Journal of Gastrointestinal & Digestive System
Open Access

Abstract

Chronic pancreatitis (CP), characterized by inflammation-induced continuous damage to the structure and function, or both, of the pancreas, is of rare occurrence in childhood. The etiology of CP in children varies and includes gene mutations, anatomic anomalies, metabolic disorders, and others. Hereditary pancreatitis (HP) is a rare cause of CP, first described by Comfort in 1952. HP is an autosomal dominant disorder with penetrance of approximately 80-90%. In 1996, association between HP and mutation (p.R122H) in the cationic trypsinogen gene (PRSS1) was described by Whitcomb et al., which was further confirmed by an independent study. HP develops mainly in childhood. The precise prevalence of HP in the general population is still unknown. The prevalence of HP patients in the CP group varies from 1% to 8%. Available data from adult patients do not reflect the natural history of HP in children. Although the incidence of HP in pediatric population is low, pancreatitis might cause significant morbidity and even mortality. Most of the information is found within individual case reports or small case studies. The Children�s Memorial Health Institute in Warsaw, a leading national pancreatic center, admits the majority of Polish children with CP. Our group of children with CP (over 300 patients) is one of the largest single-center cohorts in the world. Thus, investigations of clinical presentation and treatment in this group could deliver essential findings for medical practice.

Biography

Email: agoracz@wp.pl