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Risk factors for chronic or acute recurrent pancreatitis in children

Risk factors for chronic or acute recurrent pancreatitis in children
Risk category Percent of patients with risk factor
(among those examined for the risk)
Genetic
Any mutation 61 (United States/Canada/Israel)*
39 (Japan)
PRSS1 33 (United States/Canada/Israel)*
15 (Japan)
SPINK1 20 (United States/Canada/Israel)*
18 (Japan)
CFTR 28 (United States/Canada/Israel)*
CTRC 7 (United States/Canada/Israel)*
2 (Japan)
CPA1 2 to 4 (Japan)Δ
1 to 3 (Europe)Δ
Obstructive/biliary
Any obstructive/biliary risk factor 33*
Pancreas divisum 12*
Other obstructive/biliary risk factors◊ Approximately 21*
Toxic-metabolic
Any toxic-metabolic factor 21*
Hyperlipidemia (most commonly hypertriglyceridemia) 6*
Drugs (eg, metronidazole, mercaptopurine, valproate, isoniazid) 12*
Other toxic-metabolic risk factor§ Approximately 9*
Autoimmune
Autoimmune pancreatitis 14*
This table shows the proportion of patients with certain risk factors for chronic or acute recurrent pancreatitis from several different populations. Note that some patients may have more than one risk factor.
PRSS1: protease serine 1 gene; SPINK1: serine protease inhibitor Kazal-type 1 gene; CFTR: cystic fibrosis transmembrane conductance regulator gene; CTRC: chymotrypsin C gene; CPA1: carboxypeptidase A1.
* Data from the INSPPIRE cohort (United States, Canada, and Israel) of 301 children <19 years (mean age at onset of acute pancreatitis 8.1 years)[1].
¶ Data from a Japanese cohort of 128 children <16 years old (mean age at onset of acute pancreatitis 7.6 years)[2].
Δ Data from adults with non-alcoholic chronic pancreatitis from Germany (n = 944), France (n = 456), Czech Republic (n = 21), Poland (n = 123), India (n = 230), and Japan (n = 247)[3].
◊ Other obstructive/biliary risk factors include pancreatobiliary malunion, gallstones, trauma, sphincter of Oddi dysfunction, annular pancreas, and other duct obstructions.
§ Other toxic-metabolic includes active and passive tobacco smoking, alcohol use, chronic kidney disease, and organic acidemias.
References:
  1. Kumar S, Ooi CY, Werlin S, et al. Risk factors associated with pediatric acute recurrent and chronic pancreatitis: Lessons from INSPPIRE. JAMA Pediatr 2016; 170:562.
  2. Saito N, Suzuki M, Sakurai Y, et al. Genetic analysis of Japanese children with acute recurrent and chronic pancreatitis. JPGN 2016; 63:431.
  3. Witt H, Beer S, Rosendahl J, et al. Variants in CPA1 are strongly associated with early onset chronic pancreatitis. Nat Genet 2013; 45:1216.
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