Abstract

Patients with ostomies are at high risk of bacterial overgrowth. We report a case of a patient with PFIC-1 who had partial biliary diversion and secretory diarrhea. HISTORY: 3yo male with PFIC-1 diagnosed by biopsy and genetic testing. Biliary diversion done at age 18mo. Last winter after an acute “viral” illness he developed worsening jaundice and severe diarrhea. Treatment consisted of IV antibiotics for presumed cholangitis without significant improvement of his jaundice. Loss of large quantities of fluid and solid food from the stoma required IV normal saline supplementation of 1L/d. Attempt at endoscopic stomal cholangiogram was complicated by perforation. Exploratory laparotomy and intraoperative cholangiogram did not identify any obtruction. He was treated empirically with IV antibiotics, probiotics, and oral ciprofloxacin without improvement in the stool output (800 to >1000cc/day). Jaundice gradually resolved. Due to persistent foul smelling excessive drainage, pt was treated with rifaximin 200 mg TID for 2 weeks. Ostomy drainage decreased from averaging more than 1000cc/night to 400cc/night. His drainage became greener with no further visible food particles. Impression: this patient demonstrates secretory diarrhea likely due to bacterial overgrowth. Previous antibiotic therapy with ciprofloxacin was not broad enough to cover possible anerobic contamination. Rifaximin is an oral, non-systemic, gut-selective antibiotic with minimal absorption by the GI tract. Its in vitro activity is against both gram-positive, gram-negative, aerobic and anaerobic isolates. Its use in pediatrics has been limited to infectious diarrhea but it has been approved for use in the treatment of traveler's diarrhea in adults but not approved for use in children. It has been shown to be useful in gut decontamination for treatment of hepatic encephalopathy, and anecdotally in IBD and bacterial overgrowth in adults. Due to the disruption of his quality of life secondary to daily normal saline infusions, use of this non-approved agent was tried. Ciprofloxacin, which is not approved for use in children, was already tried without success. Metronidazole was not attempted due to its poor palatability as a suspension and also due to side effect profile. Rifaximin was well tolerated in our patient and had no side effects. Treatment was short term with long term results significantly improving our patient's quality of life.

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