this brief study describes all the basic aspects of Wilsons disease , including management. as this is a congenital abnormality associated with severe complications on the future proper diagnosis management , and lifestyle modifications , …..surgical options are also needed ,if necessary . thank you martinsuja369@gmail.com

1. WILSONS DISEASE –A BRIEF
MEDICAL STUDY
PREPARED BY
DR. MARTIN SHAJI

2.  Wilsons disease is a
genetic disorder, where
there is too much copper
in the body.
 Small amounts of copper
are needed in the body,
but to much can be
hazardous to the liver,
brain, eyes, and other
org...
 Another name for Wilson's disease
is Hepatolenticular degeneration.
 The special proteins in the liver are
called Hepacytes.
 Degeneration of the liver and the
central nervous system.
A BRIEF DESCRIPTION

3. How It Related To Bile Secretions…
• Copper is absorbed
from your food, and
any excess is excreted
through bile, required
for the digestion of
food, is excreted by
the liver into passages
In Wilson disease,
copper does not pass
from the ...
Wilson's disease is
named after Dr.
Wilson, a doctor at
the National
Neurology Hospital
in London.
He discovered the
disease in 1911

4. • Wilson's disease is caused by
a
mutation located on the 13th
chromosome called ATP7B.
• Mutations in the ATP7B gene
produce a protein with
decreased
ability to bind copper.
Inheritance
• Autosomal recessive disease
In order to inherit Wilson disease,
both parents must carry one
genetic mutation
• Wilson disease carriers, who
have
only one copy of the abnormal
gene, do not have symptoms

5. Symptoms
 Wilson's disease first attacks the liver, the
 Central nervous system, or both.
 Swelling of the liver or spleen
 Jaundice
 Fluid build-up in the legs or abdomen
 Fatigue
 A tendency to bruise easily

6. COMPLICATIONS

7.  Chelating agents such as d-penicillamine and
trientine.
Other agents include sodium dimercaptosuccinate,
dimercaptosuccinic acid, zinc, and tetrathiomolybdate.
MANAGEMENT
patients who are de-coppered and dependent to
penicillamine. This medicine combines with excess copper
in the body and may prevent your body from absorbing
the copper in the foods you eat.(this is an exclusive drug
for Wilson's disease)

8.  The use of surgical decompression or trans jugular
intrahepatic shunting (TIPS) in the treatment of portal
hypertension is reserved for individuals with recurrent or
uncontrolled variceal bleeding that is unresponsive to standard
conservative measures.
 Liver transplantation or prolong period or severe cases in
accordance with the opinion of surgical gastroenterologist and
hepatologist.
 Healthy diet
 Zinc salts (zinc acetate) act as inductors of
metallothionein's, which favor a negative copper
balance and a reduction of free plasmatic copper.
must follow a diet that is low in copper.
Foods to avoid include beef liver, cashews, black-eyed peas, vegetable juice,
shellfish, mushrooms, and cocoa.
People with this disease should not take any vitamin or mineral supplement
that contains copper

9. THANK YOU