Abdominal Pain in Cystic Fibrosis

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Presents case of a 5-year-old girl with cystic fibrosis (CF). K.B.'s mother reports by phone that K.B. has had intermittent severe right lower quadrant abdominal pain for 2 days followed by fever up to 102 F and was seen in the emergency department, where an abdominal radiograph revealed constipation. K.B. was evaluated by and admitted to the surgery service. It was decided that K.B. was too great a surgical risk for an appendectomy. Therefore, a paracentesis was performed but was unsuccessful in draining the peritoneal fluid. After 3 weeks of oral therapy, a laparoscopic appendectomy was performed as an outpatient procedure. K.B. quickly recovered from the surgery and returned to a regular diet, regular activity, and to school. K.B. has continued to have occasional constipation, she has not had abdominal pain in more than a year. The patient’s medical history reveals that she was diagnosed with CF at 22 months of age. Since her diagnosis, K.B.’s lungs have been stable without pulmonary exacerbations and she has maintained a body mass index (BMI) percentile that is above the 75th percentile. The diagnosis of appendicitis is frequently delayed in patients with CF because of many factors, such as low incidence of appendicitis in patients with CF, abnormal appearance of the appendix in patients with CF at baseline on ultrasound, and difficulty in differentiation between Distal intestinal obstruction syndrome and symptoms of appendicitis.





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