Chronic and Hereditary Pancreatitis

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Chronic pancreatitis is the continuation of progressive inflammatory damage to the pancreas, which can lead to endocrine and/or exocrine dysfunction. It is associated with significantly impaired quality of life in affected children, with intractable pain, missed days of school, and frequent emergency department or hospital evaluations. Diagnosis can be established with a combination of characteristic pancreatic pain, imaging findings, or endocrine and/or exocrine dysfunction. Careful assessment of risk factors, especially for hereditary factors, may assist with tailoring of interventions or determining prognosis. Management is complex and requires a multidisciplinary approach to optimize medical, endoscopic, and surgical therapy. Total pancreatectomy, islet autotransplant, could be considered with failure to respond to maximal therapies.

Original languageEnglish (US)
Title of host publicationTextbook of Pediatric Gastroenterology, Hepatology and Nutrition
Subtitle of host publicationA Comprehensive Guide to Practice: Second Edition
PublisherSpringer International Publishing
Pages461-471
Number of pages11
ISBN (Electronic)9783030800680
ISBN (Print)9783030800673
DOIs
StatePublished - Jan 1 2021

Bibliographical note

Publisher Copyright:
© The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2022.

Keywords

  • CFTR (cystic fibrosis transmembrane receptor gene)
  • Chronic pancreatitis
  • ERCP (endoscopic retrograde cholangiopancreatography)
  • Exocrine pancreatic insufficiency
  • Hereditary pancreatitis
  • INSPPIRE (INternational Study group of Pediatric Pancreatitis: In search for a cuRE)
  • MRCP (magnetic resonance cholangiopancreatography)
  • PERT (pancreatic enzyme replacement therapy)
  • PRSS1 (cationic trypsinogen gene)
  • TIGAR-O (Toxic/metabolic, Idiopathic, Genetic, Autoimmune, Recurrent/severe acute pancreatitis, and Obstructive risk factors)
  • TPIAT (total pancreatectomy, islet autotransplant)

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