Paeds · gastroenterology-hepatology-and-nutrition
Pancreatitis and pancreatic disorders
Also known as Pancreatitis · Acute pancreatitis · Chronic pancreatitis · Acute recurrent pancreatitis · Hereditary pancreatitis · Pancreatic exocrine insufficiency · Pancreatitis in children
Fellowship guide to pancreatitis and pancreatic disorders in children: the NASPGHAN classification and two-of-three diagnostic criteria for acute pancreatitis, the premature intracellular trypsin activation pathophysiology, the three paediatric risk-factor domains of toxic-metabolic, genetic and obstructive causes, the INSPPIRE definitions separating acute from acute recurrent and chronic pancreatitis, early aggressive hydration and the shift to early enteral feeding, the management of pain and complications, pancreatic exocrine insufficiency diagnosed by faecal elastase and treated with pancreatic enzyme replacement, hereditary PRSS1 pancreatitis and its cancer risk, autoimmune pancreatitis, traumatic pancreatitis, and total pancreatectomy with islet autotransplantation.
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Red flags
- A child on valproate or L-asparaginase who develops acute abdominal pain has pancreatitis until proven otherwise; check a serum lipase urgently and stop the offending drug
- A child with epigastric pain after a bicycle handlebar or blunt abdominal injury has traumatic pancreatitis with possible pancreatic duct injury until imaging proves otherwise
- A child presenting with diabetic ketoacidosis and epigastric pain may have concurrent pancreatitis that is masked by the metabolic derangement; a lipase should be sent
- Recurrent episodes of pancreatitis in a child demand a genetic workup for hereditary pancreatitis, because PRSS1 mutations carry a high lifetime risk of progression to chronic disease and pancreatic cancer
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- Pancreatitis and pancreatic disorders
- Pancreatitis and pancreatic disorders: the NASPGHAN and INSPPIRE classification, the genetic and toxic-metabolic risk factors, and the progression to chronic disease
- Acute pancreatitis in children: the two-of-three diagnostic criteria, early aggressive hydration, and the shift to early enteral feeding
- Short case: the child with recurrent pancreatitis and a family history
- Long case: the child with chronic pancreatitis, exocrine insufficiency and pancreatic enzyme replacement
- Pancreatitis: pathophysiology, diagnosis, and management in children
- Gastroenterology: the child with acute pancreatitis
- Gastroenterology: pancreatitis and exocrine pancreatic insufficiency in children
- Patient Care: pancreatitis and pancreatic disorders in children
- Medical Expert: pancreatitis and pancreatic disorders in children
Overview & Definition
The pancreas is a single gland that does two very different jobs, and pancreatitis in a child is the failure of one of them. The exocrine pancreas releases digestive enzymes into the gut, and the endocrine pancreas releases insulin into the blood. When the gland inflames, enzymes activate inside it instead of in the bowel lumen, the pancreas digests itself, and the child arrives in the emergency department with severe epigastric pain that drives everything that follows. [1]
Acute pancreatitis in a child is defined by the NASPGHAN criteria as the presence of at least two of three features: characteristic epigastric pain that may radiate to the back, a serum lipase or amylase at or above three times the upper limit of normal, and imaging findings consistent with pancreatic inflammation. The definition is deliberately practical, because the child does not always have all three at the first presentation, and the clinician who waits for the complete triad will miss the early window for treatment. [1]
The paediatric disease differs from the adult disease in almost every way that matters. The causes are different — children do not typically have gallstones or alcohol, and the dominant paediatric causes are medications, trauma and genetic variants. The severity tends to be lower, the recovery faster, and the stakes are in the long tail: a child who has one episode may have a genetic predisposition that drives recurrence and the eventual slide into chronic disease with exocrine and endocrine failure. The modern approach is therefore not just to treat the acute attack but to hunt the underlying cause, because finding a PRSS1 mutation or stopping valproate changes the child's whole trajectory. [5] [1]
References12ShowHide
- [1]Abu-El-Haija M; Kumar S; Szabo F; Jażdżewska M; Ranganathan S; Werlin SL Classification of Acute Pancreatitis in the Pediatric Population: Clinical Report From the NASPGHAN Pancreas Committee. J Pediatr Gastroenterol Nutr, 2017.PMID 28333771
- [2]Abu-El-Haija M; Kumar S; Quiros JA; Balzer B; Durie PR; Elinoff B Management of Acute Pancreatitis in the Pediatric Population: A Clinical Report From the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition Pancreas Committee. J Pediatr Gastroenterol Nutr, 2018.PMID 29280782
- [3]Párniczky A; Abu-El-Haija M; Husain S; Lowe M; Amir L; Brodzicki J EPC/HPSG evidence-based guidelines for the management of pediatric pancreatitis. Pancreatology, 2018.PMID 29398347
- [4]Taylor CJ; Chen K; Horvath K; Hughes J; Rothbaum R; Shun-Shin M ESPGHAN and NASPGHAN Report on the Assessment of Exocrine Pancreatic Function and Pancreatitis in Children. J Pediatr Gastroenterol Nutr, 2015.PMID 25915425
- [5]Kumar S; Ooi CY; Werlin S; Abu-El-Haija M; Barth B; Bellin MD Risk Factors Associated With Pediatric Acute Recurrent and Chronic Pancreatitis: Lessons From INSPPIRE. JAMA Pediatr, 2016.PMID 27064572
- [6]Uc A; Cress GA; Wang F; Abou-Resh N; Barth BA; Bellin MD Analysis of INSPPIRE-2 Cohort: Risk Factors and Disease Burden in Children With Acute Recurrent or Chronic Pancreatitis. J Pediatr Gastroenterol Nutr, 2022.PMID 35976273
- [7]Abu-El-Haija M; Uc A; Werlin SL; Freeman AJ; Geiger A; Homme J 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.PMID 29927872
- [8]Scheers I; Palermo JJ; Freedman S; Wilschanski M; Smith G; Ali MF Recommendations for Diagnosis and Management of Autoimmune Pancreatitis in Childhood: Consensus From INSPPIRE. J Pediatr Gastroenterol Nutr, 2018.PMID 29746340
- [9]Husain SZ; Morinville V; Pohl J; Rabinowitz S; Arsenescu R; Barth BA Toxic-metabolic Risk Factors in Pediatric Pancreatitis: Recommendations for Diagnosis, Management, and Future Research. J Pediatr Gastroenterol Nutr, 2016.PMID 26594832
- [10]Troendle DM; Fishman DS; Barth BA; Giefer MJ; Lin TK; Palermo JJ Therapeutic Endoscopic Retrograde Cholangiopancreatography in Pediatric Patients With Acute Recurrent and Chronic Pancreatitis: Data From the INSPPIRE Study. Pancreas, 2017.PMID 28609364
- [11]Dike CR; Zimmerman B; Zheng Y; Lin TK; Hornung L; Bisgaard SG Clinical and Practice Variations in Pediatric Acute Recurrent or Chronic Pancreatitis: Report From the INSPPIRE Study. J Pediatr Gastroenterol Nutr, 2020.PMID 32079978
- [12]Heinzman C; Hornung L; Lin TK; Stevens J; Spence AB; Bunch K Total pancreatectomy with islet autotransplantation reduces opioid use and improves nutritional support in children with debilitating pancreatitis. PLoS One, 2023.PMID 37540665