Curriculum
Module 05 · 65 min
Lipid & Amino Acid Metabolism
Beta-oxidation, ketogenesis, urea cycle, and the bedside-relevant inborn errors.
CoreClinicalResearch
Topics
What this module covers
- 01Beta-oxidation and carnitine shuttle
- 02Ketogenesis and DKA
- 03Urea cycle and hyperammonemia
- 04Branched-chain amino acid disorders
Learning objectives
By the end of this module you will be able to
- L01Trace a fatty acid from cytoplasm to acetyl-CoA via the carnitine shuttle.
- L02Explain when and why ketogenesis occurs.
- L03Recognize urea cycle defects from clinical presentation.
Expected takeaways
What you should walk away believing
- →MCAD deficiency presents with hypoketotic hypoglycemia after fasting.
- →DKA combines insulinopenia, lipolysis, and unrestrained ketogenesis.
- →Hyperammonemia in adults with no liver disease should prompt urea cycle evaluation.
Core summary
At the Core level
Fats and proteins enter central metabolism through dedicated pathways: beta-oxidation chops fatty acids into acetyl-CoA; transamination feeds amino-acid carbon skeletons into glycolysis or the TCA. Disrupting either pathway has dramatic clinical signatures.
Evidence-graded claims
Claims, scored A–F
A
Newborn screening can detect MCAD before first crisis
Standard of care in most high-income countries.
C
Ketogenic diet cures epilepsy
Evidence supports specific pediatric epilepsies; not a universal cure.
Quiz
Check your understanding
Q1. Carnitine shuttles…
Flashcards
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Front
MCAD deficiency presentation?
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