Hospital-based Introduction of Untested High-risk Foods for Down Syndrome Infant with Severe Food Protein-induced Enterocolitis Syndrome: A Case Report

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Down syndrome (DS) is a risk factor for severe food protein-induced enterocolitis syndrome (FPIES), with DS patients tending to have multiple-food FPIES. This is the first case where a DS infant with a history of severe chronic FPIES to milk and soy could effectively be introduced with some untested high-risk foods through hospital-based oral food challenges (OFCs).

The infant is a 20-month-old girl with DS, who was diagnosed with milk- and soy-induced FPIES. Considering her history of intensive care unit care for severe FPIES reactions, we considered that introducing other high-risk foods, such as wheat and hen's egg (white and yolk), at home was not appropriate for her. We offered hospital-based OFCs effectively and safely by introducing wheat and hen's egg as high-risk foods against FPIES to the 20-month-old infant. As a result, she tolerated soy-based seasoning, wheat, and egg whites without any symptoms, but she developed frequent vomiting after ingesting egg yolk. We did a prompt intervention with intravenous fluid replacement to prevent severe adverse conditions. After discharge, she exhibited an FPIES symptom as a consequence of ingesting green peas and miso; hence, we recommended the elimination of peas, in addition to soy, milk, and egg yolk, from her diet. She remained symptom-free since adhering to this dietary regimen.

In severe FPIES children, it is encouraged to introduce unconsumed high-risk foods in the hospital safely to avoid severe reactions at home and prevent unnecessary food eliminations.