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Vol. 24 - Num. 94

Clinical Reviews

Hyperammonaemia as onset of food protein-induced enterocolitis syndrome

Sara López Lópeza, Valewska G. Wallis Gómezb, Luis Peña Quintanac

aPediatra. CS de San Gregorio. Telde. Servicio de Pediatría. Complejo Hospitalario Universitario Insular Materno-Infantil. Las Palmas de Gran Canaria. España.
bUnidad de Cuidados Paliativos Pediátricos. Servicio de Pediatría. Complejo Hospitalario Universitario Insular Materno-Infantil. Las Palmas de Gran Canaria. España.
cSección de Gastroenterología, Hepatología y Nutrición Pediátrica. Servicio de Pediatría. Complejo Hospitalario Universitario Insular Materno-Infantil. CIBEROBN. ACIP. Universidad de Las Palmas de Gran Canaria. España.

Correspondence: S López. E-mail: sara.lopez0192@gmail.com

Reference of this article: López López S, Wallis Gómez VG, Peña Quintana L. Hyperammonaemia as onset of food protein-induced enterocolitis syndrome. Rev Pediatr Aten Primaria. 2022;24:e229-e233.

Published in Internet: 20-06-2022 - Visits: 2247

Abstract

Food protein-induced enterocolitis syndrome (FPIES) is the most severe non-IgE-mediated allergies, and has a broad clinical spectrum. Chronic FPIES usually manifests with lethargy, pallor, undernutrition, intermittent vomiting and/or chronic diarrhoea associated with multiple laboratory abnormalities (leucocytosis with left shift, eosinophilia, anaemia, thrombocytosis, hypoproteinaemia, hypoalbuminaemia, methemoglobinemia and metabolic acidosis). It resolves 3 to 10 days after exclusion of the causative allergen, and most patients develop tolerance to the protein again at approximately 3 to 5 years of age. We present the case of a 47-day-old infant who presented with vomiting and diarrhoea with severe dehydration, metabolic acidosis, methaemoglobinaemia, hypoproteinaemia, hypoalbuminaemia and hyperammonaemia, the latter being a feature that has only been described in one other case before.

Keywords

Cow's milk protein allergy Dehydration Differential diagnosis Enterocolitis Hyperammonemia

 

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