Tom K Lin1, Maisam Abu-El-Haija1, Jaimie D Nathan1, Joseph P Palermo1, Bradley Barth2, Melena Bellin3, Douglas S Fishman4, Steven D Freedman5, Cheryl E Gariepy6, Matthew J Giefer7, Tanja Gonska8, Melvin B Heyman9, Ryan Himes4, Sohail Z Husain10, Quin Liu11, Asim Maqbool12, Maria Mascarenhas12, Brian McFerron13, Veronique D Morinville14, Chee Y Ooi15, Emily Perito9, John F Pohl16, Sue Rhee9, Sarah Jane Schwarzenberg3, Uzma Shah17, David Troendle2, Steven L Werlin18, Michael Wilschanski19, M Bridget Zimmerman20, Mark E Lowe21, Aliye Uc22. 1. Cincinnati Children's Hospital Medical Center, Cincinnati. 2. University of Texas Southwestern Medical School, Dallas. 3. Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, MN. 4. Department of Pediatrics, Baylor College of Medicine, Houston, TX. 5. Harvard Medical School. 6. Nationwide Children's Hospital, Columbus, OH. 7. Seattle Children's Hospital, Seattle, WA. 8. Hospital for Sick Children, Toronto, ON. 9. University of California San Francisco, San Francisco. 10. Children's Hospital of Pittsburgh, Pittsburgh. 11. Cedars-Sinai Medical Center, Los Angeles, CA. 12. Children's Hospital of Philadelphia, Philadelphia, PA. 13. Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN. 14. Montreal Children's Hospital, McGill University, Montreal, QC, Canada. 15. School of Women's and Children's Health, Medicine, University of New South Wales and Sydney Children's Hospital Randwick, Sydney, NSW, Australia. 16. University of Utah, Salt Lake City, UT. 17. Massachusetts General Hospital for Children, Harvard Medical School, Boston, MA. 18. Medical College of Wisconsin, Milwaukee, WI. 19. Hadassah Hebrew University Hospital, Jerusalem, Israel. 20. Department of Biostatistics. 21. Washington University School of Medicine, St Louis, MO. 22. Department of Pediatrics, Stead Family Children's Hospital, University of Iowa, Iowa City, IA.
Abstract
INTRODUCTION: The significance of pancreas divisum (PD) as a risk factor for pancreatitis is controversial. We analyzed the characteristics of children with PD associated with acute recurrent or chronic pancreatitis to better understand its impact. PATIENTS AND METHODS: We compared children with or without PD in the well-phenotyped INSPPIRE (INternational Study group of Pediatric Pancreatitis: In search for a cuRE) cohort. Differences were analyzed using 2-sample t test or Wilcoxon rank sum test for continuous variables, Pearson χ or Fisher exact test for categorical variables. RESULTS: PD was found in 52 of 359 (14.5%) subjects, a higher prevalence than the general population (∼7%). Females more commonly had PD (71% vs. 55%; P=0.02). Children with PD did not have a higher incidence of mutations in SPINK1, CFTR, CTRC compared with children with no PD. Children with PD were less likely to have PRSS1 mutations (10% vs. 34%; P<0.01) or a family history of pancreatitis (P<0.05), and more likely to have hypertriglyceridemia (11% vs. 3%; P=0.03). Children with PD underwent significantly more endoscopic procedures and pancreatic sphincterotomy. Patients with PD had fewer attacks of acute pancreatitis (P=0.03) and were less likely to develop exocrine pancreatic insufficiency (P=0.01). Therapeutic endoscopic retrograde cholangiopancreatography was considered most helpful if pancreatic duct was impacted with stones (83% helpful). CONCLUSIONS: PD is likely a risk factor for acute recurrent pancreatitis and chronic pancreatitis in children that appears to act independently of genetic risk factors. Patients with PD and stones obstructing the pancreatic duct benefit most from therapeutic endoscopic retrograde cholangiopancreatography.
INTRODUCTION: The significance of pancreas divisum (PD) as a risk factor for pancreatitis is controversial. We analyzed the characteristics of children with PD associated with acute recurrent or chronic pancreatitis to better understand its impact. PATIENTS AND METHODS: We compared children with or without PD in the well-phenotyped INSPPIRE (INternational Study group of Pediatric Pancreatitis: In search for a cuRE) cohort. Differences were analyzed using 2-sample t test or Wilcoxon rank sum test for continuous variables, Pearson χ or Fisher exact test for categorical variables. RESULTS:PD was found in 52 of 359 (14.5%) subjects, a higher prevalence than the general population (∼7%). Females more commonly had PD (71% vs. 55%; P=0.02). Children with PD did not have a higher incidence of mutations in SPINK1, CFTR, CTRC compared with children with no PD. Children with PD were less likely to have PRSS1 mutations (10% vs. 34%; P<0.01) or a family history of pancreatitis (P<0.05), and more likely to have hypertriglyceridemia (11% vs. 3%; P=0.03). Children with PD underwent significantly more endoscopic procedures and pancreatic sphincterotomy. Patients with PD had fewer attacks of acute pancreatitis (P=0.03) and were less likely to develop exocrine pancreatic insufficiency (P=0.01). Therapeutic endoscopic retrograde cholangiopancreatography was considered most helpful if pancreatic duct was impacted with stones (83% helpful). CONCLUSIONS:PD is likely a risk factor for acute recurrent pancreatitis and chronic pancreatitis in children that appears to act independently of genetic risk factors. Patients with PD and stones obstructing the pancreatic duct benefit most from therapeutic endoscopic retrograde cholangiopancreatography.
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