Food Protein-Induced Enterocolitis Syndrome in a Neonate with Peripheral Epimerase Deficiency Galactosemia

ABSTRACT

Food protein-induced enterocolitis syndrome (FPIES) is a severe infantile form of non-immunoglobulin E-mediated gastrointestinal food hypersensitivity that manifests as profuse, repetitive vomiting, often with diarrhea, which leads to acute dehydration and lethargy and failure to thrive if chronic. Symptoms such as dehydration and lethargy are also observed in sepsis, viral infection, and food poisoning. It is difficult to differentiate FPIES from sepsis-like illness. The diagnosis is based on clinical criteria and/or an oral food challenge. FPIES developed in the patient with peripheral epimerase deficiency galactosemia after the use of soy formula. The change in feeding to soy formula is not required of a patient with peripheral epimerase deficiency galactosemia. Early intake of soy formula in our patient was harmful. Therefore, we think the changing the formula should be taken carefully. Another important point is the diagnosis. Late diagnosis and misdiagnosis are common, and inappropriate treatment or invasive treatment can occur.