The primary objectives of the INSPPIRE 2 study are (1) to comprehensively characterize the pediatric population with ARP and CP and (2) to determine predictors for early onset CP and its sequelae including disease burden, exocrine pancreatic insufficiency (EPI), and diabetes. INSPPIRE 2 will provide a longitudinal cohort of well-phenotyped patients and biological samples to achieve these objectives and for future research defining the pathogenesis and testing novel therapies.

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We hypothesize that childhood-onset ARP follows a severe disease course with rapid progression to CP and early development of complications including persistent abdominal pain, growth and nutritional disturbances, EPI, glycemic abnormalities, diabetes, repeated hospitalizations and procedures, all of which result in socioeconomic burden and impaired quality of life. We also hypothesize that specific risk factors predispose children to early progression from ARP to CP, and specific risk factors predispose children to CP sequelae and high disease burden. Furthermore, the prospective collection of biological samples from children with ARP and CP will provide a framework to develop biomarkers for early diagnosis of CP in children with ARP and identify disease predictors for its complications (i.e. chronic abdominal pain, growth and nutritional disturbances, EPI, glycemic abnormalities, diabetes).