Food protein-induced enterocolitis and enteropathy — structured clinical encounter

Station brief (candidate)

You are the paediatric registrar in the emergency department. A seven-month-old is brought in pale, lethargic and vomiting repetitively. Two hours earlier the child ate rice cereal for the second time, and the identical pattern occurred two weeks ago after rice. The team is treating the child as a possible case of sepsis and has asked you to establish the diagnosis, to direct the resuscitation, and to plan the longer-term pathway. You have 12 minutes with the team and 5 minutes for examiner discussion. [1]

Information available on request

• Seven months old, previously well; repetitive vomiting, marked pallor and lethargy beginning about two hours after rice cereal; watery diarrhoea followed in the emergency department. [2]
• Identical episode two weeks earlier after rice; no urticaria, angio-oedema, stridor or wheeze on either occasion; otherwise well and growing between episodes. [1]
• Examination: pale, reduced tone, cool peripheries, tachycardic; capillary refill four seconds; no skin or respiratory features of IgE-mediated allergy. [8]
• Bloods (on request): haemoglobin 112 g/L, white-cell count 21 with neutrophilia; venous blood gas pH 7.24 with a metabolic acidosis; sodium 136, potassium 3.4, glucose 4.8; C-reactive protein 6. [1]
• Cultures and stool studies (on request): blood and urine cultures sterile; stool culture and viral panel negative. [11]
• Allergy testing (on request, after the event): skin-prick test and specific immunoglobulin E to rice both negative. [1]

Tasks

1. Give the diagnosis and apply the 2017 international consensus criteria that support it, and explain why this is not IgE-mediated allergy. [1]
2. Identify the dangerous mimics and explain how the history and the results separate them. [11]
3. Direct the immediate resuscitation, including fluids and specific drugs, with doses. [8]
4. Outline the longer-term plan: avoidance, the substitute feed, the written action plan, and the role of the oral food challenge. [6]

Marking anchors

Must-hit

• Recognises acute food protein-induced enterocolitis syndrome to rice from the delayed vomiting one to four hours after the trigger, the recurrence with the same food, the pallor and lethargy, and the absence of classic IgE-mediated skin or respiratory features; applies the 2017 consensus major criterion (delayed vomiting one to four hours without IgE features) with the minor criteria present (repeat reaction to the same food, lethargy, pallor, emergency presentation, neutrophilia), of which three or more are required. [1]
• Resuscitates the child as a volume-depleting, acidotic collapse: airway-breathing-circulation, intravenous access, repeated 20 mL/kg boluses of isotonic crystalloid until perfusion is restored, intravenous ondansetron for ongoing vomiting with a single dose of methylprednisolone considered, and correction of the metabolic acidosis and hypokalaemia. [1] [8]
• Separates FPIES from sepsis (recurring food-timed pattern, sterile cultures, neutrophilia without a source) and from IgE-mediated allergy (delay rather than minutes, no urticaria or wheeze, negative skin-prick and specific immunoglobulin E). [1] [11]

Merit

• Outlines the longer-term plan: strict avoidance of rice and its cross-reactive grains with dietetic support, a written FPIES action plan (the ASCIA action plan in Australia and New Zealand), referral to a paediatric allergist, and a medically supervised oral food challenge with intravenous access available and four-to-six-hour observation to confirm the diagnosis and to test tolerance over time, noting that most children outgrow FPIES. [1] [6]

Fail

• Anchors on recurrent sepsis, gives antibiotics and fluids, and discharges the child back onto rice without recognising the food-timed pattern or providing an avoidance plan. [1]
• Relies on skin-prick tests or specific immunoglobulin E to make or to exclude the diagnosis, or confuses the delayed reaction with immediate IgE-mediated anaphylaxis and manages it only with adrenaline. [6]