[Genetic mutations as a cause of acute recurrent pancreatitis in children - case report and literature review]

Dev Period Med. 2016;20(3):228-234.
[Article in Polish]

Abstract

Acute recurrent pancreatitis is not common in children. The epidemiology, etiology and clinical presentation of pediatric acute recurrent pancreatitis are not well understood. The etiology is diverse and multifactorial, with many cases being idiopathic. The most common etiology of acute recurrent pancreatitis in children are genetic factors, biliary duct disorders, anatomic anomalies of the pancreatobiliary system and metabolic diseases. Mutations are most commonly found in the cationic trypsynogen gene (PRSS1), the pancreatic secretory trypsin inhibitor gene (SPINK1) and the cystic fibrosis transmembrane conductance regulator gene (CFTR). The case described here is that of a 6-year-old boy, without a family history of pancreatitis, who was hospitalized several times over 5 years, with epigastric pain and high serum levels of amylase and lipase. Genetic testing showed a heterozygous variation, c.194+2T>C (IVS3+2T>C) in the SPINK1 gene and variation c.1210-34TG(11) T(5) (IVS8-5T+(TG)11) in the CFTR gene. Other etiological factors also occurred leading to the initiation and relapses of the disease.

Keywords: acute recurrent pancreatitis; children; etiology; genetic mutation.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Acute Disease
  • Carrier Proteins / genetics
  • Child
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Humans
  • Male
  • Pancreatitis / genetics*
  • Polymorphism, Genetic / genetics*
  • Trypsin / genetics
  • Trypsin Inhibitor, Kazal Pancreatic

Substances

  • CFTR protein, human
  • Carrier Proteins
  • SPINK1 protein, human
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • Trypsin Inhibitor, Kazal Pancreatic
  • PRSS1 protein, human
  • Trypsin