Connect
MJA
MJA

South Australian experience with paediatric total pancreatectomy and islet autotransplantation for PRSS1‐associated hereditary pancreatitis

Jessica Eldredge, Michael R Couper, David J Moore, Sanjeev Khurana, John WC Chen, Jennifer J Couper, Christopher J Drogemuller, Toni Radford, Thomas W Kay, Tom Loudovaris, Michael Wilks, Patrick T Coates and Richard TL Couper
Med J Aust 2021; 215 (7): . || doi: 10.5694/mja2.51247
Published online: 4 October 2021

Evidence supports the establishment of a national TP‐IAT program, delivered at sites with a concentration of expertise and experience

Chronic pancreatitis is probably an underestimated cause of chronic abdominal pain in children, and its true incidence remains unknown.1 Chronic inflammatory cell infiltration and fibrosis of pancreatic tissue results in exocrine and endocrine insufficiency with malabsorption and ultimately diabetes mellitus. Although obstruction, toxins and other factors can be associated, genetic mutations contribute toward childhood chronic pancreatitis in 65–75% of cases.2,3


  • 1 Women's and Children's Hospital, Adelaide, SA
  • 2 University of Adelaide, Adelaide, SA
  • 3 Flinders Medical Centre, Adelaide, SA
  • 4 Flinders University, Adelaide, SA
  • 5 Royal Adelaide Hospital, Adelaide, SA
  • 6 St Vincent's Institute of Medical Research, Melbourne, VIC
  • 7 University of Melbourne, Melbourne, VIC
  • 8 Radiology SA, Adelaide, SA


Correspondence: richard.couper@sa.gov.au

Acknowledgements: 

We thank Paul Flynn and the Hospital Research Foundation, South Australia, who enabled funding of four of five TP‐IAT procedures undertaken, along with the attendance of international experts to help with the islet isolation process. We also thank Melena Bellin (University of Minnesota), Ashley Dennison (University of Leicester), Greg Szot (University of California, San Francisco) and Appakalai Balamurugan (University of Louisville) for their contributing expertise. We also acknowledge the clinicians involved in the patients’ care during and following the procedures, including from the islet transplant and interventional radiology services at the Royal Adelaide Hospital; the gastroenterology, endocrinology and paediatric intensive care units at the Women’s and Children’s Hospital, Adelaide; and the islet transplantation laboratory at St Vincent’s Institute of Medical Research, Melbourne.

Competing interests:

Patrick Coates has received grants and funding from Kidney Transplant Diabetes Research Australia.

  • 1. Nydegger A, Couper RT, Oliver MR. Childhood pancreatitis. J Gastroenterol Hepatol 2006; 21: 499–509.
  • 2. 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: 562–569.
  • 3. Giefer MJ, Lowe ME, Werlin SL, et al. Early‐onset acute recurrent and chronic pancreatitis is associated with PRSS1 or CTRC gene mutations. J Pediatr 2017; 186: 95–100.
  • 4. Gorry MC, Gabbaizedeh D, Furey W et al. Mutations in the cationic trypsinogen gene are associated with recurrent acute and chronic pancreatitis. Gastroenterology 1997; 113: 1063–1068.
  • 5. Bellin MD, Freeman ML, Schwarzenberg SJ, et al. Quality of life improves for pediatric patients after total pancreatectomy and islet autotransplant for chronic pancreatitis. Clin Gastroenterol Hepatol 2011; 9: 793–799.
  • 6. Chinnakotla S, Bellin MD, Schwarzenberg SJ, et al. Total pancreatectomy and islet autotransplantation in children for chronic pancreatitis: indication, surgical techniques, postoperative management, and long‐term outcomes. Ann Surg 2014; 260: 56–64.
  • 7. Bellin MD, Forlenza GP, Majumder K, et al. Total pancreatectomy with islet autotransplantation resolves pain in young children with severe chronic pancreatitis. J Pediatr Gastroenterol Nutr 2017; 64: 440–445.
  • 8. Sutherland DE, Radosevich DM, Bellin MD, et al. Total pancreatectomy and islet autotransplantation for chronic pancreatitis. J Am Coll Surg 2012; 214: 409–424; discussion, 424–426.
  • 9. Wahoff DC, Paplois BE, Najarian JS, et al. Islet autotransplantation after total pancreatectomy in a child. J Pediatr Surg 1996; 31: 132–135.
  • 10. Webster AC, Hedley JA, Anderson PF, et al. Australia and New Zealand Islet and Pancreas Transplant Registry annual report 2018: islet donations, islet isolations, and islet transplants. Transplant Direct 2019; 5: e421.
  • 11. O’Connell PJ, Holmes‐Walker DJ, Goodman D, et al. Multicenter Australian trial of islet transplantation: improving accessibility and outcomes. Am J Transplant 2013; 13: 1850–1858.
  • 12. Bellin MD, Freeman ML, Gelrud A, et al. Total pancreatectomy and islet autotransplantation in chronic pancreatitis: recommendations from PancreasFest. Pancreatology 2014; 14: 27–35.
  • 13. Johnston PC, Lin YK, Walsh RM, et al. Factors associated with islet yield and insulin independence after total pancreatectomy and islet cell autotransplantation in patients with chronic pancreatitis utilizing off‐site islet isolation: Cleveland Clinic experience. J Clin Endocrinol Metab 2015; 100: 1765–1770.
  • 14. Tai DS, Shen N, Szot GL, et al. Autologous islet transplantation with remote islet isolation after pancreas resection for chronic pancreatitis. JAMA Surg 2015; 150: 118–124.
  • 15. Tu CG, Khurana S, Couper RT, Ford AW. Kasai hepatoportoenterostomy in South Australia: a case for ‘centralized decentralization’. ANZ J Surg 2015; 85: 865–868.

Author

remove_circle_outline Delete Author
add_circle_outline Add Author

Comment
Do you have any competing interests to declare? *

I/we agree to assign copyright to the Medical Journal of Australia and agree to the Conditions of publication *
I/we agree to the Terms of use of the Medical Journal of Australia *
Email me when people comment on this article

Online responses are no longer available. Please refer to our instructions for authors page for more information.