Chronic Pancreatitis, Function Tests, & Pain Management: Pancreatic Disease
With Dr. Joe Palermo & Dr. Andrew Trout & Dr. Doctor Goldschneider · hosted by Dr. em gootee · StayCurrentMD
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
Patients can have acute recurrent pancreatitis without evidence of ductal changes, pancreatic atrophy, or pancreatic function loss.
In the pediatric cohort, the main risk factors for chronic pancreatitis were genetic, followed by obstructive or anatomic processes.
In a local cohort of 50 patients (34 ARP, 16 CP), 55% of ARP patients and 68% of CP patients tested positive for mutations in CFTR, PRSS1, CTRC, or SPINK1.
Chronic pancreatitis patients were diagnosed earlier with the first attack, had more additional attacks, and were more likely to be exocrine insufficient compared to ARP patients.
CFTR gene was more commonly found in chronic pancreatitis patients compared to acute recurrent patients, and patients were more likely to have two genes affected than one.
A next-generation sequencing pancreas panel covering 10 genes will launch in October at Cincinnati Children's, offering efficient, low-cost testing with faster turnaround than whole exome sequencing.
Heterozygous CFTR (one gene copy) increases the risk for chronic pancreatitis in children.
Combination of SPINK1 and CFTR mutations confers an approximately 800-fold relative risk of chronic pancreatitis.
The CF Foundation has an extensive pipeline of small molecules optimizing CFTR function, which may offer therapeutic options for pancreatitis patients with CFTR mutations.
Some patients with pancreas divisum do not develop pancreatitis, suggesting a two-hit or multi-hit hypothesis where additional risk factors (e.g., genetic mutations) increase disease propensity.
Direct pancreatic function testing means directly obtaining pancreatic function from the pancreas, not relying on stool testing like fecal elastase.
The endoscopic pancreatic function test protocol uses secretin (0.2 mcg/kg) or CCK (0.4 mcg/kg) at time zero, then collects duodenal aspirates via ERCP catheter every 5 minutes for three samples.
Samples are sent to Women's Children's Hospital lab to measure activities of trypsin, amylase, lipase, and chymotrypsin.
Pancreatic function can be abnormal if checked around an acute attack.
Pancreatic enzyme maturation occurs over the first 2 to 3 years of life.
MR pancreatic function testing (MRPFT) acquires identical fluid-sensitive imaging pre- and post-secretin, then quantifies the volume of fluid secreted into the gastrointestinal tract.
In the MRPFT protocol, there is about a 15-minute delay between pre- and post-secretin imaging to allow fluid accumulation.
MRPFT can threshold images and quantify secreted fluid volume by subtracting pre-secretin fluid from post-secretin fluid.
Correlation between MRPFT volumetric analysis and endoscopic pancreatic function testing is under study in about 35 patients.
Qualitative assessment of exocrine function by MRPFT may differ between pediatric and adult patients, possibly due to size or weight dependency.
In chronic pancreatitis patients with dilated and abnormal ducts at baseline, secretin may not add diagnostic value for duct visualization, though it is used primarily for exocrine assessment.
Pancreatic enzyme replacement may improve bloating and other GI symptoms in patients with exocrine insufficiency, but does not prevent attacks.
The multidisciplinary pain team comprises a pain physician (medical/psychosocial assessment, medication, interventional procedures), a psychologist (cognitive behavioral therapy, school reintegration, family intervention), and a nurse.
Psychology involvement is 100% of the time in the pain clinic, not optional, to address pain coping, functional and emotional impact, and school reintegration.
Opioids are a tool requiring risk assessment, mitigation, informed consent (mandated by Ohio law for chronic use in minors), controlled substance agreements, prescription history tracking, and use of the lowest effective dose.
In the case of a 13-year-old with annular pancreas and chronic pancreatitis, multidisciplinary pain management (topiramate for migraines, low-dose methadone for 2 years, psychology, family intervention) resulted in pain resolution, return to school and activities, and indefinite deferral of Whipple surgery.
Between 7 and 34% of pediatric patients with acute pancreatitis will eventually develop recurrent episodes.
Acute recurrent pancreatitis is defined as two distinct episodes of acute pancreatitis with either complete resolution between episodes or complete normalization of enzymes between episodes.
Chronic pancreatitis requires a combination of clinical presentation and abnormal imaging findings: ductal irregularities, calcifications, or pancreatic gland atrophy, seen on CT, MRCP, or ERCP.
Chronic pancreatitis diagnosis also requires abdominal pain consistent with pancreatic origin or evidence of endocrine or exocrine pancreatic insufficiency.
In adults, up to 70% of chronic pancreatitis cases are caused by alcohol.
PRSS1 (cationic trypsinogen gene) is a gain-of-function gene causing hereditary pancreatitis, autosomal dominant, with 80% penetrance for acute pancreatitis, 50% for chronic pancreatitis, and more than 40% risk of pancreatic cancer.
SPINK1 (trypsin inhibitor gene) is a modifier gene, autosomal recessive, but heterozygous patients can also develop disease.
Severe CFTR mutations (e.g., delta 508) cause exocrine pancreatic insufficiency; patients who are pancreatic sufficient with CF mutations are at risk for pancreatitis and chronic pancreatitis.
CTRC gene has been shown through functional studies to be linked to development of chronic pancreatitis and acute recurrent pancreatitis.
Six additional genes beyond the original four (PRSS1, SPINK1, CFTR, CTRC) are now known to be associated with ARP and CP.
PRSS1 penetrance is incomplete: half of patients with the gene develop chronic pancreatitis, not all.
Low-fat diet, pancreatic enzyme replacement, antioxidants, and steroids have never been shown to prevent recurrent pancreatitis episodes or pancreatic pain episodes.
Cognitive behavioral therapy is the standard, state-of-the-art therapy for pain management.