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- Wearable artificial kidney and wearable ultrafiltration device vascular access-future directionsPublication . Castro, Ana; Neri, M.; Nayak Karopadi, A.; Lorenzin, A.; Marchionna, N.; Ronco, C.Background: Since 2005, three human clinical trials have been performed with the Wearable Artificial Kidney (WAK) and Wearable Ultrafiltration (WUF) device. The lack of an adequate vascular access (VA) has been pointed out as the main limitation to their implementation. Based on the current level of understanding, we will make the first conceptual proposal of an adequate VA suitable for the WAK and the WUF. Methods: All the literature related to WAK and WUF was reviewed. Based on eight main publications the VA major characteristics were defined: a mean blood flow of 100 mL/min; the capability to allow prolonged and frequent dialysis treatments, without interfering in activities of daily living (ADL); safe and convenient connection/disconnection systems; reduced risk of biofilm formation and coagulation; high biocompatibility. A research was done in order to answer to each necessary technological prerequisites. Results: The use of a device similar to a CVC with a 5Fr lumen, seems to be the most feasible option. Totally subcutaneous port devices, like the LifeSite(R) or Dialock (R) systems can be a solution to allow WAK or WUF to operate continuously while patients carry out their ADL. Recently, macromolecules that reduce the risk of thrombosis and infection and are integrated into a CVC have been developed and have the capability of overcoming these major limitations. Conclusion: With an adequate VA, portable HD devices can be acceptable options to address several unmet clinical needs of HD patients.
- Over Restriction of Dietary Protein Allowance: The Importance of Ongoing Reassessment of Natural Protein Tolerance in PhenylketonuriaPublication . Pinto, Alex; Almeida, Manuela Ferreira; MacDonald, Anita; Ramos, Paula Cristina; Rocha, Sara; Guimas, Arlindo; Ribeiro, Rosa; Martins, Esmeralda; Bandeira, Anabela; Jackson, Richard; van Spronsen, Francjan; Payne, Anne; Rocha, Júlio CésarPhenylalanine (Phe) tolerance is highly variable in phenylketonuria (PKU) and rarely described in patients aged ≥12 years. Patients ≥12 years of age with PKU were systematically challenged with additional natural protein (NP) if blood Phe levels remained below 480 µmol/L (i.e., upper target blood Phe level for patients aged ≥12 years using Portuguese PKU guidelines). In PKU patients, NP tolerance was calculated at baseline and a median of 6 months after systematic challenge with NP whilst patients were maintaining a blood Phe ≤480 μmol/L. Anthropometry was assessed at both times. Routine blood Phe levels were collected. We studied 40 well-controlled PKU patients (10 hyperphenylalaninemia (HPA), 23 mild and 7 classic PKU), on a low-Phe diet with a mean age of 17 years (12-29 years). Median daily NP intake significantly increased between assessments (35 vs. 40 g/day, p = 0.01). Twenty-six patients (65%) were able to increase their median NP intake by a median 12 g/day (2-42 g)/day and still maintain blood Phe within target range. Out of the previous 26 patients, 20 (77%) (8 HPA, 11 mild and 1 classical PKU) increased NP from animal sources (e.g. dairy products, fish and meat) and 6 patients (23%) (3 mild and 3 classical PKU) from plant foods (bread, pasta, potatoes). Median protein equivalent intake from Phe-free/low-Phe protein substitute decreased (0.82 vs. 0.75 g/kg, p = 0.01), while median blood Phe levels remained unchanged (279 vs. 288 μmol/L, p = 0.06). Almost two-thirds of patients with PKU tolerated additional NP when challenged and still maintained blood Phe within the national target range. This suggests that some patients with PKU treated by a low-Phe diet only may over restrict their NP intake. In order to minimise the burden of treatment and optimise NP intake, it is important to challenge with additional NP at periodic intervals.