Pancreatitis and pancreatic disorders — formative SAQs

SAQ 1 — The child on valproate with acute pancreatitis (20 marks, ~15 minutes)

An eight-year-old boy with epilepsy presents to the emergency department with a two-day history of severe, constant epigastric abdominal pain radiating to the back, nausea and vomiting. He has been on valproate for three years. On examination he is tachycardic with marked epigastric tenderness. His serum lipase is six times the upper limit of normal. An abdominal ultrasound shows an enlarged, oedematous pancreas with no gallstones. [9]

Questions

1. Give the diagnosis and state the NASPGHAN criteria that confirm it. (4 marks) [1]
2. Outline the early management in the first forty-eight hours, including fluid resuscitation, analgesia and nutrition. (6 marks) [2]
3. Explain the role of valproate and the immediate action required regarding the drug. (3 marks) [9]
4. State the position on prophylactic antibiotics and explain the reasoning. (3 marks) [2]
5. Outline the follow-up plan and the investigations to prevent recurrence. (4 marks) [5]

Model answer (must-hit)

1. The diagnosis is acute pancreatitis, likely medication-induced from valproate. The NASPGHAN criteria require at least two of three: characteristic abdominal pain (present — severe epigastric pain radiating to the back), a serum lipase or amylase at or above three times the upper limit of normal (present — six times), and imaging consistent with pancreatitis (present — enlarged oedematous pancreas on ultrasound). All three are met here, confirming the diagnosis. [1]

2. Early aggressive hydration with isotonic crystalloid, normal saline or lactated Ringer solution, at one and a half to two times the maintenance rate for the first twenty-four to forty-eight hours, titrated to urine output and clinical response. Adequate analgesia with age-appropriate opioids, because acute pancreatitis is severely painful and adequate analgesia does not mask surgical pathology once the diagnosis is established. Early enteral feeding within forty-eight to seventy-two hours as the child improves, starting with clear fluids and advancing to a low-fat diet, because early feeding reduces complications and length of stay compared with prolonged fasting. [2]

3. Valproate is one of the commonest medication causes of paediatric pancreatitis, acting through a direct toxic or idiosyncratic mechanism. It can occur at any time during treatment. The immediate action is to stop valproate and to engage the neurology team to find an alternative antiseizure medication, because continuing the drug risks recurrence. [9]

4. Prophylactic antibiotics are not recommended in acute pancreatitis, including in severe disease with necrosis, unless there is documented or strongly suspected infected necrosis. Routine antibiotics do not prevent infection, select resistant organisms, and do not improve outcomes. Antibiotics are reserved for proven infected necrosis, cholangitis or another documented infection. [2]

5. The follow-up plan includes confirming full biochemical recovery with a normal lipase before discharge, a medication review to ensure the offending drug is permanently replaced, counselling of the family on the drug trigger, and surveillance for recurrence. If the child has further episodes, a genetic panel including PRSS1, SPINK1, CFTR and CTRC, a metabolic screen for triglycerides and calcium, and a magnetic resonance cholangiopancreatography for ductal anatomy are indicated. [5]

SAQ 2 — The child with recurrent pancreatitis (20 marks, ~15 minutes)

A ten-year-old girl has had three discrete episodes of acute pancreatitis over the past two years, each confirmed by lipase elevation and imaging, with complete recovery between episodes including normalisation of enzymes. Her father had recurrent abdominal pain in childhood and was diagnosed with pancreatic cancer at forty-five. Her ultrasound is unremarkable. [5]

Questions

1. Define acute recurrent pancreatitis and confirm the diagnosis in this child. (4 marks) [5]
2. Give the most likely underlying cause and the genetic test that would confirm it. (5 marks) [5]
3. Explain the inheritance pattern and the long-term risks this carries. (4 marks) [5]
4. Describe the investigation pathway beyond the genetic test. (4 marks) [5]
5. Outline the principles of long-term management. (3 marks) [5]

Model answer (must-hit)

1. Acute recurrent pancreatitis is defined by the INSPPIRE criteria as two or more discrete episodes of acute pancreatitis with complete recovery, including normalisation of enzymes and resolution of symptoms, between episodes. This child meets the definition with three documented episodes and full inter-episode recovery, and the diagnosis is confirmed. [5]

2. The most likely underlying cause is hereditary pancreatitis caused by a gain-of-function mutation in the PRSS1 gene encoding cationic trypsinogen. The strong family history of recurrent abdominal pain in childhood and early-onset pancreatic cancer in the father strongly supports this. The genetic test is a PRSS1 gene analysis, which should be sent as the first-line genetic test for suspected hereditary pancreatitis. [5]

3. Hereditary PRSS1 pancreatitis follows an autosomal dominant inheritance pattern. The gain-of-function mutation causes trypsinogen to autoactivate prematurely inside the acinar cell, driving recurrent attacks from childhood. The long-term risks are a high probability of progression to chronic pancreatitis with exocrine and endocrine insufficiency, and a substantially elevated lifetime risk of pancreatic cancer that rises with the duration of disease, which is why surveillance in adulthood is recommended. Genetic counselling of the family and testing of first-degree relatives is essential. [5]

4. The investigation pathway beyond PRSS1 includes a broader genetic panel covering SPINK1, CFTR, CTRC and CASR if PRSS1 is negative or as additional modifier testing. Magnetic resonance cholangiopancreatography evaluates the ductal anatomy for pancreas divisum, strictures or stones. Faecal pancreatic elastase screens for early exocrine insufficiency below two hundred micrograms per gram. Serum triglycerides and calcium screen for metabolic triggers, and an immunoglobulin G4 level screens for autoimmune pancreatitis. [5]

5. The long-term management is multidisciplinary, coordinated by paediatric gastroenterology, with a low-fat diet to reduce secretory demand, alcohol avoidance counselling in adolescence, a multi-modal pain strategy that minimises opioid use, pancreatic enzyme replacement if exocrine insufficiency develops, glucose surveillance for pancreatic diabetes, and genetic counselling with planned cancer surveillance in adulthood. For intractable disease, total pancreatectomy with islet autotransplantation may be considered at a specialist centre. [5]