Recurrent abdominal pain since childhood or infancy, possibly precipitated by meals, lasting two to seven days. Attacks can occur every few weeks to every few years.
Pain starts epigastric, but later involves the entire abdomen and radiates to the back.
Severity and frequency of attacks improves with age. First described in 1952, autosomal dominant gene with incomplete penetrance. Males and females equally affected with all reported cases in caucasians. Etiology is unknown, but many patients have congenital malformations of the ductal system or sphincter of Oddi. It has been linked with hyperthyroidism, hyperlipoproteinemia, and aminoaciduria.