The Disease Burden of Hereditary Pancreatitis on Children and Their Families: A Systematic Review

Objective

To systematically review existing research evidence on the disease burden of hereditary pancreatitis (HP) in children, focusing on physical, psychological, social and financial consequences for families.

Methods

The review followed Synthesis Without Meta-analysis (SWiM) and PRSIMA guidelines. A structured search of PubMed, Google Scholar, and EMBASE was conducted. The PCC (Population, Concept, Context) framework guided study selection, targeting children (0-18 years) with HP and assessing physical, psychological, socioeconomic impacts. Data was extracted using a standardised form, and study quality was appraised using the Joanna Briggs Institute Checklist. Due to heterogeneity, a narrative synthesis was conducted using thematic analysis.

Results

Six studies met inclusion criteria. Children with HP presented chronic abdominal pain and hospitalisations, with median lifetime hospitalisations of 4 in chronic versus 1 in acute recurrent cases. Exocrine pancreatic insufficiency (EPI) were more common in chronic pancreatitis (CP) (33% vs 10%), and diabetes occurred in 8.7% versus 5.9%. Psychological distress was prevalent, with 19% showing clinically significant internalising behaviours and reduced quality of life. Up to 70% missed ≥1 school day per month, with greater pain-related social interference among females. High treatment costs with annual treatment exceeding $40,000 per patient.

Conclusion

HP significantly affects the physical and mental health of children and imposes financial and social strain on families. A structured, multidisciplinary approach to care including improved medical management, psychological support, and financial assistance is essential. Further research is required to develop targeted interventions aimed at managing psychological distress, reducing hospitalisations, and improving the quality of life of these children.

What is already known

• Hereditary pancreatitis (HP) is a rare genetic disorder, associated with lifelong complications including abdominal pain, nausea and increased risk of pancreatic cancer.

• Evidence on wider psychosocial and financial impacts on affected children and their families has been fragmented and underreported.

What this study adds

• This review shows that HP disrupts education, increases psychosocial distress, and imposes heavy financial burden on the healthcare system.

How this study might affect research, practice or policy

• Multidisciplinary care models that include psychological and social support, alongside medical management, are urgently needed.

• Further research should focus on targeted interventions to reduced hospitalisations and improve quality of life in affected children.