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Anaesthesia and pyruvate dehydrogenase deficiency
Canadian Anaesthetists' Society Journal
|July 1, 1983
Summary
Pyruvate dehydrogenase deficiency in infants can cause dangerous metabolic acidosis during anesthesia. Careful anesthetic management, avoiding certain drugs and solutions, is crucial for patient safety.
Area of Science:
- Biochemistry
- Pediatric Anesthesiology
- Medical Genetics
Background:
- Pyruvate dehydrogenase deficiency (PDHD) is a rare genetic disorder impairing cellular energy metabolism.
- Infants with PDHD struggle to metabolize pyruvate, leading to lactate accumulation and metabolic acidosis.
- Anesthesia presents unique challenges for patients with metabolic disorders due to altered physiological states.
Observation:
- A 10-month-old infant with diagnosed PDHD experienced increased arterial lactate levels and metabolic acidosis during two separate anesthetic procedures.
- Different anesthetic techniques were employed, yet both instances resulted in similar adverse metabolic events.
- Potential contributing factors identified include the direct effects of PDHD, anesthetic agents inhibiting gluconeogenesis (e.g., halothane, thiopentone), hypocarbia, and high carbohydrate intake.
Findings:
- The patient's PDHD exacerbated metabolic acidosis under anesthesia, indicated by rising lactate levels.
- Anesthetic agents like halothane and thiopentone may worsen acidosis by inhibiting gluconeogenesis.
- Hypocarbia (low carbon dioxide levels) was also implicated as a potential contributor to increased lactate.
Implications:
- Anesthesiologists should exercise extreme caution when managing patients with PDHD.
- Recommendations include avoiding halogenated anesthetics and lactate-containing intravenous fluids.
- Maintaining normocarbia (normal carbon dioxide levels) and ensuring a stress-free anesthetic environment are vital for mitigating risks.