Abstract
SESSION TITLE: Tuesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/22/2019 01:00 PM - 02:00 PM INTRODUCTION: Pancreatic enzyme replacement therapy (PERT) improves nutrition and growth in patients with cystic fibrosis and pancreatic insufficiency. CASE PRESENTATION: A 2-month old term female presented with bloody stools and poor weight gain. Her medical history was significant for cystic fibrosis (ΔF508 and 1210-2_1210-1delAG splice site mutation), complicated by pancreatic insufficiency, meconium ileus, and in-utero bowel perforation requiring postnatal bowel resection. Patient was discharged from the NICU on PERT and 22 kcal per oz of Pregestimil formula. She was followed by a nutritionist who recently increased her formula to 26 kcal per oz. Her PERT was increased from 3000 to 6000 lipase units per feed. Abdominal radiographs revealed peripheral, bubble lucencies around the bowel concerning for extensive pneumatosis. Patient was admitted for management of necrotizing enterocolitis (NEC). She was kept NPO and started on 7 days of IV piperacillin-tazobactam and parenteral nutrition. Repeat studies showed resolution of pneumatosis. Contrast enema was reassuring with no sign of stricture. Patient was restarted on oral feeds on Day 8. Within 72 hours, patient developed abdominal distension. Repeat imaging revealed new evidence of pneumatosis, requiring another course of antibiotics and bowel rest prior to slow re-initiation of feeds. 1 month after second occurrence of NEC, patient was discharged on full feeds of 22 kcal per oz formula with PERT at 6000 lipase units per feed. Due to continued difficulty gaining weight, patient was later readmitted for gastrostomy tube placement. To facilitate more uniform administration of PERT, an immobilized lipase cartridge (Relizorb) was used in-line with her continuous tube feeds. Though this route of PERT administration was not approved for use in infants, good outcomes were reported in children as young as 5 years old. Patient's weight increased over the next month from 4.08 to 4.46 kg. She was discharged home with no complications. DISCUSSION: It was concluded that the inciting event for this patient’s necrotizing enterocolitis was likely the increased caloric density of formula. Little data exists to describe the incidence of NEC in infants with cystic fibrosis, but it is assumed to be rare. Careful reintroduction of enteral feedings is paramount in NEC and may be problematic in cystic fibrosis patients who receive tube feeds, as they often require pancreatic enzyme replacement to prevent bowel obstruction. Traditionally this requires interruption of tube feeds with periodic administration via enteral tube. In this case, an in-line immobilized lipase cartridge was utilized successfully to provide consistent enzyme administration during tube feeds. CONCLUSIONS: Our case suggests there may be a benefit for additional study of immobilized lipase within the pediatric population in order to address and improve malabsorption challenges in cystic fibrosis patients. Reference #1: Borowitz DS, Grant RJ. PR, the Consensus Committee. Pancreatic Enzymes Clinical Care Guidelines. J Pediatrics. 1995; 127:681-84. Reference #2: Paranjape S, MD and Mogayzel PJ, MD, PhD. Cystic Fibrosis. Pediatrics in Review. May 2014, 35 (5) 194-205; https://doi.org/10.1542/pir.35-5-194 Reference #3: Abbo O, Harper L, Michel JL, Ramful D, Breden A, Sauvat F. Necrotizing enterocolitis in full term neonates: is there always an underlying cause?. J Neonatal Surg. 2013;2(3):29. Published 2013 Jul 1. DISCLOSURES: No relevant relationships by Manuel Izquierdo, source=Web Response No relevant relationships by Nicole Stephenson, source=Web Response No relevant relationships by William Wooten, source=Web Response
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