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Protein intake and prevalence of overweight in patients with phenylketonuria: A...

Rodrigues, C; Barbosa, CS; de Almeida, MF; Bandeira, A; Martins, E; Rocha, S; Guimas, A; Ribeiro, R; Soares, A; Moreira-Rosário, A; Dias, CC

Background: Overweight has been identified as a comorbidity associated with phenylketonuria (PKU). A systematic review with meta-analysis found that although patients with PKU had a similar body mass index (BMI) when compared to non-PKU controls, there was a significantly higher BMI when measured in patients described as having classical PKU. Objective: The aim of this retrospective longitudinal study was to id...


Continuous use of glycomacropeptide in the nutritional management of patients w...

Pena, MJ; Pinto, A; de Almeida, MF; Barbosa, CD; Ramos, PC; Rocha, S; Guimas, A; Ribeiro, R; Martins, E; Bandeira, A; Dias, CC; MacDonald, A

Background In phenylketonuria (PKU), modified casein glycomacropeptide supplements (CGMP-AA) are used as an alternative to the traditional phenylalanine (Phe)-free L-amino acid supplements (L-AA). However, studies focusing on the long-term nutritional status of CGMP-AA are lacking. This retrospective study evaluated the long-term impact of CGMP-AA over a mean of 29 months in 11 patients with a mean age at CGMP-...


The Role of a Medical Intermediate Care Unit in the Management of Budd-Chiari S...

Cravo, M; Oliveira, DG; Guimas, A; Vita, P; Rego, AL; Alves, R; Valadares, D; Carvalheiras, G; Ricardo, M; Pinto, A; Pessegueiro, H; Nery, F

Budd-Chiari syndrome (BCS) has a wide spectrum of presentations, from an asymptomatic status to acute liver failure (ALF). The therapeutic approach depends on disease severity and related etiology with patients with severe forms of presentation classically managed in intensive care units (ICUs). Here, we report a series of five BCS patients managed in a medical intermediate care unit (IntCU), with three of them...


Metabolic Control in Patients With Phenylketonuria Pre- and Post-Sapropterin Lo...

Sousa Barbosa, C; Almeida, MF; Sousa, C; Rocha, S; Guimas, A; Ribeiro, R; Martins, E; Bandeira, A; Bruno M P M Oliveira; Borges, Nuno; MacDonald, A


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