Sarah Jane Schwarzenberg1, Melena Bellin1, Sohail Z Husain2, Monika Ahuja3, Bradley Barth4, Heather Davis3, Peter R Durie5, Douglas S Fishman6, Steven D Freedman7, Cheryl E Gariepy8, Matthew J Giefer9, Tanja Gonska5, Melvin B Heyman10, Ryan Himes6, Soma Kumar8, Veronique D Morinville11, Mark E Lowe2, Neil E Nuehring3, Chee Y Ooi12, John F Pohl13, David Troendle4, Steven L Werlin14, Michael Wilschanski15, Elizabeth Yen10, Aliye Uc3. 1. University of Minnesota Children's Hospital, Minneapolis, Minnesota, USA. 2. Children's Hospital of Pittsburgh, Pittsburgh, PA, USA. 3. University of Iowa Carver College of Medicine, Iowa City, IA, USA. 4. University of Texas Southwestern Medical School, Dallas, TX, USA. 5. Hospital for Sick Children, Toronto, ON, Canada. 6. Baylor College of Medicine, Houston, TX, USA. 7. Harvard Medical School, Boston, MA, USA. 8. Nationwide Children's Hospital, Columbus, OH, USA. 9. Seattle Children's Hospital, Seattle, WA, USA. 10. University of California at San Francisco, San Francisco, CA, USA. 11. Montreal Children's Hospital, McGill University, Montreal, QC, Canada. 12. Discipline of Paediatrics, School of Women's and Children's Health, Medicine, University of New South Wales and Sydney Children's Hospital Randwick Sydney, Australia. 13. University of Utah, Salt Lake City, UT, USA. 14. Medical College of Wisconsin, Milwaukee, WI, USA. 15. Hadassah Hebrew University Hospital, Jerusalem, Israel.
Abstract
OBJECTIVE: To determine the clinical presentation, diagnostic variables, risk factors, and disease burden in children with chronic pancreatitis. STUDY DESIGN: We performed a cross-sectional study of data from the International Study Group of Pediatric Pancreatitis: In Search for a Cure, a registry of children with acute recurrent pancreatitis and chronic pancreatitis. Between-group differences were compared using Wilcoxon rank-sum test. RESULTS: Among 170 subjects in the registry, 76 (45%) had chronic pancreatitis; 57% were female, 80% were white; median age at diagnosis was 9.9 years. Pancreatitis-predisposing genetic mutations were identified in 51 (67%) and obstructive risk factors in 25 (33%). Toxic/metabolic and autoimmune factors were uncommon. Imaging demonstrated ductal abnormalities and pancreatic atrophy more commonly than calcifications. Fifty-nine (77%) reported abdominal pain within the past year; pain was reported as constant and receiving narcotics in 28%. Children with chronic pancreatitis reported a median of 3 emergency department visits and 2 hospitalizations in the last year. Forty-seven subjects (70%) missed 1 day of school in the past month as the result of chronic pancreatitis; 26 (34%) missed 3 or more days. Children reporting constant pain were more likely to miss school (P = .002), visit the emergency department (P = .01), and experience hospitalizations (P = .03) compared with children with episodic pain. Thirty-three children (43%) underwent therapeutic endoscopic retrograde pancreatography; one or more pancreatic surgeries were performed in 30 (39%). CONCLUSIONS: Chronic pancreatitis occurs at a young age with distinct clinical features. Genetic and obstructive risk factors are common, and disease burden is substantial.
OBJECTIVE: To determine the clinical presentation, diagnostic variables, risk factors, and disease burden in children with chronic pancreatitis. STUDY DESIGN: We performed a cross-sectional study of data from the International Study Group of Pediatric Pancreatitis: In Search for a Cure, a registry of children with acute recurrent pancreatitis and chronic pancreatitis. Between-group differences were compared using Wilcoxon rank-sum test. RESULTS: Among 170 subjects in the registry, 76 (45%) had chronic pancreatitis; 57% were female, 80% were white; median age at diagnosis was 9.9 years. Pancreatitis-predisposing genetic mutations were identified in 51 (67%) and obstructive risk factors in 25 (33%). Toxic/metabolic and autoimmune factors were uncommon. Imaging demonstrated ductal abnormalities and pancreatic atrophy more commonly than calcifications. Fifty-nine (77%) reported abdominal pain within the past year; pain was reported as constant and receiving narcotics in 28%. Children with chronic pancreatitis reported a median of 3 emergency department visits and 2 hospitalizations in the last year. Forty-seven subjects (70%) missed 1 day of school in the past month as the result of chronic pancreatitis; 26 (34%) missed 3 or more days. Children reporting constant pain were more likely to miss school (P = .002), visit the emergency department (P = .01), and experience hospitalizations (P = .03) compared with children with episodic pain. Thirty-three children (43%) underwent therapeutic endoscopic retrograde pancreatography; one or more pancreatic surgeries were performed in 30 (39%). CONCLUSIONS:Chronic pancreatitis occurs at a young age with distinct clinical features. Genetic and obstructive risk factors are common, and disease burden is substantial.
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