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Abdominal Pain in a 17-Year-Old Girl With Pancreatic Insufficient Cystic Fibrosis

A 17-year-old Caucasian girl with known cystic fibrosis (CF; F508del/621 +1G > T), associated pancreatic insufficiency, and congenital hypothyroidism presented by telephone to the University of Minnesota Cystic Fibrosis Clinic with 1 week of low-grade fevers, fatigue, and 1 month of intermittent abdominal pain described as diffuse and crampy in nature. Her abdominal symptoms were thought to be secondary to a prior diagnosis of constipation and were relieved with as-needed polyethylene glycol. She also reported having an upper respiratory infection 1 week prior to when the fevers began; however, her respiratory symptoms had completely resolved without treatment. Given the duration of symptoms, she was instructed to be evaluated the next day at a local primary care clinic. At that time, she did not have an identifiable source of infection, she had no respiratory symptoms, and a monospot was negative; however, she had an elevated white blood cell (WBC) count. The primary care physician was concerned for an early CF pulmonary exacerbation so she was prescribed a 2-week course of oral ciprofloxacin. The following day, the patient called the CF clinic with a fever to 102°F, decreased appetite, and sharp upper abdominal pain that caused her to electively stop her high-frequency chest wall oscillatory therapy treatments. Secondary to these complaints, she was seen in the CF clinic where her abdominal pain was described as sharp and localized to the lower quadrants. She reported normal stools for the past few days, nausea, one episode of emesis, and a new productive cough. On presentation, she was tachycardic (heart rate 114) and tachypneic (respiratory rate 32) with a temperature of 100.1°F, blood pressure of 111/74, and an oxygen saturation of 94% on room air. Physical exam was significant for pallor and bilateral lower quadrant abdominal pain with guarding.