Liu, Quin Y.Abu-El-Haija, MaisamHusain, Sohail Z.Barth, BradleyBellin, MelenaFishman, Douglas S.Freedman, Steven D.Gariepy, Cheryl E.Giefer, Matthew J.Gonska, TanjaHeyman, Melvin B.Himes, RyanLin, Tom K.Maqbool, AsimMascarenhas, MariaMcFerron, Brian A.Morinville, Veronique D.Nathan, Jaimie D.Ooi, Chee Y.Perito, Emily R.Pohl, John F.Rhee, SueSchwarzenberg, Sarah J.Shah, UzmaTroendle, DavidWerlin, Steven L.Wilschanski, MichaelZimmerman, M. BridgetLowe, Mark E.Uc, Aliye2021-04-292021-04-292019-08-01Liu, Q. Y., Abu-El-Haija, M., Husain, S. Z., Barth, B., Bellin, M., Fishman, D. S., Freedman, S. D., Gariepy, C. E., Giefer, M. J., Gonska, T., Heyman, M. B., Himes, R., Lin, T. K., Maqbool, A., Mascarenhas, M., McFerron, B. A., Morinville, V. D., Nathan, J. D., Ooi, C. Y., … Uc, A. (2019). Risk Factors for Rapid Progression From Acute Recurrent to Chronic Pancreatitis in Children: Report From INSPPIRE. Journal of Pediatric Gastroenterology and Nutrition, 69(2), 206–211. https://doi.org/10.1097/MPG.00000000000024050277-2116https://hdl.handle.net/1805/25808Objective 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 (non-progression) probability distribution between groups. Cox proportional hazard regression models were fitted to obtain hazard ratio (with 95% CI) of progression for each risk variable. Results Of 442 children, 251 had ARP, 191 CP. The median time of progression from initial attack of AP to CP was 3.79 years. The progression was faster in those age ≥6 years at the first episode of AP compared to those age <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 six years after the initial AP attack, cumulative proportion with exocrine pancreatic insufficiency (EPI) 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, EPI and diabetes. The progression to CP is faster in children who were ≥6 years at the first episode of AP or with pathogenic PRSS1 variants. The factors that impact the aggressive disease course in childhood warrant further investigation.en-USPRSS1pediatric pancreatitisdiabetes mellituspancreatic insufficiencynatural historyArticle