Objectives There is limited literature on acute pancreatitis (AP), acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) in children. The INSPPIRE (International Study group of Pediatric Pancreatitis: In search for a cure) consortium was formed to standardize definitions, develop diagnostic algorithms, investigate disease pathophysiology and design prospective multicenter studies in pediatric pancreatitis. Methods Subcommittees were formed to delineate definitions of pancreatitis and a survey was conducted to analyze current practice. Results Acute pancreatitis (AP) was defined as requiring 2 of: (1) abdominal pain compatible with AP, (2) serum amylase and/or lipase values ≥3 times upper limits of normal, (3) imaging findings of AP. ARP was defined as: ≥2 distinct episodes of AP with intervening return to baseline. CP was diagnosed in the presence of: (1) typical abdominal pain plus characteristic imaging findings or; (2) exocrine insufficiency plus imaging findings or; (3) endocrine insufficiency plus imaging findings. We found that children with pancreatitis were primarily managed by pediatric gastroenterologists. Unless the etiology was known, initial investigations included serum liver enzymes, triglycerides, calcium, and abdominal ultrasound. Further investigations (usually for ARP and CP) included magnetic resonance or other imaging, sweat chloride, and genetic testing. Respondents’ future goals for INSPPIRE included: (1) determining natural history of pancreatitis; (2) eveloping algorithms to evaluate and manage pancreatitis; and (3) validating diagnostic criteria. Conclusions INSPPIRE represents the first initiative to create a multicenter approach to systematically characterize pancreatitis in children. Future aims include creation of patient database and biologic sample repository.

A primary function of the pancreas is to produce digestive enzymes that are delivered to the small intestine for the hydrolysis of complex nutrients. Much of our understanding of digestive enzymes comes from studies in animals. New technologies and the availability of the sequence of the human genome allow for a critical review of older reports and assumptions based on animal studies. This report updates our understanding of human pancreatic digestive enzymes with a focus on new insights into the biology of human proteases, lipases and amylases.

Pediatric pancreatitis has received much attention during the past few years. Numerous reports have identified an increasing trend in the diagnosis of acute pancreatitis in children and key differences in disease presentation and management between infants and older children. The present review provides a brief, evidence-based focus on the latest progress in the clinical field. It also poses important questions for emerging multicenter registries to answer about the natural history and management of affected children with pancreatitis.

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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 INSPPIRE (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 Caucasian, 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 room visits and 2 hospitalizations in the last year. Forty-seven subjects (70%) missed one day of school in the past month due to chronic pancreatitis; 26 (34%) missed 3 or more days. Children reporting constant pain were more likely to miss school (p=0.002), visit emergency room (p=0.01) and experience hospitalizations (p=0.03) compared with children with episodic pain. Thirty-three children (43%) underwent therapeutic ERCP; 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.