Food protein-induced enterocolitis syndrome

Background

• Non-IgE mediated immune reaction to food protein
• Peak incidence in infants 0-9 months
• Cow's milk and soy (in patient's diet or maternal diet if breast fed) are most common culprits

Clinical Features

• Acute reactions can cause life-threatening volume depletion
  • Onset of symptoms ~1-6 hours after ingesting culprit (possibly reintroduced after period of not consuming)
  • Profuse, repetitive vomiting and voluminous diarrhea
  • Can lead to profound dehydration, shock, severe electrolyte abnormalities
  • 75% of infants with FPIES appear seriously ill, with ~15% requiring hospitalization for hypotension
• Symptoms may be mild-moderate if chronically exposed to offending food
  • Chronic watery diarrhea with blood or mucus, vomiting, failure to thrive

Differential Diagnosis

Nausea and vomiting (newborn)

Newborn	'
Obstructive intestinal anomalies	Esophageal stenosis/atresia Pyloric stenosis Intestinal stenosis/atresia Duodenal atresia Malrotation ± volvulus Incarcerated hernia Meconium ileus/plug Hirschsprung's disease Imperforate anus Enteric duplications
Neurologic	Intracranial bleed/mass Hydrocephalus Cerebral edema Kernicterus
Renal	Urinary tract infection Obstructive uropathy Renal insufficiency
Infectious	Viral illness Acute gastroenteritis Meningitis Sepsis
Metabolic/endocrine	Inborn errors of metabolism Adrenal insufficiency Congenital adrenal hyperplasia
Miscellaneous	Ileus Gastroesophageal reflux disease Necrotizing enterocolitis Milk allergy, FPIES GI perforation

Nausea and vomiting infant (<12 mo)

	'
Obstructive intestinal anomalies	Pyloric stenosis Malrotation ± volvulus Incarcerated hernia Hirschsprung's disease Enteric duplications Intussusception Foreign body Bezoar Meckel diverticulum
Neurologic	Intracranial hemorrhage/mass Hydrocephalus Cerebral edema (e.g. in DKA or cancer)
Renal	Urinary tract infection Obstructive uropathy Renal insufficiency
Infectious	Viral illness Acute gastroenteritis Meningitis Sepsis Otitis media Pneumonia Pertussis Hepatitis
Metabolic/endocrine	Inborn errors of metabolism Adrenal insufficiency Renal tubular acidosis DKA
Miscellaneous	Ileus Gastroesophageal reflux disease Post-tussive Peritonitis Drug overdose

• Overfeeding
• Starvation stools
• Short bowel syndrome
• Cystic fibrosis
• Celiac disease
• Disaccharidase deficiency
• Secretory neoplasms
• Immunodeficiency
• Inflammatory bowel disease
• Antibiotic-associated diarrhea
• Neonatal drug withdrawal
• Toxins
• Hemolytic uremic syndrome

Evaluation

• Clinical diagnosis
• Labs to assess for consequences of GI losses (e.g. BMP, Mg/Phos, VBG) +/- workup to exclude alternative diagnoses

Management

• Remove offending agent from diet (and maternal diet if patient breastfed)
• Volume resuscitation (see: dehydration (peds))
• Correct electrolyte abnormalities
• Antiemetics prn
• If severe (e.g. intractable vomiting, ill-appearing, lethargic, hypotensive)
  • Methylprednisolone 1mg/kg IV
  • Epinephrine 0.01 mg/kg IM if shock/hypotension refractory to IV fluids

Disposition

• Admit if requires IV rehydration, electrolyte abnormalities, or intractable symptoms