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Paeds Vivasgastroenterology-hepatology-and-nutrition

Paeds Vivas · gastroenterology-hepatology-and-nutrition

Food protein-induced enterocolitis and enteropathy — branching viva

Branching viva from the definition and the 2017 consensus diagnostic criteria of food protein-induced enterocolitis syndrome through the infant with a delayed pallid vomiting collapse, the faltering formula-fed infant with chronic FPIES, the substitute-feed choice, the oral food challenge, and the natural history of tolerance.

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Target exams

RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics

Target exams

RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
You are the paediatric registrar in a general clinic. The consultant asks you to talk through four children referred with possible food protein-induced enterocolitis syndrome: an infant with a delayed pallid vomiting collapse after rice, a six-week-old with faltering growth on cow's-milk formula, an older child with fish FPIES, and a toddler whose family asks whether the allergy has been outgrown.

Station opening

Examiner: "Define food protein-induced enterocolitis syndrome, and explain how you would tell it apart from sepsis and from IgE-mediated food allergy." [1]

Strong candidate (must-hit)

  • Defines FPIES as a non-IgE-mediated, cell-mediated food allergy in which a trigger protein produces delayed repetitive vomiting one to four hours after ingestion with lethargy, pallor and watery diarrhoea, in the absence of classic IgE-mediated skin or respiratory features; separates it from sepsis by the recurring, food-timed, culture-negative pattern and from IgE-mediated allergy by the delay and the absence of urticaria, wheeze and angio-oedema; states that skin-prick tests and specific immunoglobulin E are negative in classic disease. [1]

Weak candidate

  • "It is a kind of food allergy, and I would do a skin-prick test to confirm it." [1]

Branch A — The infant with a delayed collapse after rice

Examiner: "A seven-month-old becomes pale, lethargic and vomits repetitively two hours after rice cereal, having had the identical pattern two weeks earlier. How do you apply the diagnostic criteria, and what is your immediate management?" [1]

Strong

  • Applies the 2017 international consensus criteria: the major criterion of delayed vomiting one to four hours without IgE features is met, with minor criteria of a repeat reaction to the same food, lethargy, pallor and emergency presentation (three or more required); manages the acute reaction as a volume-depleting collapse with airway-breathing-circulation, intravenous access, repeated 20 mL/kg boluses of isotonic crystalloid to perfusion, intravenous ondansetron for vomiting and a single dose of methylprednisolone considered, with correction of acid-base and electrolytes; then plans strict rice avoidance and a supervised oral food challenge. [1] [8]

Weak

  • "I would treat it as sepsis with fluids and antibiotics and not change the diet." [1]

Branch B — The faltering formula-fed infant

Examiner: "A six-week-old on cow's-milk formula has three weeks of intermittent vomiting, watery stools and a fall across two weight centiles, resolving over several days when the feed is changed. What is the diagnosis, why are the allergy tests negative, and how do you choose the substitute feed?" [6]

Strong

  • Diagnoses chronic FPIES to cow's milk from ongoing ingestion, resolving on elimination; explains that the T-cell-mediated mechanism makes skin-prick tests and specific immunoglobulin E negative in classic disease and unable to confirm it; chooses an extensively hydrolysed formula first-line, escalating to an amino-acid formula if symptoms persist, and uses soy with caution because a meaningful proportion of cow's-milk-FPIES infants also react to soy; confirms and tests tolerance with a medically supervised oral food challenge. [1] [6]

Weak

  • "I would start a soy formula and check a cow's-milk immunoglobulin E panel to confirm the allergy." [6]

Branch C — The older child with fish FPIES

Examiner: "A four-year-old has had two delayed vomiting and pallor reactions after fish. What is particular about fish and shellfish as triggers, and how does the outlook differ from cow's-milk FPIES?" [9]

Strong

  • Identifies fish and shellfish as triggers that present more often in older children and carry a regional pattern, prominent in Mediterranean and some Asian cohorts; explains that fish and shellfish FPIES tends to persist longer than cow's-milk FPIES, so tolerance testing is continued at intervals by supervised challenge rather than assumed; manages with strict avoidance, school-staff education, label reading and an up-to-date action plan. [9]

Weak

  • "Fish allergy is always IgE-mediated, so I would manage it with an adrenaline autoinjector and a skin-prick test." [9]

Branch D — Has the allergy been outgrown?

Examiner: "The family of a three-year-old with cow's-milk FPIES asks whether the allergy has gone. How do you assess tolerance, and what is the likely outlook?" [4]

Strong

  • Explains that most children outgrow FPIES and that cow's-milk FPIES resolves earliest, typically within the first few years; assesses tolerance at intervals by a medically supervised oral food challenge with intravenous access available and four-to-six-hour observation, after which a negative challenge permits home reintroduction of cow's milk under guidance, while a positive challenge continues avoidance until the next assessment. [4] [1]

Weak

  • "I would check a cow's-milk immunoglobulin E level, and if it is normal the allergy has gone." [4]

Close

Examiner: "Summarise your approach to food protein-induced enterocolitis syndrome in one sentence." [1]

Strong

  • "Food protein-induced enterocolitis syndrome is a non-IgE-mediated food allergy with delayed vomiting one to four hours after a trigger and pallor, lethargy and watery diarrhoea: I recognise it from the timing and the absence of urticaria, separate it from sepsis and IgE-mediated allergy, confirm it with the consensus criteria and a supervised oral food challenge rather than skin-prick tests, resuscitate the acute reaction with intravenous fluids and ondansetron, and manage long term with strict avoidance, a substitute feed, a written action plan and planned tolerance testing, because most children outgrow it." [1] [6]

References

  1. [1]Nowak-Węgrzyn A; Chehade M; Groetch ME; Spergel JM; Wood RA; Allen K International consensus guidelines for the diagnosis and management of food protein-induced enterocolitis syndrome: Executive summary-Workgroup Report of the Adverse Reactions to Foods Committee, American Academy of Allergy, Asthma & Immunology. J Allergy Clin Immunol, 2017.PMID 28167094
  2. [2]Sicherer SH; Eigenmann PA; Sampson HA Clinical features of food protein-induced enterocolitis syndrome. J Pediatr, 1998.PMID 9709708
  3. [4]Caubet JC; Ford LS; Sickles L; Järvinen KM; Sicherer SH; Sampson HA; Nowak-Węgrzyn A Clinical features and resolution of food protein-induced enterocolitis syndrome: 10-year experience. J Allergy Clin Immunol, 2014.PMID 24880634
  4. [6]Leonard SA; Pecora V; Fiocchi AG; Nowak-Węgrzyn A Food protein-induced enterocolitis syndrome: a review of the new guidelines. World Allergy Organ J, 2018.PMID 29441147
  5. [8]Feuille E; Nowak-Węgrzyn A Medical Algorithms: Recognizing and treating food protein-induced enterocolitis syndrome. Allergy, 2019.PMID 31070799
  6. [9]Miceli Sopo S; Monaco S; Badina L; Barni S; Toscano A; Tripodi S; Meglio P Food protein-induced enterocolitis syndrome caused by fish and/or shellfish in Italy. Pediatr Allergy Immunol, 2015.PMID 26287446