Exocrine Pancreatic Insufficiency

1. Samuel Bitton, MD* 2. Michael J Pettei, MD, PhD* 1. *Hofstra North Shore-LIJ School of Medicine/Steven and Alexandra Cohen Children's Medical Center of NY, Lake Success, NY. 1. 1. Taylor CJ, 2. Chen K, 3. Horvath K, 4. et al ESPGHAN and NASPGHAN Report on the Assessment of Exocrine Pancreatic Function and Pancreatitis in Children. Taylor CJ, Chen K, Horvath K, et al. J Pediatr Gastroenterol Nutr. 2015;61(1):144–153 [OpenUrl][1][PubMed][2] 2. 1. Lindkvist B Diagnosis and Treatment of Pancreatic Exocrine Insufficiency. Lindkvist B.World J Gastroenterol. 2013;19(42):7258–7266. [OpenUrl][3][CrossRef][4][PubMed][5] 3. 1. Fitzsimmons SC, 2. Burkhart GA, 3. Borowitz D, 4. et al High-dose Pancreatic-enzyme Supplements and Fibrosing Colonopathy in Children with Cystic Fibrosis. Fitzsimmons SC, Burkhart GA, Borowitz D, et al. N Engl J Med. 1997;336(18):1283–1289. [OpenUrl][6][CrossRef][7][PubMed][8][Web of Science][9] Exocrine pancreatic insufficiency (EPI) exists when pancreatic enzyme activity within the lumen of the small intestine is less than needed for normal digestion of nutrients. Although pancreatic enzymes are involved in protein, carbohydrate, and lipid digestion, enzyme insufficiency primarily affects lipid digestion. Steatorrhea, defined as excess fat in the stool, develops from an inability to absorb fat adequately, with resulting weight loss or failure to thrive, often accompanied by fat-soluble vitamin deficiencies. The pancreas is formed from the fusion of dorsal and ventral foregut outgrowths. The main pancreatic duct empties, along with the common bile duct, into the duodenum at the ampulla of Vater. Secretion of the pancreatic digestive enzymes is stimulated in response to luminal nutrients by the small intestinal peptide hormones cholecystokinin (CCK), produced from I-cells, and secretin, produced from S-cells. CCK stimulates the release of the pancreatic enzymes in inactive form from the pancreatic acinar cells into the pancreatic duct. Secretin stimulates the secretion of bicarbonate by the pancreatic ductal epithelium, which is largely mediated by the cystic fibrosis transmembrane conductance regulator (CFTR) channel protein. Bicarbonate is required to neutralize gastric acid entering the duodenum to maintain enzymatic activity. EPI results when the concentration of luminal lipase is insufficient to achieve lipid digestion, which does not occur until lipase is reduced to 5% to 10% of physiologic amounts. Conditions that commonly give rise to this situation usually involve destruction of the pancreatic parenchyma, as with cystic fibrosis (CF) and chronic pancreatitis, but EPI can also develop from nonpancreatic … [1]: {openurl}?query=rft.jtitle%253DJ%2BPediatr%2BGastroenterol%2BNutr%26rft.volume%253D61%26rft.spage%253D144%26rft_id%253Dinfo%253Apmid%252F25915425%26rft.genre%253Darticle%26rft_val_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Ajournal%26ctx_ver%253DZ39.88-2004%26url_ver%253DZ39.88-2004%26url_ctx_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Actx [2]: /lookup/external-ref?access_num=25915425&link_type=MED&atom=%2Fpedsinreview%2F37%2F2%2F85.atom [3]: {openurl}?query=rft.jtitle%253DWorld%2BJ%2BGastroenterol.%26rft.volume%253D19%26rft.spage%253D7258%26rft_id%253Dinfo%253Adoi%252F10.3748%252Fwjg.v19.i42.7258%26rft_id%253Dinfo%253Apmid%252F24259956%26rft.genre%253Darticle%26rft_val_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Ajournal%26ctx_ver%253DZ39.88-2004%26url_ver%253DZ39.88-2004%26url_ctx_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Actx [4]: /lookup/external-ref?access_num=10.3748/wjg.v19.i42.7258&link_type=DOI [5]: /lookup/external-ref?access_num=24259956&link_type=MED&atom=%2Fpedsinreview%2F37%2F2%2F85.atom [6]: {openurl}?query=rft.jtitle%253DNew%2BEngland%2BJournal%2Bof%2BMedicine%26rft.stitle%253DNEJM%26rft.aulast%253DFitzSimmons%26rft.auinit1%253DS.%2BC.%26rft.volume%253D336%26rft.issue%253D18%26rft.spage%253D1283%26rft.epage%253D1289%26rft.atitle%253DHigh-Dose%2BPancreatic-Enzyme%2BSupplements%2Band%2BFibrosing%2BColonopathy%2Bin%2BChildren%2Bwith%2BCystic%2BFibrosis%26rft_id%253Dinfo%253Adoi%252F10.1056%252FNEJM199705013361803%26rft_id%253Dinfo%253Apmid%252F9113931%26rft.genre%253Darticle%26rft_val_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Ajournal%26ctx_ver%253DZ39.88-2004%26url_ver%253DZ39.88-2004%26url_ctx_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Actx [7]: /lookup/external-ref?access_num=10.1056/NEJM199705013361803&link_type=DOI [8]: /lookup/external-ref?access_num=9113931&link_type=MED&atom=%2Fpedsinreview%2F37%2F2%2F85.atom [9]: /lookup/external-ref?access_num=A1997WW26000003&link_type=ISI