Logo for University of Iowa Health Care This logo represents the University of Iowa Health Care

About Our Research

Aliye UcINSPPIRED to find a cure for pediatric pancreatitis

Aliye Uc, is a professor of pediatrics, pediatric gastroenterologist, the Director of Pediatric Gastroenterology, Hepatology, Pancreatology, and Nutrition Division, and the lead Principle Investigator of the INternational Study Group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE) project. The INSPPIRE group was formed in 2009 and consists of pediatric gastroenterologists, radiologists, endocrinologists. In 2012, INSPPIRE was funded by a R21 grant from the National Institutes of Health (NIH). INSPPIRE is the first multinational, multi-center effort to systematically characterize pediatric pancreatitis.

The pancreas performs important functions by aiding in digestion and helping to regulate blood sugar. Pediatric pancreatitis is considered rare among children. It presents with swelling or irritation or so-called inflammation of the pancreas. In most cases, inflammation is acute (acute pancreatitis or AP), rarely severe and children improve without sequela. In some (15-35%), pancreatitis attacks recur (acute recurrent pancreatitis or ARP) and some progress to chronic pancreatitis (CP), manifested by scarring of the pancreas, causing pain. Over time, children with CP may suffer from failure of pancreas digesting food, thus requiring to take pancreatic enzymes by mouth or regulating blood sugar, needing insulin injections. Pancreatitis causes abdominal pain, nausea, and vomiting. Children with pancreatitis often miss school, they are hospitalized, have frequent emergency room visits, and require pain management.

Recent studies, have shown that the number children who experience pancreatitis has increased, and children now contract the disease almost as often as adults (approximately 1 in 10,000). The increase may be due to a more accurate diagnosis and testing of this rare disease, according to some studies. Preliminary data from INSPPIRE study show that pediatric pancreatitis differs from adult pancreatitis, “So it is imperative that we, as pediatricians, do not continue to draw our information from the adult literature,” said Dr. Aliye Uc.

The INSPPIRE team has enrolled over 560 children with acute recurrent and chronic pancreatitis from 20 sites (including centers in Australia, Canada, and Israel). These subjects have completed up to 4 annual follow-ups with subjects located throughout the consortium. From this data, the INSPPIRE investigators developed operative definitions of pediatric AP, ARP, and CP; collected over 200 bio-specimens from subjects, and assessed current clinical practice leading to the development of standards of care. The data revealed a substantial disease burden and high frequency of gene mutations in children with ARP (48%), and particularly those with CP (73%). 

To date, the INSPPIRE investigators have published more than 20 manuscripts and written 5 book chapters about pediatric pancreatitis. Data from INSPPIRE has been presented at 10 lectures and presented on more than 10 posters at NASPHAN, DDW, ESPGHAN, APA, WCPGHAN, and other conferences. Members of the INSPPIRE team have conducted more than 25 lectures at national and international conferences and symposiums. In November, 2017, a Single Topic Symposium titled “Frontiers in Pediatric Pancreatology” was held at the annual NASPGHAN meeting. Among the 14 lectures, 9 were presented by members of the INSPPIRE team.

In 2015, the INSPPIRE team received a 5 year, $2.1M grant from the National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases. This grant supports the INSPPIRE 2 project which is part of the Consortium for the Study of Chronic Pancreatitis, Diabetes, and Pancreatic Cancer (CPDPC) in the pediatric and adult population.  The CPDPC consortium was formed to develop a well-characterized cohort of both adult and pediatric patients and bio-specimens. INSPPIRE 2 will continue to collect longitudinal data to determine the natural history of pediatric chronic pancreatitis and identify risk factors and genetic modifiers for its onset and sequelae. From this data, our goal is to develop approaches to improve clinical outcomes. The INSPPIRE 2 protocol plans to enroll 860 children at 22 sites (18 US and 4 international) by August, 2020.