Quin Y Liu1, Maisam Abu-El-Haija2, Sohail Z Husain3, Bradley Barth4, Melena Bellin5, Douglas S Fishman6, Steven D Freedman7, Cheryl E Gariepy8, Matthew J Giefer9, Tanja Gonska10, Melvin B Heyman11, Ryan Himes6, Tom K Lin2, Asim Maqbool12, Maria Mascarenhas12, Brian A McFerron13, Veronique D Morinville14, Jaimie D Nathan2, Chee Y Ooi15, Emily R Perito11, John F Pohl16, Sue Rhee11, Sarah J Schwarzenberg6, Uzma Shah17, David Troendle4, Steven L Werlin18, Michael Wilschanski19, M Bridget Zimmerman20, Mark E Lowe21, Aliye Uc22. 1. Cedars-Sinai Medical Center, Los Angeles, CA. 2. Department of Pediatrics, College of Medicine, University of Cincinnati, Cincinnati, OH. 3. Department of Pediatrics, Stanford University, Palo Alto, CA. 4. University of Texas Southwestern Medical School, Dallas, TX. 5. University of Minnesota Masonic Children's Hospital, Minneapolis, MN. 6. Baylor College of Medicine, Houston, TX. 7. Harvard Medical School, Boston, MA. 8. Nationwide Children's Hospital, Columbus, OH. 9. Seattle Children's Hospital, Seattle, WA. 10. Hospital for Sick Children, Toronto, ON, Canada. 11. University of California San Francisco, San Francisco, 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, 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, University of Iowa, Iowa City, IA. 21. Washington University School of Medicine, St Louis, MO. 22. University of Iowa Stead Family Children's Hospital, Iowa City, IA.
Abstract
OBJECTIVE: The aim of the study was to determine the rate of progression from acute recurrent pancreatitis (ARP) to chronic pancreatitis (CP) in children and assess risk factors. STUDY DESIGN: Data were collected from the INternational Study group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE) cohort. Kaplan-Meier curves were constructed to calculate duration of progression from initial attack of acute pancreatitis (AP) to CP. Log-rank test was used to compare survival (nonprogression) probability distribution between groups. Cox proportional hazard regression models were fitted to obtain hazard ratio (with 95% confidence interval [CI]) of progression for each risk variable. RESULTS: Of 442 children, 251 had ARP and 191 had CP. The median time of progression from initial attack of AP to CP was 3.79 years. The progression was faster in those ages 6 years or older at the first episode of AP compared to those younger than 6 years (median time to CP: 2.91 vs 4.92 years; P = 0.01). Children with pathogenic PRSS1 variants progressed more rapidly to CP compared to children without PRSS1 variants (median time to CP: 2.52 vs 4.48 years; P = 0.003). Within 6 years after the initial AP attack, cumulative proportion with exocrine pancreatic insufficiency was 18.0% (95% CI: 12.4%, 25.6%); diabetes mellitus was 7.7% (95% CI: 4.2%, 14.1%). CONCLUSIONS: Children with ARP rapidly progress to CP, exocrine pancreatic insufficiency, and diabetes. The progression to CP is faster in children who were 6 years or older at the first episode of AP or with pathogenic PRSS1 variants. The factors that affect the aggressive disease course in childhood warrant further investigation.
OBJECTIVE: The aim of the study was to determine the rate of progression from acute recurrent pancreatitis (ARP) to chronic pancreatitis (CP) in children and assess risk factors. STUDY DESIGN: Data were collected from the INternational Study group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE) cohort. Kaplan-Meier curves were constructed to calculate duration of progression from initial attack of acute pancreatitis (AP) to CP. Log-rank test was used to compare survival (nonprogression) probability distribution between groups. Cox proportional hazard regression models were fitted to obtain hazard ratio (with 95% confidence interval [CI]) of progression for each risk variable. RESULTS: Of 442 children, 251 had ARP and 191 had CP. The median time of progression from initial attack of AP to CP was 3.79 years. The progression was faster in those ages 6 years or older at the first episode of AP compared to those younger than 6 years (median time to CP: 2.91 vs 4.92 years; P = 0.01). Children with pathogenic PRSS1 variants progressed more rapidly to CP compared to children without PRSS1 variants (median time to CP: 2.52 vs 4.48 years; P = 0.003). Within 6 years after the initial AP attack, cumulative proportion with exocrine pancreatic insufficiency was 18.0% (95% CI: 12.4%, 25.6%); diabetes mellitus was 7.7% (95% CI: 4.2%, 14.1%). CONCLUSIONS:Children with ARP rapidly progress to CP, exocrine pancreatic insufficiency, and diabetes. The progression to CP is faster in children who were 6 years or older at the first episode of AP or with pathogenic PRSS1 variants. The factors that affect the aggressive disease course in childhood warrant further investigation.
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