Abstract

LOPEZ-MEJIA, Lizbeth; GUILLEN-LOPEZ, Sara  and  VELA-AMIEVA, Marcela. Metabolic formulas for phenylketonuric patients available in Mexico. Bol. Med. Hosp. Infant. Mex. [online]. 2021, vol.78, n.6, pp.612-620.  Epub Sep 29, 2021. ISSN 1665-1146.  https://doi.org/10.24875/bmhim.20000399.

Hyperphenylalaninemias such as phenylketonuria are rare genetic diseases whose detection is currently mandated nationwide in both the public and private sectors in Mexico. Timely detection, diagnosis and treatment have been shown to prevent the neurological manifestations and disability that characterize this disease. Therefore, the importance of health personnel in charge of these patients to know, understand, and be able to implement an adequate nutritional management. Currently, although there are several treatments approaches, the most common has been dietary restriction of phenylalanine. Nutritional treatment includes the use of the so-called “medical formula” or “phenylalanine-free metabolic formula”, which was conceived from the first third of the 20th century. Subsequently, many studies and modifications have been performed to improve patient outcomes. This review aimed to describe the main characteristics and the differences between the metabolic follow-up formulas available in Mexico, so that health personnel have elements for their correct prescription.

Keywords : Phenylketonuria; Specialized foods; Proteins; Micronutrients.

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