This activity is intended for gastroenterologists, emergency medicine physicians, primary care physicians, and other healthcare providers involved in the diagnosis and management of porphyrias.
The goal of this activity is to improve the recognition of the signs and symptoms of acute intermittent porphyria (AIP), the ability to establish a diagnosis, and appropriate tailoring of management options for patients with AIP.
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CME / ABIM MOC / CE Released: 3/24/2021
Valid for credit through: 3/24/2022, 11:59 PM EST
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The following cases are modeled on the interactive grand rounds approach. The questions within the activity are designed to test your current knowledge. After each question, you will be able to see whether you answered correctly and read evidence-based information that supports the most appropriate answer choice. The questions are designed to challenge you; you will not be penalized for answering the questions incorrectly. At the end of the activity, there will be a short post-test assessment based on the material presented.
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Eliza is a 32-year-old white female who visits her OB/GYN with recent onset of severe lower abdominal pain, severe constipation, nausea, vomiting, and headache. She was referred by her primary care physician (PCP) for evaluation of possible endometriosis. She has visited the emergency department (ED) three times during the last two years with similar symptoms. At the first two visits, she was discharged without a diagnosis. At the third visit one year ago, she had a temperature of 38°C and mild leukocytosis. Although Eliza's abdominal pain was not localized to McBurney's point, and abdominal/pelvic computed tomography (CT) scans showed nothing remarkable, she underwent appendectomy for presumptive appendicitis. The pathology report noted no evidence of acute or severe inflammation or edema; there were scattered lymphocytes in the wall of the appendix, and the final impression was "mild chronic appendicitis."
The OB/GYN performed a laparoscopy and did not find evidence of endometriosis. On physical examination, Eliza's abdomen is soft, with no peritoneal signs. She is afebrile but has tachycardia (HR 115 bpm) and hypertension (BP 165/100 mm Hg). She is sweating profusely and appears distressed. A medical student obtaining Eliza's symptom history elicits that her abdominal pain started 18 hours ago and gradually progressed from diffuse mild cramping to severe "stabbing" in the lower abdomen. Her constipation started 10 days earlier, and nausea and vomiting began as her abdominal pain worsened. She has been avoiding food and drink and denies using alcohol, nicotine, or prescribed or recreational drugs. Eliza has regular menstrual cycles; her last period started 14 days ago, and she expects her next period in about 12 days. She denies being sexually active. She reports that her urine is a reddish-brown color since the pain started. Eliza reports that her aunt was hospitalized once for similar symptoms.