Dimensions of critical care nursing : DCCN
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Dimens Crit Care Nurs · May 2006
Case ReportsAbdominal pain due to acute intermittent porphyria: when is the sound of hoof-beats not horses, but zebras? A case report.
Acute intermittent porphyria is a rare autosomal dominant hereditary inborn error of metabolism of the heme biosynthetic pathway that can be exacerbated through a multitude of environmental factors. This article is a case study describing the pathophysiology, clinical presentation management, and exacerbation prevention of acute intermittent porphyria. ⋯ Supportive treatment includes opiate analgesia, monitoring for and treating complications such as hypertension and hyponatremia. Follow-up should include family counseling regarding genetic defects and individual counseling regarding lifestyle changes including avoidance of environmental factors that have been implicated in the exacerbation of the disease.