Supplementation for Performance and Health in Patients with Phenylketonuria: An Exercise-Based Approach to Improving Dietary Adherence.

Supplementation is crucial for improving performance and health in phenylketonuria (PKU) patients, who face dietary challenges. Proteins are vital for athletes, supporting muscle growth, minimizing catabolism, and aiding muscle repair and glycogen replenishment post-exercise. However, PKU individuals must limit phenylalanine (Phe) intake, requiring supplementation with Phe-free amino acids or glycomacropeptides. Tailored to meet nutritional needs, these substitutes lack Phe but fulfill protein requirements. Due to limited supplement availability, athletes with PKU may need higher protein intake. Various factors affect tolerated Phe levels, including supplement quantity and age. Adhering to supplement regimens optimizes performance and addresses PKU challenges. Strategically-timed protein substitutes can safely enhance muscle synthesis and sports performance. Individualized intake is essential for optimal outcomes, recognizing proteins' multifaceted role. Here, we explore protein substitute supplementation in PKU patients within the context of physical activity, considering limited evidence.

Infectious complications in home parenteral nutrition: A long-term study with peripherally inserted central catheters, tunneled catheters, and ports.

OBJECTIVE: Home parenteral nutrition (HPN) has become a common therapy, with tunneled central venous catheters (CVCs) being the preferred route of administration. Peripherally inserted central catheters (PICCs) have been used increasingly, but whether they should be preferred over other types of CVCs is still controversial. The aim of this study was to evaluate catheter-related complications of CVC in patients receiving HPN. METHODS: All patients treated at our center for HPN from 2007 to 2017 were prospectively included. A specialized intravenous therapy team took care of these patients. Catheter-related bloodstream infections (CRBSI) were confirmed with positive, simultaneous, differential blood cultures drawn through the CVC and peripheral vein and then semiquantitative or quantitative culture of the catheter tip. RESULTS: In all, 151 patients received HPN during the 11-y study period. Of these patients, 95 were women (63%) and 55 were men (37%), with a mean age of 58 ± 13 y. Twenty-six were non-cancer patients (17%) and the remaining 125 patients had an underlying malignancy (83%). Regarding the CVC, 116 were PICCs, 18 Hickman, and 36 ports. Confirmed CRBSI per catheter-days showed 0.15 episodes per 1000 catheter-days for PICCs, 0.72 for Hickman, and 2.02 for ports. PICCs had less-confirmed CRBSIs per 1000 catheter-days than ports (φ = 0.54, P = 0.005), but no difference between PICCs and Hickman was found (φ = 0.32, P = 0.110). Confirmed episodes of CRBSI (2 versus 13%, χ2 = 6.625, P = 0.036) were more frequent with multilumen catheters. CONCLUSIONS: In our setting, single-lumen PICC and Hickman catheters showed low infectious complications.