I need the answer in term of lipids metabolism. Time 11:55 AM. A sixteen-year-ol
ID: 255470 • Letter: I
Question
I need the answer in term of lipids metabolism.
Time 11:55 AM. A sixteen-year-old girl is rushed to the emergency room due to extreme fatigue during P.E. Patient History -
Since young she has had recurrent episodes of extreme fatigue.
- Episodes occurred only if she fasted for more than 8 hours
- Her mother routinely gave her food late at night and woke her early for breakfast
- Her developed height and weight are normal
- Today, she missed breakfast and by noon was extremely fatigued. Treatment in ER At the hospital she was immediately given glucose intravenously. Her symptoms responded dramatically.
Her initial blood work showed: Serum glucose was low (38 mg/dl; normal is 70-100) Blood urea slightly elevated Free fatty acid levels were elevated Ketone bodies were below normal
1-What is wrong with her? Explain.
2- Which enzyme is defective? Explain.
3-What treatment will help her? Explain.
Explanation / Answer
She is having low ketone bodies that is caused by fatty acid oxidation defects. Hence due to defect in fatty acid, oxidation, fatty acids are not able to break into acetyl-CoA in the mitochondria. That resulted, in the less energy in her to do any process and got fatigue. Enzyme defective in this condition is Acetyl –CoA synthetase. She has to take diet rich in fats and drink lot of glucose and water.