Definitions of pediatric pancreatitis and survey of present clinical practices

Abstract

Objectives: There is limited literature on acute pancreatitis (AP), acute recurrent pancreatitis (ARP), and chronic pancreatitis (CP) in children. The International Study Group of Pediatric Pancreatitis: In Search for a Cure (INSPPIRE) 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 present practice.

Results: AP was defined as requiring 2 of the following: abdominal pain compatible with AP, serum amylase and/or lipase values ≥3 times upper limits of normal, and 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 typical abdominal pain plus characteristic imaging findings, or exocrine insufficiency plus imaging findings, or 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 determining natural history of pancreatitis, developing algorithms to evaluate and manage pancreatitis, and 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.

Figures

Figure 1. INSPPIRE Survey-Demographics

Respondents were asked to estimate the number of children they followed at their institution with (A) ARP, and (B) CP. Results show the number of children with ARP and CP that are seen by the respondents.

Figure 2. INSPPIRE Survey-Diagnostics

Respondents were presented with clinical case scenarios and asked to choose within a list of suggested investigations for: (A) single episode mild AP without known etiology or family history of pancreatitis; (B) ARP (2 episodes within 1 year) without known etiology or family history of pancreatitis; and (C) CP without known etiology or family history of pancreatitis or pancreatic cancer. LFT: liver function tests; TG: serum triglycerides; Ca: serum calcium; RUQ U/S: right upper quadrant ultrasound; CT: computerized tomography; MRI/MRCP: magnetic resonance imaging/magnetic resonance cholangiopancreatography; MRCP/S: magnetic resonance cholangiopancreatography with secretin; ERCP: endoscopic retrograde cholangiopancreatography; genetics: gene testing for PRSS1, SPINK1, CFTR mutations; VD U/S: vas deferens ultrasound; Sw Cl: sweat chloride.