Food protein-induced enterocolitis syndrome
ICD-10 K52.2 · ICD-11 DA94.22

Treatment of Severe Acute FPIES with Hypotonia, Lethargy, and Cyanotic Appearance

This protocol addresses the management of severe acute food protein-induced enterocolitis syndrome (FPIES) in a presentation defined by more than three episodes of emesis accompanied by severe lethargy, hypotonia, and an ashen or cyanotic skin appearance.

Clinical Presentation
  • More than 3 episodes of emesis
  • Severe lethargy
  • Hypotonia
  • Ashen or cyanotic skin appearance
Treatment Approach (Partial Overview)

Immediate management centres on rapid haemodynamic stabilisation through aggressive intravenous fluid resuscitation. Depending on age and clinical response, specific antiemetic therapy is incorporated. The protocol additionally addresses respiratory insufficiency and a range of metabolic complications that may arise in this severe presentation.

The complete structured regimen — including all agents, sequencing, criteria for each intervention, and supportive measures — is available in full via the link below.

Clinical Goals
  • Correction of hypotension and stable haemodynamics
  • Correction of acid-base and electrolyte abnormalities
  • Correction of methemoglobinemia
  • Patient back to baseline and tolerating oral fluids within 4–6 hours of reaction onset
Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.jaci.2016.12.966

>3 Episodes of emesis, with severe lethargy, hypotonia, ashen or cyanotic appearance

Place a peripheral intravenous line and administer normal saline bolus, 20 mL/kg rapidly; repeat as needed to correct hypotension

The priority in management of severe FPIES is restoration of stable hemodynamics through aggressive isotonic fluid resuscitation (eg, 10-20 mL/kg boluses of normal saline) repeated as needed and dextrose saline as a continuous intravenous maintenance infusion (Table VI).

In severe reactions patients might require supplemental oxygen, mechanical ventilation, or noninvasive positive pressure ventilation for respiratory insufficiency or failure, vasopressors for hypotension, bicarbonate for acidemia, and methylene blue for methemoglobulinemia.

Monitor and correct acid base and electrolyte abnormalities. Correct methemoglobinemia, if present. Discharge after 4-6 h from the onset of a reaction when the patient is back to baseline and is tolerating oral fluids.

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