Pancreatitis is an emerging problem in pediatrics with significant social and economic burden to society. Most children with acute pancreatitis recover uneventfully, but a fraction of patients develop recurrent episodes of acute pancreatitis (acute recurrent pancreatitis or ARP) or progress to chronic pancreatitis (CP). There are currently no effective treatments to stop the recurrent episodes of pancreatitis or prevent its progression to CP. A few pediatric studies were performed as retrospective chart reviews at tertiary care centers and on a small number of patients. Without prospective studies focusing on a well-phenotyped cohort of patients with pediatric pancreatitis, it is impossible to draw conclusions on the epidemiology, etiologies, pathogenesis, natural history and outcome of these disorders or to design diagnostic algorithms and therapeutic alternatives. To meet the need for the careful collection of data and a registry of well-phenotyped patients for clinical studies, we formed a pediatric pancreatitis interest group (INSPPIRE: International Study Group of Pediatric Pancreatitis: In search for a cure). Our immediate goal is to develop a database of children with ARP and CP and to understand the epidemiology, etiologies, natural history and outcome in a well-phenotyped cohort of children with ARP and CP. In this proposal, we propose to determine the feasibility of creating a collaborative, national network of pediatric centers to engage in prospective studies and analyses of children with ARP and CP. Our initial project is to define pediatric ARP and CP, understand its epidemiology and build a patient registry and sample repository. We have the following specific aims: (1) Develop a multi-center consortium to prospectively study pediatric ARP and CP and determine the feasibility of long-term studies;(2) Determine the epidemiology and potential etiologic factors of ARP and CP in children. In the first aim, we will solidify the administrative and scientific structure of the INSPPIRE Consortium and create an electronic database to accurately collect and share the clinical data and biological samples from children with ARP and CP from 14 participating sites. In the second aim, we will analyze the epidemiology, etiologies, diagnostic approaches, complications, outcome, and surgical therapies of children with ARP and CP. The objective of this application is to develop a database of children with ARP and CP, build a patient registry and sample repository and determine the feasibility of long-term studies. The constitution of our group and the proposal represents the first initiative to create a multi-center approach to systematically characterize pancreatitis in children. We expect that we will identify children with ARP and CP who have distinctive pathology and outcomes that will require unique, pathogenesis-directed therapies. These analyses will provide the rationale, feasibility and infrastructure for additional studies of patients with ARP and CP.
Pancreatitis is an emerging, but poorly understood condition in children that carries significant social and economic burden to society. The objective of this application is to develop a database of children with recurrent acute and chronic pancreatitis, build a patient registry and sample repository and determine the feasibility of long-term studies. Our long-term goal is to develop novel and effective therapies for children with recurrent acute and chronic pancreatitis.
|Uc, Aliye; Andersen, Dana K; Bellin, Melena D et al. (2016) Chronic Pancreatitis in the 21st Century - Research Challenges and Opportunities: Summary of a National Institute of Diabetes and Digestive and Kidney Diseases Workshop. Pancreas 45:1365-1375|
|Hegyi, PÃ©ter; Wilschanski, Michael; Muallem, Shmuel et al. (2016) CFTR: A New Horizon in the Pathomechanism and Treatment of Pancreatitis. Rev Physiol Biochem Pharmacol 170:37-66|
|Ting, Jie; Wilson, Leslie; Schwarzenberg, Sarah Jane et al. (2016) Direct Costs of Acute Recurrent and Chronic Pancreatitis in Children in the INSPPIRE Registry. J Pediatr Gastroenterol Nutr 62:443-9|
|Pohl, John; Morinville, Veronique; Husain, Sohail Z et al. (2016) Toxic-Metabolic Risk Factors Are Uncommon in Pediatric Chronic Pancreatitis. J Pediatr Gastroenterol Nutr 62:e66-7|
|Husain, Sohail Z; Morinville, Veronique; Pohl, John et al. (2016) Toxic-metabolic Risk Factors in Pediatric Pancreatitis: Recommendations for Diagnosis, Management, and Future Research. J Pediatr Gastroenterol Nutr 62:609-17|
|Uc, Aliye (2015) Death in Pediatric Intensive Care Unit: Not for Children With Acute Pancreatitis. J Pediatr Gastroenterol Nutr 61:1-2|
|Uc, Aliye; Olivier, Alicia K; Griffin, Michelle A et al. (2015) Glycaemic regulation and insulin secretion are abnormal in cystic fibrosis pigs despite sparing of islet cell mass. Clin Sci (Lond) 128:131-42|
|Schwarzenberg, Sarah Jane; Bellin, Melena; Husain, Sohail Z et al. (2015) Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden. J Pediatr 166:890-6.e1|
|Pohl, John F; Uc, Aliye (2015) Paediatric pancreatitis. Curr Opin Gastroenterol 31:380-6|
|Azhari, Hassan; Rahhal, Riad; Uc, Aliye (2015) Is Total Pancreatectomy with Islet Autotransplantation A Reasonable Choice for Pediatric Pancreatitis? JOP 16:335-41|
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