title: Therapeutic step-up strategy for management of hereditary pancreatitis in children.
abstract: OBJECTIVE
  Various different regimes exist for the treatment of hereditary pancreatitis in childhood. Here, we propose a therapeutic pathway with emphasis on endoscopic and surgical procedures.
  
  
  METHODS
  From 2006 to 2013, 12 patients with a diagnosis of hereditary pancreatitis were prospectively included in a therapeutic step-up schema. The treatment outcome was evaluated and correlated to aetiological factors and pathoanatomic findings.
  
  
  RESULTS
  After diagnostic work-up (laboratory data, ultrasound examination, magnetic resonance cholangiopancreatography and genetic testing), all 12 patients underwent early endoscopic retrograde cholangiopancreatography (ERCP), which was successfully performed in ten children. Obstructive pancreatitis was found in eight children, and required sphincterotomy, dilation and stenting for 12 months. In two children with unsuccessful ERCP, open surgical drainage procedures were performed. After a mean follow-up of 32 months all children are free of recurrence of pancreatitis without any impairment of everyday activities.
  
  
  CONCLUSIONS
  For children with hereditary pancreatitis, a therapeutic step plan with early ERCP and open surgical drainage procedures in case of impossible or insufficient endoscopic treatment prevents recurring pancreatitis and offers a normal quality of life without any major complications.
authors.affiliation: Department of Pediatric Surgery, Women's and Children's Hospital Linz. Electronic address: simon.kargl@gespag.at.
authors.forename: S
authors.initials: S
authors.lastname: Kargl
authors.affiliation: Department of Gastroenterology and Hepatology, Elisabethinen Hospital Linz.
authors.forename: M
authors.initials: M
authors.lastname: Kienbauer
authors.affiliation: Department of Genetics, Women's and Children's Hospital Linz.
authors.forename: H C
authors.initials: HC
authors.lastname: Duba
authors.affiliation: Department of Gastroenterology and Hepatology, Elisabethinen Hospital Linz.
authors.forename: R
authors.initials: R
authors.lastname: Schöfl
authors.affiliation: Department of Pediatric Surgery, Women's and Children's Hospital Linz.
authors.forename: W
authors.initials: W
authors.lastname: Pumberger
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002760:Cholangiopancreatography, Endoscopic Retrograde / Q000379:methods*; D049448:Cholangiopancreatography, Magnetic Resonance / Q000379:methods*; D003937:Diagnosis, Differential; D019468:Disease Management; D005260:Female; D005820:Genetic Testing; D006801:Humans; D008297:Male; D050500:Pancreatitis, Chronic / Q000175:diagnosis / Q000601:surgery*; D017410:Practice Guidelines as Topic; D016896:Treatment Outcome
keywords: Endoscopic retrograde cholangiopancreatography; Hereditary pancreatitis; Pancreatic surgery