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Anesthetic considerations in hepatic porphyrias
1Rowan Regional Medical Center, Salisbury, NC 28144, USA.
CRNA : the Clinical Forum for Nurse Anesthetists
|October 3, 1999
Summary
Porphyrias are inherited disorders affecting heme synthesis, presenting symptoms that can mimic surgical conditions. Management focuses on avoiding specific drugs and utilizing glucose infusions to prevent or treat attacks.
Area of Science:
- Biochemistry
- Genetics
- Internal Medicine
Background:
- Porphyrias are inherited metabolic disorders impacting heme biosynthesis.
- Clinical presentations can be diverse, often mimicking acute surgical conditions.
- Certain medications can precipitate acute porphyric attacks.
Purpose of the Study:
- To outline the key features of porphyrias relevant to surgical presentations.
- To emphasize drug-induced triggers and safe management strategies.
- To highlight the role of glucose in porphyria management.
Main Methods:
- Review of clinical manifestations of porphyrias.
- Analysis of drug-induced exacerbations.
- Discussion of therapeutic interventions, including drug avoidance and glucose administration.
Main Results:
- Porphyrias can present with symptoms like abdominal pain, vomiting, tachycardia, hypertension, and neurological disturbances.
- Barbiturates, benzodiazepines, and ketamines are among the drugs that can provoke attacks.
- Glucose infusions are crucial for preventing and treating porphyric crises, as starvation can induce attacks.
Conclusions:
- Effective management of porphyria necessitates avoiding specific trigger medications.
- Non-triggering pharmacologic agents should be selected for patients with porphyria.
- Glucose administration is a vital component in the prevention and treatment of porphyric episodes.