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Wilson´s Disease

by Carlo Raj, MD
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    We're going to our second metabolic disease of the liver: And this is Wilson's disease. As soon as you hear Wilson's, close your eyes, your telling me what element? Copper. Copper. Copper. That is where you begin. Hemochromatosis, what is your element? Iron. Iron. Iron Pathology. Wilson – the copper is causing damage, once again from head and down to you liver. If copper accumulates in the basal ganglia, what does it look like? And what's your walk? Shuffling gait. What about your flexion of the elbow? cogwheel rigidity What is all this? This is a sign of often awful lot like your Parkinson's But it is not Parkinson's It is Parkinson-like right? Be careful, so the copper here is accumulating at the basal ganglia giving symptoms in your patient of Parkinsonism. Next, I'll show you the iris; the copper accumulating in the iris. That is know as Kayser-Fleischer ring. What's the topic here? Liver disease. The copper accumulating in your liver’s causing death to your liver. Let's talk about pathogenesis, it's important Autosomal recessive; excessive accumulation of what kind of copper? What do you mean what kind of copper Dr. Raj? Copper is copper. No. In the body, they're either bound or free. Which faction of copper is elevated? What is this copper accumulating on the basal ganglia, and the iris, and the liver and so forth? Free copper. Why? The liver is missing the binding protein for copper. Do you remember the name of that is? We will see. Presents under the age of 40, you're right. I know you are. But let's just make sure you know, we have the full picture first. Age, about 40, young, hemolytic anemia, jaundice, elevated ALT, or fulminant liver, neurological symptoms. There you have it, Free copper accumulating in different places,...

    About the Lecture

    The lecture Wilson´s Disease by Carlo Raj, MD is from the course Cirrhosis – Liver Diseases.


    Included Quiz Questions

    1. Ceruloplasmin deficiency
    2. Sphingomyelinase deficiency
    3. Galactosidase deficiency
    4. Glucocerebrosidase deficiency
    5. Arylsulfatase deficiency
    1. Kayser- Fleischer rings
    2. Golden color of skin
    3. Increased ALT
    4. Pruritis
    5. Icterus
    1. Basal ganglia
    2. Uncus
    3. Hippocampus
    4. Medulla
    5. Cerebellum
    1. Descment's membrane
    2. Iris
    3. Anterior chamber
    4. Pupils
    5. Lens
    1. Increased urinary copper
    2. Decreased urinary copper
    3. Increased ceruloplasmin
    4. Increased stool copper
    5. Decreased stool copper
    1. Total copper levels - Low Bound copper levels -Low Free copper levels- High Ceruloplasmin levels -Low Liver biopsy - Increased free copper
    2. Total copper levels - High Bound copper levels -Low Free copper levels- High Ceruloplasmin levels -Low Liver biopsy - increased free copper
    3. Total copper levels - Low Bound copper levels -High Free copper levels- High Ceruloplasmin levels -Low Liver biopsy - increased free copper
    4. Total copper levels - Low Bound copper levels -Low Free copper levels- Low Ceruloplasmin levels -Low Liver biopsy - Increased free copper
    5. Total copper levels - Low Bound copper levels -Low Free copper levels- High Ceruloplasmin levels -High Liver biopsy - Increased free copper
    1. Phlebotomy
    2. D-penicillamine
    3. Trientene
    4. Zinc maintenance
    5. Liver transplant

    Author of lecture Wilson´s Disease

     Carlo Raj, MD

    Carlo Raj, MD


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