OSCE and Learning points

OSCE AND LEARNING POINTS

PREFINAL PRACTICAL EXAMS

NAME : VAGISHA RANI

ROLL NO: 138

DATE: 4 December '23

Blog link:

https://vagisharani.blogspot.com/2023/12/48-year-old-male-patient-from-west.html

QUESTIONS 

1) West haven criteria clinical grade for hepatic encephalopathy.

2)Wilson's disease and cirrhosis of liver 

Wilsons diease is an inborn errors of copper metabolism characterised by increased total body copper.

Etiology:

Molecular defect within the copper transporting ATPase encoded by gene ( ATP7B) located on chromosome 13

Pathology: 

• Copper enters the body through the digestive tract with the help of a transporter protein in the cells of the small bowel, copper membrane transporter 1 (Ctr1; SLC31A1).
•  In response to rising copper levels, an enzyme called ATP7A releases copper into the portal vein to the liver. Liver cells carry the CMT1 protein and metallothionein, and then ATOX1 binds it inside the cell. Once here, ATP7B links the copper to ceruloplasmin and releases it into the bloodstream, removing the excess copper by secreting it into bile.
• Both functions of ATP7B are dysfunctional in Wilson disease. 
• Copper accumulates in the liver, and ceruloplasmin is secreted in a form that lacks copper and is rapidly degraded in the bloodstream.
• When the copper level in the liver overwhelms the proteins that normally bind it, it results in oxidative damage through a process known as Fenton chemistry. This damage results in chronic active hepatitis, fibrosis, and cirrhosis.

Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7330668/

3) Autoimmune hepatitis

• Autoimmune hepatitis is when the body's infection-fighting system (immune system) attacks your liver cells.
• It is a long-term chronic liver disease that causes inflammation and liver damage.
• Experts don’t know what causes it, but it is more likely to appear in people living with other autoimmune conditions. Certain medications can also trigger autoimmune hepatitis.
• It affects more women than men.
• It can lead to scarring of the liver (cirrhosis) and liver failure.

4) Alpha 1 antitrypsin deficiency leading to cirrhosis

• Alpha 1 anti trypsin deficiency is the leading genetic cause of liver disease among children. 
• It is a protein-folding disorder in which toxic insoluble ATZ proteins aggregate in the ER of hepatocytes leading to inflammation, fibrosis, cirrhosis, and also increased risk of hepatocellular carcinoma.

Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5780543/#:~:text=Summary,increased%20risk%20of%20hepatocellular%20carcinoma.

LEARNING POINTS 

• Formation of rapport is an essential part of doctor and patient relationship.
• I understood the importance of taking detailed history.(Even a symptom like insomnia could be a first finding suggestive of hepatic encephalopathy).
• I learnt how other causes like Wilson's disease, hemochromatosis and alpha 1 antitrypsin can lead to cirrhosis of liver.
• We discussed about various etiological factors which can lead to liver damage.(Since my patient is an alcoholic so previously I was more confined to an alcoholic cause of for his liver damage.)
• I learnt about various tests for hepatic encephalopathy.