ALA Dehydratase Deficiency Porphyria Treatment & Management
- Author: Smeeta Sinha, MD; Chief Editor: Emmanuel C Besa, MD more...
Treatment of the acute attack of ALAD deficiency porphyria (ADP) should begin by removing the precipitating factors. This includes discontinuing drugs that induce the cytochrome P-450 system, promptly treating infections, and maintaining a high-carbohydrate intake.
Treatment considerations are as follows:
- Pain control can be safely achieved with parenteral morphine.
- Nausea, vomiting, and anxiety may be safely treated with phenothiazines.
- Acute attacks can cause respiratory muscle paralysis; airway protection should be frequently assessed and mechanical ventilation implemented, as needed.
- Seizure control may be achieved with benzodiazepines; alternatively, gabapentin and vigabatrin are two antiepileptic medications safe for use in ADP. Correcting underlying hyponatremia is essential.
- Treat tachycardia and hypertension with beta-blockers.
- Intravenous hematin should be administered during acute attacks of ADP (see Medication).
Liver transplantation has not been shown to be useful in treating ADP.
Patients with ALAD deficiency porphyria (ADP) should maintain a diet high in carbohydrates, consuming at least 300 g of glucose daily. Glucose inhibits ALA synthetase, thus decreasing ALA levels.
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