Spectrum of emergency department presentation in patients of acute intermittent porphyria: experience from a North Indian tertiary care center.

摘要

Of the porphyrias, acute intermittent porphyria (AIP) is the most frequently encountered porphyria. The clinical characteristics of thirteen patients of AIP who presented to the Emergency Department were analyzed. The most common precipitating factor was drugs. Eleven patients presented with pain abdomen. Neurological manifestations included: Seizures in six and motor weakness in six. Of the four patients with hyponatremia, three had associated neuropathy and the fourth patient demonstrated a severe course marked by pontine-extrapontine myelinolysis and profound adrenergic activity. In conclusion, even though AIP is less frequently reported from India the emergency physicians should be vigilant to exclude the diagnostic possibility of AIP in a patient with an appropriate clinical setting.