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Long-term disease progression in pediatric acute recurrent and chronic pancreatitis: A report from INSPPIRE
Elissa M Downs1, Emily R Perito2, Fuchenchu Wang3
1University of Minnesota, United States.
Insights
Nearly 1 in 5 children with acute recurrent pancreatitis (ARP) progressed to chronic pancreatitis (CP). Risk factors for progression to CP include genetics and frequent acute pancreatitis episodes.
Area of Science:
- Pediatric Gastroenterology
- Pancreatology
Background:
- Childhood acute recurrent pancreatitis (ARP) can lead to chronic pancreatitis (CP).
- The INternational Study group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE) cohort offers insights into disease progression.
Purpose of the Study:
- To analyze the progression of ARP to CP in children.
- To identify risk factors and sequelae associated with pancreatitis progression in a pediatric cohort.
Main Methods:
- Categorized 626 children into persistent ARP (pARP), incident CP (iCP), or prevalent CP (pCP).
- Analyzed time-to-sequelae and risk factors for progression using prospectively collected data.
Main Results:
- 21.1% of ARP cases progressed to iCP.
- Incident CP cases were more likely to have PRSS1 mutations, obstructive factors, and more acute pancreatitis episodes.
- Exocrine pancreatic insufficiency (EPI) developed in 24% of children during follow-up; 32% of iCP cases developed EPI.
- Diabetes mellitus (DM) developed in 8% during follow-up.
Conclusions:
- Nearly one in five children with ARP progressed to CP.
- Genetics, obstructive disease, and high episode frequency are risk factors for progression.
- A subset of children developed irreversible sequelae such as EPI and DM.
Background:
Acute recurrent pancreatitis (ARP) in childhood can rapidly progress to chronic pancreatitis (CP). Prospectively-collected data from the INternational Study group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE) provides novel insight into disease progression.
Methods:
INSPPIRE subjects were categorized as persistent ARP (pARP = remained ARP through last follow-up), incident CP (iCP = ARP at enrollment, developed CP), or prevalent CP (pCP = CP at enrollment). Time-to-sequelae and risk factors were analyzed.
Results:
Of 626 total children, 384 (61%) were ARP at baseline; of these, 81 (21.1%) were iCP at follow-up. iCP were more likely to have PRSS1 mutations, obstructive risk factors, and more acute pancreatitis episodes (AP) vs. pARP, but didn't differ in age at first AP. Exocrine pancreatic insufficiency (EPI) developed in 24% during follow-up, 10% of pARP, 32% of iCP. In all CP, 50% had EPI by 17.7yrs, median 10yrs after first AP. Diabetes mellitus (DM) developed in 8% during follow-up, 6% of pARP, 5% of iCP. In all CP, median event-free survival from birth and after first AP was not reached.
Conclusions:
Prospective follow-up of children with ARP revealed nearly 1 in 5 progressed to CP, with a subset developing irreversible sequelae, and highlighted risk factors associated with progression including genetics, obstructive disease, and episode frequency.
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