Tag Archives: exocrine pancreatic insufficiency

Kids With Acute Pancreatitis Need Followup

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F Ahmed, M Abu-El-Haija. Gastroenterol 2025; 169: 572-584. Open Access! Acute Pancreatitis in Children: It’s Not Just a Simple Attack

This is a really good review of acute pancreatitis covering epidemiology, diagnosis, severity classification, management, microbiome/metabolite derangements, genetics, and complications. Most of these topics have been covered in numerous blog posts (see below).

Selected Key Points:

  • Diagnostic testing -Amylase/Lipase:  “The diagnostic efficacy of amylase for AP, in terms of sensitivity and specificity, is contingent on the selected threshold value. Elevating the cut-off point to 1000 IU/L results in a high specificity of approximately 95%. However, this comes at the cost of reduced sensitivity, which some studies report to be as low as 61%… the activity of serum lipase remains elevated for a longer duration, typically between 8 and 14 days,… Lipase demonstrates superior accuracy with most studies reporting specificities exceeding 95% and sensitivities ranging from 55%–100% at a threshold activity level of 600 IU/L…hese tests have excellent sensitivities, they may have a few limitations such as being poor predictors of severity”
  • Risk Factors in Children (from Figure 1):
  • Incidence and Severity in Children (from Figure 1):

[At a recent lecture, Jay Freeman (How to Upgrade Pancreas Care –Jay Freeman MD (Part 1)) noted that severe pancreatitis is often defined by degree of organ dysfunction (eg. cardiac, pulmonary, renal). A practical definition of severe pancreatitis in children is whether the patient requires admission to an ICU]

  • Diagnostic testing -Imaging: “Imaging techniques are crucial for diagnosing and managing AP in children…NASPGHAN) and the Society for Pediatric Radiology formed consensus guidelines where transabdominal ultrasonography was recommended as the primary imaging technique for pediatric cases with suspected AP…Recent studies in the pediatric population have indicated that US’s sensitivity for AP detection ranges from 47%–52%.25 …Magnetic resonance cholangiopancreatography (MRCP) is useful for anatomical assessment without radiation but may require sedation”
  • Management: “The cornerstones of therapy are early feeding and intravenous fluids… Allowing patients to eat on admission was feasible and was associated with lower length of stay. Rates of intravenous fluids are recommended at 1.5–2 times maintenance rates,49 and the preferred fluid is Lactated Ringer’s due to limited studies including a recent randomized controlled study that showed that Lactated Ringer’s was associated with a faster discharge rate when administered compared with normal saline.50“
  • Genetics:  “A recently conducted study investigated the importance of genetics in pediatric AP patients…use of an extensive panel of 8 genes… PRSS1, CFTR, SPINK1, CPA1, CTRC, CLDN2, CASR, and SBDS… genetics is a major component in all types of pancreatitis in children, with genetic variants being most prevalent in CP cases at 31%, followed by AP at 19%, and ARP at 6%. A key discovery was that variants in SPINK1, CFTR, or PRSS1 genes were associated with faster progression from first episode of AP toward CP.53“
  • Complications (from Figure 1): “After the first episode of AP, the QoL is decreased, and it may lead to other disorders such as exocrine dysfunction, endocrine dysfunction and diabetes, nutritional deficiencies, and acute recurrent pancreatitis and CP.”

My take: Even after a single episode of acute pancreatitis, there are risks for long-term complications and patients need to follow-up.

Related blog posts:

Measurement of Exocrine Pancreatic Insufficiency in IBD and the Real-World

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J Fernandez et al. JPGN 2023; 76: 475-479. Prevalence of Exocrine Pancreatic Dysfunction Based on Direct Function Testing in Pediatric Inflammatory Bowel Disease

Methods: Direct stimulated endoscopic pancreatic function test (ePFT) was performed in 74 children with IBD

Key findings:

  • 42 (56.7%) children had either generalized or partial exocrine pancreatic insufficiency (EPI). 
  • Weight z scores were significantly lower in those with abnormal ePFT (Crohn cases: P = 0.008; UC cases: P = 0.046). 

In their discussion, the authors assert: “We can confidently recommend ePFT in established or new IBD patients who have stricturing and/or penetrating CD, weight loss, low weight Z-score, or qualify for the diagnosis of malnutrition.”

My take: In my real-world experience (~30 years), I have yet to have one patient presenting with IBD who needed pancreatic enzyme supplementation to reverse growth failure/malnutrition. As a consequence, I have a difficult time accepting the premise that more than 50% have EPI. To me, this suggests that testing children when they are acutely-ill or malnourished is yielding unreliable results.

Related blog posts:

Tumamoc Hill, Tucson AZ

Endoscopic Pancreatic Function Testing -NASPGHAN Position Paper

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N Patel et al. JPGN 2021; 72: 144-150. full text Endoscopic Pancreatic Function Testing (ePFT) in Children: A Position Paper From the NASPGHAN Pancreas Committee

This articles serves as a good review of exocrine pancreatic insufficiency (EPI).

Etiologies:

  • “Cystic fibrosis is the most common cause of EPI in children .” Other congenital causes include  aberrant embryonic development of the pancreas, “Shwachman-Diamond syndrome, Johanson-Blizzard syndrome, Pearson marrow pancreas syndrome, and Jeune syndrome”
  • “Acquired causes of EPI can be transient, such as in the aftermath of acute pancreatitis (which can persist weeks to months)”
  • Also, infants, compared to adults, have “physiological” EPI. Lipase output is 5–10% of adult values during the 1st 6 months of life.

Advantages/Disadvantages of Endoscopic Testing for EPI:

  • Advantages:
    • • Safe, technically easy, and quick procedure to perform in conjunction to routine investigative EGD
    • • Allows assessment of acinar and ductal function
    • • High sensitivity and specificity in detection of isolated and generalized enzyme deficiencies
    • • Can diagnose minor and more severe degrees of EPI and aid in early diagnosis of CP in patients with unremarkable radiological changes
  • Disadvantages:
    • • Can be done only in conjunction with EGD and the patient will likely require sedation• Prolongs routine EGD
    • • Assesses peak enzyme activity and bicarbonate concentrations rather than total secretory capacity
    • • No standardized pancreatic fluid collection frequency or duration in pediatrics
    • • Lack of age-specific standardized reference ranges in pediatrics

Endoscopic Testing Caveats:

  • Any sample with a pH less than 7 may be unreliable as it is below the pH optimum of the enzymes and may reflect contamination with gastric fluid; however, ” the inability to increase pH, or bicarbonate, upon secretin stimulation may be reflective of loss of cystic fibrosis transmembrane conductance regulator (CFTR) function”
  • Samples need to be frozen or placed on dry ice
  • “Commonly used laboratory (Kaleida Health Children’s Hospital Laboratory, Buffalo, NY) are: trypsin >55.4 nmol · mL−1 · minute−1, amylase >32 μmol · mL−1 · minute−1, lipase >146 μmol · mL−1 · minute−1, and chymotrypsin >2.5 μmol · mL−1 · minute−1“

My take: With careful clinical judgement, endoscopic EPI testing is rarely needed. First of all, fecal elastase measurements can detect most patients with EPI. In addition, a lot of patients with poor growth and suspected malabsorption are too young for reliable endoscopic EPI testing.

Related blog posts: