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The Clinical Prehistory of Food-Protein Induced Enterocolitis Syndrome (FPIES)

      Rationale

      Misdiagnosis and delays in diagnosis for children with Food Protein-Induced FPIES is common. We evaluated the diagnostic itinerary of FPIES children before the correct diagnosis.

      Methods

      A retrospective assessment of clinical records of 23 children diagnosed with FPIES was performed.

      Results

      Between August 2012 and July 2015, twenty-three children (14 males and 9 females) were diagnosed with FPIES (mean age at onset: 3.85±2.66 months). The diagnosis was made in seven cases at the first clinical food reaction. In the remaining 16 children, the mean time lapse between the first episode and the diagnosis was 10.02± 9.99 months with an average of three events (SD 1.58) before the proper identification. Specialists consulted for differential clinical suspect included:
      • Gastroenterologist (Meckel’s diverticulum, intussusception, pyloric stenosis, gastroenteritis, celiac disease, megacolon, congenital microvillus atrophy) in 9/23 cases;
      • Cardiologist (congenital cardiopathy) in 6/23;
      • Neurologist (seizures, intracranial hemorrhage) in 6/23;
      • Infectious disease specialist (sepsis) in 6/23;
      • Pediatric Surgeon (pyloric stenosis) in 4/23;
      • Endocrinologist (adrenal insufficiency, diabetes insipidus) in 4/23;
      • Dietician (Enteral nutrition) in 4/23;
      • Anesthetist (hypotension, tachycardia, arrhythmia, hyperpnea) in 3/23;
      • Metabolic disease specialist (acidosis, Hereditary Fructose Intolerance) in 3/23;
      • Nephrologist (urinary infection) in 3/23;
      • Immunologist (IPEX, primary immunodeficiency, hypogammaglobulinaemia) in 2/23;
      • Haematologist (Anemia, methemoglobinemia) in 2/23;
      • Geneticist (Genetic syndrome) in 2/23.

      Conclusions

      Delay in FPIES identification incur costs of specialist consultations, unnecessary (often-painful) procedures and the experience of multiple episodes. Educational training courses on FPIES for hospital-based pediatricians may reduce the diagnostic delay.