A 34-year-old female has a history of intermittent episodes of severe abdominal pain. She has had multiple abdominal surgeries and exploratory procedures with no abnormal findings. Her urine appears dark during an attack and gets even darker if exposed to sunlight. The attacks seem to peak after she takes erythromycin, because of her penicillin allergy. This patient most likely has difficulty in synthesizing which one of the following?
The patient has acute intermittent porphyria, which is a defect in one of the early steps leading to heme synthesis. The buildup of the intermediate that cannot continue along the pathway leads to the dark urine, and it turns darker when ultraviolet (UV) light interacts with the conjugated double bonds in the molecule. Erythromycin is metabolized through an induced P450 system, which requires increased heme synthesis. This leads to metabolite buildup to the level where the abdominal pain appears. The defect in heme synthesis does not affect creatine phosphate, cysteine, thymine, or methionine levels.
Heme
Creatine phosphate
Cysteine
Thymine
Proteins and Amino Acids