atp
Thank you