Dataset Information

Current Practices and Challenges in the Diagnosis and Management of PKU in Latin America: A Multicenter Survey.

ABSTRACT: This study aimed to describe the current practices in the diagnosis and dietary management of phenylketonuria (PKU) in Latin America, as well as the main barriers to treatment. We developed a 44-item online survey aimed at health professionals. After a pilot test, the final version was sent to 25 practitioners working with inborn errors of metabolism (IEM) in 14 countries. Our results include 22 centers in 13 countries. Most countries (12/13) screened newborns for PKU. Phenylalanine (Phe) targets at different ages were very heterogeneous among centers, with greater consistency at the 0-1 year age group (14/22 sought 120-240 µmol/L) and the lowest at >12 years (10 targets reported). Most countries had only unflavored powdered amino acid substitutes (10/13) and did not have low-protein foods (8/13). Only 3/13 countries had regional databases of the Phe content of foods, and only 4/22 centers had nutrient analysis software. The perceived obstacles to treatment were: low purchasing power (62%), limited/insufficient availability of low-protein foods (60%), poor adherence, and lack of technical resources to manage the diet (50% each). We observed a heterogeneous scenario in the dietary management of PKU, and most countries experienced a lack of dietary resources for both patients and health professionals.

SUBMITTER: Poloni S

PROVIDER: S-EPMC8399454 | biostudies-literature | 2021 Jul

REPOSITORIES: biostudies-literature

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Publications

Current Practices and Challenges in the Diagnosis and Management of PKU in Latin America: A Multicenter Survey.

Poloni Soraia S Dos Santos Bruna Bento BB Chiesa Ana A Specola Norma N Pereyra Marcela M Saborío-Rocafort Manuel M Salazar María Florencia MF Leal-Witt María Jesús MJ Castro Gabriela G Peñaloza Felipe F Wong Sunling Palma SP Porras Ramsés Badilla RB Paranza Lourdes Ortiz LO Sanabria Marta Cristina MC Amieva Marcela Vela MV Morales Marco M Naranjo Amanda Rocío Caro ARC Mahfoud Antonieta A Colmenares Ana Rosa AR Lemes Aida A Sotillo-Lindo José Fernando JF Perez Ceila C Rey Laritza Martínez LM Torriente Georgina María Zayas GMZ Refosco Lilia Farret LF Schwartz Ida Vanessa Doederlein IVD Cornejo Veronica V

Nutrients 20210727 8

This study aimed to describe the current practices in the diagnosis and dietary management of phenylketonuria (PKU) in Latin America, as well as the main barriers to treatment. We developed a 44-item online survey aimed at health professionals. After a pilot test, the final version was sent to 25 practitioners working with inborn errors of metabolism (IEM) in 14 countries. Our results include 22 centers in 13 countries. Most countries (12/13) screened newborns for PKU. Phenylalanine (Phe) target ...[more]

PMID: 34444728

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Project description:BACKGROUND:Malnutrition in critically ill adults in the intensive care unit (ICU) is associated with a significantly elevated risk of mortality. Adequate nutrition therapy is crucial to optimise outcomes. Currently, there is a paucity of such data in Latin America. Our aims were to characterise current clinical nutrition practices in the ICU setting in Latin America and evaluate whether current practices meet caloric and protein requirements in critically ill patients receiving nutrition therapy. METHODS:We conducted a cross-sectional, retrospective, observational study in eight Latin American countries (Argentina, Brazil, Chile, Colombia, Ecuador, Mexico, Panama, and Peru). Eligible patients were critically ill adults hospitalised in the ICU and receiving enteral nutrition (EN) and/or parenteral nutrition (PN) on the Screening Day and the previous day (day -1). Caloric and protein balance on day -1, nutritional status, and prescribed nutrition therapy were recorded. Multivariable logistic regression analysis was performed to identify independent predictors of reaching daily caloric and protein targets. RESULTS:The analysis included 1053 patients from 116 hospitals. Evaluation of nutritional status showed that 74.1% of patients had suspected/moderate or severe malnutrition according to the Subjective Global Assessment. Prescribed nutrition therapy included EN alone (79.9%), PN alone (9.4%), and EN?+?PN (10.7%). Caloric intake met >90% of the daily target in 59.7% of patients on day -1; a caloric deficit was present in 40.3%, with a mean (±SD) daily caloric deficit of -688.8?±?455.2 kcal. Multivariable logistic regression analysis showed that combined administration of EN?+?PN was associated with a statistically significant increase in the probability of meeting >90% of daily caloric and protein targets compared with EN alone (odds ratio, 1.56; 95% confidence interval, 1.02-2.39; p?=?0.038). CONCLUSIONS:In the ICU setting in Latin America, malnutrition was highly prevalent and caloric intake failed to meet targeted energy delivery in 40% of critically ill adults receiving nutrition therapy. Supplemental administration of PN was associated with improved energy and protein delivery; however, PN use was low. Collectively, these findings suggest an opportunity for more effective utilisation of supplemental PN in critically ill adults who fail to receive adequate nutrition from EN alone.

Dataset Information

Current Practices and Challenges in the Diagnosis and Management of PKU in Latin America: A Multicenter Survey.

Publications

Current Practices and Challenges in the Diagnosis and Management of PKU in Latin America: A Multicenter Survey.

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