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Urea cycle disorders in critically Ill adults.
Micah T Long1, Jacqueline M Kruser2, Shane C Quinonez3
1Departments of Anesthesiology & Internal Medicine, University of Wisconsin Hospitals and Clinics.
Current Opinion in Clinical Nutrition and Metabolic Care
|November 8, 2023
Summary
Urea cycle disorders (UCDs) can cause severe ammonia elevations and neurologic injury. Early screening and comprehensive management are vital for preventing irreversible harm in both neonates and adults.
Area of Science:
- Biochemistry
- Neurology
- Genetics
Background:
- Urea cycle disorders (UCDs) disrupt ammonia metabolism, leading to hyperammonemia.
- Severe hyperammonemia causes irreversible neurologic injury, often diagnosed in neonates.
- Mild UCDs can present in adulthood during physiological stress, necessitating broader screening.
Approach:
- This review emphasizes the importance of recognizing and screening for UCDs in adults with unexplained neurologic symptoms.
- Management strategies focus on reducing ammonia production, preventing catabolism, and enhancing ammonia clearance.
- A comprehensive, multitiered approach, including critical care, is essential for neuroprotection.
Key Points:
- Hyperammonemia's neurologic injury is time- and concentration-dependent.
- Broader screening for mild UCDs in adults is increasingly recommended.
- Exogenous ammonia clearance is a recognized adjunct therapy.
Conclusions:
- Understanding UCDs is crucial for clinicians to prevent severe neurologic injury or death.
- Timely diagnosis and management of UCDs can prevent patient deterioration.
- Focused therapy combined with supportive critical care improves outcomes for UCD patients.
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