GASTROENTEROLOGY ARTICLE OF THE WEEK
February 21, 2008
Bloomer JR, McGuire BM. Intermittent unexplained abdominal pain: Is it porphyria? Clin Gastroenterol Hepatol 2007;5:1255-1258.
1. A 21-year-old woman presents to the emergency room with excruciating abdominal pain of 4 hours’ duration. On questioning,she has had 3 similar episodes of pain of a prolonged duration over the past 3 years. She has recently been training for a college cheerleading championship requiring dieting and strenuous exercise. Associated symptoms include nausea. She smokes less than half a pack of cigarettes daily. She takes tetracycline intermittently for acne but no other medications. Her physical examination is pertinent for a young woman in mild distress. Her blood pressure is 160/110, her heart rate is 130, and the remainder of her examination is notable for a benign abdominal examination. Which of the following features in the history are reported triggering factors for porphyria?
a. Cigarette smoking.
b. Dieting.
c. Tetracycline.
d. Strenuous exercise.
2. In a patient with recurrent abdominal pain and a negative evaluation, which of the following features of the history or physical examination should suggest the diagnosis of porphyria?
a. Hypertension and tachycardia.
b. Upper-extremity pain.
c. Muscle weakness.
d. Occurrence after puberty.
e. All of the above.
3. Which of the following is true regarding porphyria?
a. Approximately 80% of individuals who carry the gene defect have clinical manifestations.
b. The most common type of porphyria in the United Statesis variegate porphyria.
c. There is a male predominance of clinical disease.
d. The pathogenesis of the clinical manifestations is autonomic neuropathy.
e. There is no available treatment.
4. A 24-year-old woman presents with abdominal pain, upper extremity pain, and weakness associated with nausea and vomiting and constipation. On physical examination her blood pressure is 160/110, and her heart rate is 120. Her respirations are somewhat labored. She recently was given the diagnosis of acute intermittent porphyria by a marked increase of urine porphobilinogen. Which of the following should now be provided?
a. Intravenous normal saline without glucose
b. Promethazine for nausea.
c. Intravenous hemin only if the patient fails to respond to intravenous glucose
d. Cimetidine to treat the acute attack
e. Intravenous glucose as D10/NS and hemin.