#140: Acute Recurrent & Chronic Pancreatitis – When Belly Pain Persists

April 30, 2025 | By Sam Masur

Audio

Summary:

Join us for part 2 of our informative discussion with Dr. David Vitale, a pediatric pancreatologist at Cincinnati Children’s Hospital. In this episode, we dive deep into acute recurrent and chronic pancreatitis, distinguishing the two, and exploring the causes, genetic predispositions, and available treatments. Whether you’re a budding pancreatologist or a PCP, this episode offers valuable insights into managing and treating this challenging condition.

Pediatric Chronic Pancreatitis Pearls

1. Acute recurrent pancreatitis (ARP) is defined by at least two distinct episodes of acute pancreatitis with symptom resolution between episodes, while chronic pancreatitis (CP) involves ongoing pancreatic damage, often due to fibrosis, leading to both exocrine and endocrine insufficiencies.
2. Common causes of ARP and CP include biliary obstructions, genetic mutations (e.g., CFTR, SPINK1), hyperlipidemia, autoimmune conditions, and drug-induced pancreatitis.
3. A thorough work-up includes evaluating symptoms, family history, genetic testing, pancreatic enzyme levels, and imaging studies like MRCP. Monitoring for complications like exocrine and endocrine insufficiencies is essential.
4. Treatment focuses on pain management, addressing specific etiologies, and managing complications like exocrine and endocrine insufficiency. Psychological support is critical due to the mental health burden associated with chronic pain.

Chronic Pancreatitis Show Notes

Definitions

Acute recurrent pancreatitis (ARP) is defined as at least two distinct episodes of acute pancreatitis with either complete resolution of pain between diagnoses (1+ month pain-free interval), or the complete normalization of serum pancreatic enzyme levels (amylase and lipase) along with complete resolution of pain symptoms, irrespective of time period. (1)

To define chronic pancreatitis (CP), a patient must have one of the three following criteria: 

1. Abdominal pain consistent with pancreatic origin and imaging findings showing pancreatic damage (ductal irregularities and parenchymal changes). 
2. Evidence of pancreatic endocrine insufficiency. 
3. Evidence of pancreatic exocrine insufficiency. (1)

Pathophysiology

Acute recurrent pancreatitis involves several discrete episodes of acute pancreatitis, which involves a premature and inappropriate activation of the pancreatic enzyme trypsinogen, leading to the destruction of pancreatic parenchyma. Chronic pancreatitis involves the replacement of normal pancreatic tissue with fibrotic tissue, leading to pancreatic atrophy. It is often preceded by recurrent episodes of acute pancreatitis. Progressive fibrosis leads to exocrine insufficiency (inability to produce digestive enzymes) and endocrine insufficiency (diabetes).

Etiologies

Common etiologies for ARP and CP include: 

• Obstructive/Biliary (found in 26% of children with ARP/CP; including gallstones, pancreas divisum, congenital biliary strictures, etc.) (2)
• Genetic mutations (approximately 65% of children with CP have genetic risk factors, most commonly CFTR, PRSS1, SPINK1, CTRC) (2)
• Drug-induced
• Hyperlipidemia (most commonly hypertriglyceridemia)
• Auto-immune

Work Up

For both diagnosis of ARP or CP, as well as pancreatic exocrine and endocrine insufficiency (complications of ARP/CP), utilize the following work up.

Treatment

Symptomatic Support:

Pain is by far the greatest burden on pediatric patients and accounts for loss of school, social time and development, and frequent hospitalizations. It is important to address pain to improve symptoms and quality of life. 

The ideal pain management regimen should be multi-modal and individualized, often including a combination of medical management (opioids or non-opioids medications), psychological support, and sometimes invasive interventions like nerve blocks or surgical options.

For patients with severe chronic pain and poor quality of life, total pancreatectomy with islet autotransplantation is an option, though it carries significant risks, including lifelong diabetes.

Etiology-Specific:

If an etiology of ARP/CP can be determined, it is important to address it directly to remove the inciting factor. For instance, in drug-induced pancreatitis, perform a thorough medication reconciliation and remove possible offenders. If there are biliary or obstructive pathologies, Dr. Vitale recommends ERCP (endoscopic retrograde cholangiopancreatogram) to relieve the stricture/obstruction. If there is an underlying systemic disease, such as cystic fibrosis or autoimmune pancreatitis, treat these conditions per each individualized disease.

Complications and Prognosis:

Exocrine insufficiency: Treat with pancreatic enzyme replacement therapy (PERT) as well as fat-soluble vitamin replacement (vitamins A, D, E and K). (The INSPIRE study suggests a possible slight decrease in ARP frequency in patients on fat-soluble vitamin supplementation.) Typical PERT dosing, per Dr. Vitale, is 500-1000 units of lipase per kilogram per meal. Dr. Vitale recommends patients take this at the start of the meal. It is also essential for patients to take PERT (typically at a reduced dose) even with snacks. 

Endocrine insufficiency: It is important to monitor for signs of impaired glucose tolerance or diabetes mellitus. An oral glucose tolerance is the best test for this, however you may opt to perform an A1c as an initial screening test. Treat insufficiency with insulin. 

Routine monitoring: Dr. Vitale recommends obtaining screening labs (vitamin levels, PT, INR, A1c, fecal elastase) every 6-12 months depending on the patient’s specific course. Perform imaging (ultrasound, MRCP) every 12-18 months or so, depending on the patient.

** Of note, the use of PERT does not interfere with fecal elastase testing, as PERT is porcine and the fecal elastase test measures human elastase. **

Social, academic and mental health repercussions: Patients with ARP or CP can be at higher risk for mental illness including depression and anxiety, often precipitated by severe and unpredictable abdominal pain. Their pain can be debilitating, which can lead to disruptions in academic, social and family activities. 

A study by Tham et al. highlighted that children with ARP and CP exhibit low physical health-related quality of life (HRQOL) and that emotional and behavioral problems are significantly associated with lower HRQOL scores. (3)[2] This underscores the importance of addressing both physical and psychosocial aspects of care in these patients.

Additionally, Thavamani et al. found that the prevalence of psychiatric disorders in hospitalized pediatric CP patients is substantial, with depression, substance abuse, and anxiety being the most common. The presence of these psychiatric disorders is associated with longer hospital stays and higher healthcare costs. (4)

Given these findings, it is crucial to incorporate psychological support and interventions, such as cognitive-behavioral therapy, into the management plan for pediatric patients with ARP and CP.

What if my patients family asks about the risk of pancreatic cancer? 

Dr. Vitale tells his families that the lifetime risk of developing pancreatic adenocarcinoma is 5 times that of the general population. (Consider a component of lead time bias in this estimation.) If you take that five-time risk, that child still has a much higher risk of developing other more common types of cancer, including colorectal or breast cancer.

For the PCP:

It is important for the primary care pediatrician to collaborate with the gastroenterologist in these medically complex, multi-faceted patients. As the typical ARP/CP patient meets with their pancreatologists once every 6-12 months, the PCP can be a more frequent touch-point to monitor signs and symptoms related to ARP/CP. 

Some specific opportunities for the PCP include:

• When the patient has symptoms above their baseline, obtain screening labs.
• Collaborate with multidisciplinary teams to screen for and address mental health concerns. 
• Work with schools and educational support programs to ensure the patient maximizes educational opportunities. 
• Ensure adequate growth curves (The NASPGHAN does not recommend any special types of diet in patients with relatively uncomplicated disease courses. The previous recommendation for a very low fat diet has been disproven amongst recent evidence. All pediatric patients with ARP or CP should receive a regular, well-balanced diet, with appropriate PERT and vitamin supplements.) (5)
• Counsel on tobacco avoidance (including second and third-hand smoke) and responsible alcohol use in folks above 21. 
• Ensure the patient is following with a Gastroenterologist with some regularity, specific to that particular patient’s case.

Citations

1. Morinville VD, Husain SZ, Bai H, et al. Definitions of pediatric pancreatitis and survey of present clinical practices [published correction appears in J Pediatr Gastroenterol Nutr. 2013 Apr;56(4):459. Abu-Al-Haija, Maisam [corrected to Abu-El-Haija, Maisam]]. J Pediatr Gastroenterol Nutr. 2012;55(3):261-265. doi:10.1097/MPG.0b013e31824f1516
2. Uc A, Cress GA, Wang F, et al. Analysis of INSPPIRE-2 Cohort: Risk Factors and Disease Burden in Children With Acute Recurrent or Chronic Pancreatitis. J Pediatr Gastroenterol Nutr. 2022;75(5):643-649. doi:10.1097/MPG.0000000000003590
3. Tham SW, Wang F, Gariepy CE, et al. Health-Related Quality of Life in Pediatric Acute Recurrent or Chronic Pancreatitis: Association With Biopsychosocial Risk Factors. J Pediatr Gastroenterol Nutr. 2022;74(5):636-642. doi:10.1097/MPG.0000000000003420
4. Thavamani A, Sterling N, Umapathi KK, Sankararaman S. Prevalence and impact of psychiatric disorders on hospitalized pediatric patients with chronic pancreatitis. Pancreatology. 2023;23(6):574-581. doi:10.1016/j.pan.2023.06.012
5. Abu-El-Haija M, Uc A, Werlin SL, et al. Nutritional Considerations in Pediatric Pancreatitis: A Position Paper from the NASPGHAN Pancreas Committee and ESPGHAN Cystic Fibrosis/Pancreas Working Group. J Pediatr Gastroenterol Nutr. 2018;67(1):131-143. doi:10.1097/MPG.0000000000002023

Goal

Listeners will explain the basic pathophysiology, diagnosis, and management of acute recurrent and chronic pancreatitis in pediatric patients.

Learning Objectives

After listening to this episode listeners will…  

1. Define acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP).
2. Explain the basic pathophysiology of ARP and CP.
3. Identify common etiologies of ARP and CP.
4. Summarize diagnostic approaches of ARP and CP. 
5. Outline basic treatment strategies for children with ARP and CP.

Disclosures

Dr Vitale reports no relevant financial disclosures. The Cribsiders report no relevant financial disclosures. 

Citation

Holloway R, Vitale D, Masur S, Berk J, Chiu C. “#140: When the Belly Pain Persists – Acute Recurrent and Chronic Pancreatitis”. The Cribsiders Pediatric Podcast. https:/www.thecribsiders.com/ April 30, 2025.