FPIES in exclusively breastfed infants: two case reports and review of the literature
Francesco Baldo1, Martina Bevacqua2, Cristiana Corrado2
1Department of Medicine, Surgery and Health Sciences, University of Trieste, Piazzale Europa 1, 34127, Trieste, Italy. francescobaldo11@yahoo.it.
Insights
Food Protein-Induced Enterocolitis Syndrome (FPIES) can occur in exclusively breastfed infants. Methemoglobin evaluation may aid diagnosis in these rare cases, simplifying the process for clinicians.
Area of Science:
- Pediatric Allergy and Immunology
- Gastroenterology
- Neonatology
Background:
- Food Protein-Induced Enterocolitis Syndrome (FPIES) is a non-IgE mediated allergic reaction typically affecting infants.
- While commonly associated with formula or solids, FPIES can manifest in exclusively breastfed infants due to maternal milk transmission of allergens, primarily cow's milk proteins.
- FPIES in exclusively breastfed infants is exceptionally rare, with limited documented cases.
Observation:
- Two cases of FPIES in exclusively breastfed infants are presented, both exhibiting severe symptoms like vomiting and diarrhea.
- One infant presented with septic-like conditions and elevated methemoglobin (13%); symptoms improved upon breastfeeding cessation and worsened with reintroduction.
- The second infant had persistent vomiting and diarrhea, elevated methemoglobin (7%), and duodenal eosinophilic infiltration confirmed via biopsy.
Findings:
- A literature review identified eight cases of FPIES in exclusively breastfed infants, with cow's milk being the most common trigger.
- Most cases were initially misdiagnosed as gastroenteritis or sepsis, highlighting diagnostic challenges.
- Methemoglobin levels were not previously assessed in these cases but were elevated in the presented infants, suggesting its diagnostic utility.
Implications:
- FPIES should be considered in exclusively breastfed infants presenting with compatible symptoms and history, even if rare.
- Measuring methemoglobin levels can be a valuable tool to aid in the diagnosis of FPIES in this population.
- Maternal dietary exclusion and amino acid-based formulas were effective in managing symptoms in the presented cases.
Background:
Food Protein-Induced Enterocolitis Syndrome (FPIES) is a non IgE-mediated food allergy that generally affects children in the first year of life. Usually symptoms break out when formula milk or solid foods are introduced for the first time but they might also appear in exclusively breastfed infants, since the trigger elements, especially cow's milk proteins, can be conveyed by maternal milk as well. FPIES in exclusively breastfed babies is a very rare clinical condition and only few cases have been reported in the medical literature.
Case Presentation:
We describe two cases of FPIES in exclusively breastfed babies. The first one is a two-month-old infant with a brief history of vomit and diarrhea that presented to the Emergency Department in septic-like conditions. The main laboratory finding was a significant increase in methemoglobin (13%). Clinically, we noted that, when breastfeeding was suspended, diarrhea drastically improved, and vice versa when maternal milk was reintroduced. An amino acid-based formula allowed a complete normalization of the symptoms. The second one is a three-month-old infant admitted for a 3 days history of persistent vomit and diarrhea. Blood tests showed a raised level of methemoglobin (7%). An esophagogastroduodenoscopy was performed and biopsies showed an eosinophilic infiltration of the duodenal mucosa. A maternal exclusion diet and an amino acid-based formula allowed a rapid regularization of the bowel function.
Conclusions:
We searched all the cases of FPIES in exclusively breastfed babies reported in the medical literature, identifying eight patients, with an average age of 3 months (range 15 days - 6 months). The majority of the cases were initially diagnosed as gastroenteritis or sepsis, five cases were characterized by an acute on chronic scenario and cow's milk was the most frequently involved food. Methemoglobin was never tested. An oral food challenge test was performed in two patients. FPIES in exclusively breastfed infants is a rare condition that, in the presence of compatible history and symptoms, should be considered also in exclusively breastfed babies. The evaluation of methemoglobin can simplify the diagnostic process.
