Biospecimen Collection to Identify Gene Mutations for High Risk Pancreatic Cancer in Pediatric Patients, INSPPIRE 2 Study

PRIMARY OBJECTIVE:

I. To comprehensively characterize the pediatric population with acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP) and determine predictors of early onset CP and its sequelae.

OUTLINE:

Patients complete quality-of-life (QoL) assessment and complete questionnaires for over 2 hours every 12 months for 4 years. Patients also undergo collection of blood and/or saliva (if blood samples are not available), urine, or stool at baseline or follow-up (if inadequate samples collected or missed at baseline).

After completion of the study, patients are followed up every 12 months.

Inclusion criteria

• All subjects/parents must sign an informed consent and/or assent indicating that they are aware of the investigational nature of this study
• Subjects/parents must have signed an authorization for the release of their or their child's protected health information
• All children must be under 18 years of age at the time of enrollment
• All children providing samples should fit the ARP or CP inclusion criteria defined below:

• Acute pancreatitis (AP): AP is defined as requiring 2 of the following:

• Abdominal pain compatible with AP
• Serum amylase and/or lipase values >= 3 times upper limits of normal
• Imaging findings of AP, such as gland enlargement, acute inflammatory changes, and fluid collections
• ARP is defined as: At least 2 episodes of acute pancreatitis with complete resolution of pain and a >= 1 month pain-free interval between episodes
• Chronic Pancreatitis:

• Children with at least:

• One irreversible structural change in the pancreas with or without abdominal pain +/- exocrine pancreatic insufficiency +/- diabetes

• Irreversible structural changes:

• Ductal calculi, dilated side branches, parenchymal calcifications found in any imaging (abdominal ultrasound [abd US], magnetic resonance imaging/magnetic resonance cholangiopancreatography [MRI/MRCP], computerized tomography [CT], endoscopic retrograde cholangiopancreatography [ERCP], endoscopic US [EUS])
• Ductal obstruction or stricture/dilatation/irregularities that are persistent (for >= 2 months) on any imaging
• Parenchymal atrophy, irregular contour, accentuated lobular architecture, cavities alone are not diagnostic findings for CP
• Surgical or pancreatic biopsy specimen demonstrating histopathologic features compatible with CP (acinar atrophy, fibrosis, protein plugs, infiltration with lymphocytes, plasma cells, macrophages)

Exclusion criteria

• Subjects must not have any significant medical illnesses that in the investigator's opinion cannot be adequately controlled with appropriate therapy or would compromise the subject's ability to tolerate study interventions