Chronic Pancreatitis in children under 18 in New South Wales, Australia: establishing suitable candidates for total pancreatectomy and islet auto transplantation (TPIAT)
Denghao Wu1, Louise Rushworth2, David J Torpy1,3, Patrick Toby H Coates1,4.
1School of Medicine, University of Adelaide, Adelaide, Australia; 2Sydney Clinical School, University of Notre Dame Australia, Sydney, Australia; 3Endocrinology , Royal Adelaide Hospital, Adelaide, Australia; 4Renal Transplantation, Royal Adelaide Hospital, Adelaide, Australia
Introduction: Chronic pancreatitis (CP) is a debilitating disease characterised by a spectrum of recurrent inflammatory disorders of the pancreas. It is typically an acquired condition related to prolong alcohol or tobacco intake. Around 1-4% of CP have a genetic cause and the disease aetiology of genetic/hereditary pancreatitis presents with early onset age, therefore CP diagnoses with earlier first age of onset are likely to be from the genetic aetiology. Due to this early and prolonged recurrent pancreatic assault and the debilitating abdominal pain caused by the inflammation, genetic or hereditary pancreatitis patients are candidates for the total pancreatectomy with islet auto-transplantation procedure. In this study, we assess the data on all hospital admissions for CP between 2006-2017 among children aged 0-18 years of age in New South Wales (NSW), Australia. The aim of the study is to verify the prevalence of chronic pancreatitis with a genetic cause in NSW and establish a registry for patients who are suitable candidates of the TPIAT program.
Methods: A dataset was extracted on all admissions to hospital for CP in NSW between 2006-2017 among children aged 0-18 from the NSW healthcare database. Exclusions criteria included [cystic fibrosis; malignancy; gall bladder/biliary causes; injury/poisoning; alcohol abuse; primary or secondary haematologic diagnoses; congenital disorders of liver/pancreas; obstetric admissions; admissions with a principal diagnosis of something major (e.g., respiratory failure); principal diagnosis of major psych disorder. Cases were not excluded based on smoking history (n=14).
Results: There were 488 admissions with a principal or comorbid diagnosis of CP. This corresponds to an approximate admission rate of 2.22/100,000/year. Among these, 126 (25.8%) admissions were under the age of 10, and 332 (68.0%) were female. 23 of the total admissions had a comorbid diagnosis of Type 1 Diabetes Mellitus.
Conclusion: The overall admission rate of CP in children under 18 in NSW was consistent with our previously reported prevalence of hereditary pancreatitis in the Australian population (1.1/ 100,000). This study successfully established the prevalence of CP in children under 18 in NSW, in which the most likely cause remains a genetic aetiology. Work is currently ongoing in further describing an Australia-wide TPIAT candidate registry which will incorporate all diagnoses of genetic/hereditary pancreatitis patients.
