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A living donor (LD) kidney transplant is the best treatment for kidney failure, but LDs safety is paramount. We sought to evaluate our LDs cohort's longitudinal changes in estimated glomerular filtration rate (eGFR). We retrospectively studied 320 LDs submitted to nephrectomy between 1998 and 2020. The primary outcome was the eGFR change until 15 years (y) post-donation. Subgroup analysis considered distinct donor characteristics and kidney function reduction rate (%KFRR) post-donation [-(eGFR6 months(M)-eGFRpre-donation)/eGFRpre-donation*100]. Donors had amean age of 47.3 ± 10.5 years, 71% female. Overall, LDs presented an average eGFR change 6 M onward of +0.35 mL/min/1.73 m²/year. The period with the highest increase was 6 M-2 Y, with a mean eGFR change of +0.85L/min/1.73 m²/year. Recovery plateaued at 10 years. Normal weight donors presented significantly better recovery of eGFR +0.59 mL/min/1.73 m²/year, compared to obese donors -0.18L/min/ 1.73 m²/year (p = 0.020). Noteworthy, these results only hold for the first 5 years. The subgroup with a lower KFRR (<26.2%) had a significantly higher decrease in eGFR overall of -0.21 mL/min/1.73 m²/year compared to the groups with higher KFRR (p < 0.001). These differences only hold for 6 M-2 Y. Moreover, an eGFR<50 mL/min/1.73 m² was a rare event, with ≤5%prevalence in the 2-15 Y span, correlating with eGFR pre-donation. Our data show that eGFR recovery is significant and may last until 10 years post-donation. However, some subgroups presented more ominous kidney function trajectories. [ABSTRACT FROM AUTHOR]

In phenylketonuria (PKU), natural protein intake is thought to increase with age, particularly during childhood and adolescence. Longitudinal dietary intake data are scarce and lifelong phenylalanine tolerance remains unknown. Nine centres managing PKU in Europe and Turkey participated in a retrospective study. Data were collected from dietetic records between 2012 and 2018 on phenylalanine (Phe), natural protein, and protein substitute intake. A total of 1323 patients (age range: 1–57 y; 51% male) participated. Dietary intake data were available on 1163 (88%) patients. Patient numbers ranged from 59 to 320 in each centre. A total of 625 (47%) had classical PKU (cPKU), n = 357 (27%) had mild PKU (mPKU), n = 325 (25%) had hyperphenylalaninemia (HPA), and n = 16 (1%) were unknown. The mean percentage of blood Phe levels within target ranged from 65 ± 54% to 88 ± 49%. When intake was expressed as g/day, the mean Phe/natural protein and protein equivalent from protein substitute gradually increased during childhood, reaching a peak in adolescence, and then remained consistent during adulthood. When intake was expressed per kg body weight (g/kg/day), there was a decline in Phe/natural protein, protein equivalent from protein substitute, and total protein with increasing age. Overall, the mean daily intake (kg/day) was as follows: Phe, 904 mg ± 761 (22 ± 23 mg/kg/day), natural protein 19 g ± 16 (0.5 g/kg/day ± 0.5), protein equivalent from protein substitute 39 g ± 22 (1.1 g/kg/day ± 0.6), and total protein 59 g ± 21 (1.7 g/kg/day ± 0.6). Natural protein tolerance was similar between males and females. Patients with mPKU tolerated around 50% less Phe/natural protein than HPA, but 50% more than cPKU. Higher intakes of natural protein were observed in Southern Europe, with a higher prevalence of HPA and mPKU compared with patients from Northern European centres. Natural protein intake doubled with sapropterin usage. In sapropterin-responsive patients, 31% no longer used protein substitutes. Close monitoring and optimisation of protein intake prescriptions are needed, along with future guidelines specifically for different age groups and severities. [ABSTRACT FROM AUTHOR]

Background: In 2011, a European phenylketonuria (PKU) survey reported that the blood phenylalanine (Phe) levels were well controlled in early life but deteriorated with age. Other studies have shown similar results across the globe. Different target blood Phe levels have been used throughout the years, and, in 2017, the European PKU guidelines defined new targets for blood Phe levels. This study aimed to evaluate blood Phe control in patients with PKU across Europe. Methods: nine centres managing PKU in Europe and Turkey participated. Data were collected retrospectively from medical and dietetic records between 2012 and 2018 on blood Phe levels, PKU severity, and medications. Results: A total of 1323 patients (age range:1–57, 51% male) participated. Patient numbers ranged from 59 to 320 in each centre. The most common phenotype was classical PKU (n = 625, 48%), followed by mild PKU (n = 357, 27%) and hyperphenylalaninemia (HPA) (n = 325, 25%). The mean percentage of blood Phe levels within the target range ranged from 65 ± 54% to 88 ± 49% for all centres. The percentage of Phe levels within the target range declined with increasing age (<2 years: 89%; 2–5 years: 84%; 6–12 years: 73%; 13–18 years: 85%; 19–30 years: 64%; 31–40 years: 59%; and ≥41 years: 40%). The mean blood Phe levels were significantly lower and the percentage within the target range was significantly higher (p < 0.001) in patients with HPA (290 ± 325 µmol/L; 96 ± 24%) and mild PKU (365 ± 224 µmol/L; 77 ± 36%) compared to classical PKU (458 ± 350 µmol/L, 54 ± 46%). There was no difference between males and females in the mean blood Phe levels (p = 0.939), but the percentage of Phe levels within the target range was higher in females among school-age children (6–12 years; 83% in females vs. 78% in males; p = 0.005), adolescents (13–18 years; 62% in females vs. 59% in males; p = 0.034) and adults (31–40 years; 65% in females vs. 41% in males; p < 0.001 and >41 years; 43% in females vs. 28% in males; p < 0.001). Patients treated with sapropterin (n = 222) had statistically significantly lower Phe levels compared to diet-only-treated patients (mean 391 ± 334 µmol/L; percentage within target 84 ± 39% vs. 406 ± 334 µmol/L; 73 ± 41%; p < 0.001), although a blood Phe mean difference of 15 µmol/L may not be clinically relevant. An increased frequency of blood Phe monitoring was associated with better metabolic control (p < 0.05). The mean blood Phe (% Phe levels within target) from blood Phe samples collected weekly was 271 ± 204 µmol/L, (81 ± 33%); for once every 2 weeks, it was 376 ± 262 µmol/L, (78 ± 42%); for once every 4 weeks, it was 426 ± 282 µmol/L, (71 ± 50%); and less than monthly samples, it was 534 ± 468 µmol/L, (70 ± 58%). Conclusions: Overall, blood Phe control deteriorated with age. A higher frequency of blood sampling was associated with better blood Phe control with less variability. The severity of PKU and the available treatments and resources may impact the blood Phe control achieved by each treatment centre. [ABSTRACT FROM AUTHOR]

Portuguese school buildings are generally characterized by an in-service thermal discomfort, due to the poor envelope thermal properties and the lack of resources for paying energy consumption. Most Portuguese schools are free-running buildings with a natural ventilation strategy. Hundreds of high and basic schools have not been refurbished and still have problems or anomalies and inadequate in-service conditions and need to be rehabilitated in the near future. There will be, therefore, an opportunity to apply the acquired knowledge of comfort, habits, energy consumption and costs that have been studied since the last refurbishments (2008). This work studies the Portuguese Brandão schools model (from the ’70s), including about 100 non-refurbished basic schools. A prototype classroom was prepared in a Brandão school, in Porto. The in situ experimental campaign consisted of temperature, relative humidity, CO2 concentration and energy consumption measurements. The main tasks of this work are: (1) the validation of an advanced hygrothermal model with experimental measurements before and after the prototype refurbishment; (2) development of a sensitivity study in order to choose the best refurbishment and heating strategies for these buildings, regarding their typology, the local climate features and the actual capacity to support the operating costs; (3) assessment of the roof insulation thickness in Brandão schools; (4) quantification of discomfort indicators.

Portuguese school buildings are generally characterized by an in service thermal discomfort. The refurbishments that the schools have recently undergone, have not fully considered the climatic diversity and the economic reality of the country and, for that reason, there are still some discomfort in these schools. This work studies the Portuguese Brandão schools model (from the 70’s), which comprises about 100 non-refurbished basic schools. A prototype classroom was prepared in a Brandão school, in Porto. The in situ experimental campaign consisted in temperature, relative humidity, CO2 concentration and energy consumption measurements. With this prototype, some studies were carried out regarding the thermal behavior before and after a refurbishment process, by experimental monitoring. The prototype construction has included the improvement of the envelope (roof insulation and solar protection) and of the technical systems (ventilation and heating). The main goal was the assessment of the discomfort in this prototype before and after the refurbishment in free-running conditions or with some intermittent heating strategies. Discomfort indexes have been developed for the assessment of the discomfort. This work presents the prototype thermal performance in pre-existing in service conditions and the improvement in the thermal behavior of the environment after the refurbishment.