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

Pediatric Longitudinal Cohort Study of Chronic Pancreatitis (INSPPIRE 2)

This clinical trial collects blood, saliva, urine, or stool samples to help identify possible genetic mutations that may increase a person's chance at developing pancreatic cancer. Finding genetic markers among pediatric patients with acute recurrent pancreatitis and chronic pancreatitis may help identify patients who are at risk of pancreatic cancer.

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