Fruit-induced FPIES masquerading as hereditary fructose intolerance

Area of Science:

• Pediatrics
• Gastroenterology
• Allergy

Background:

• Hereditary fructose intolerance (HFI) is a metabolic disorder triggered by fructose ingestion, typically presenting during weaning.
• Symptoms of HFI include vomiting, hypoglycemia, and failure to thrive after consuming fructose-containing foods.

Observation:

• A case of an infant with suspected HFI presented with recurrent vomiting and hypotension after fruit ingestion.
• Despite negative genetic testing for HFI, strict fruit avoidance resolved symptoms.
• Oral fructose challenge caused severe hypotension, not hypoglycemia or fructosuria, and allergy tests were negative.

Findings:

• The infant's presentation and response to dietary changes were diagnostic of fruit-induced food protein-induced enterocolitis syndrome (FPIES).
• FPIES is a non-IgE mediated gastrointestinal hypersensitivity causing severe vomiting, dehydration, and lethargy, mimicking HFI.

Implications:

• Pediatricians should consider FPIES in the differential diagnosis of suspected HFI, especially when genetic testing is negative.
• Accurate diagnosis of FPIES is crucial to prevent unnecessary dietary restrictions and manage symptoms effectively.
• This case underscores the importance of a thorough clinical history and diagnostic approach in differentiating food-related infant disorders.