Clinical performance of the iPREDICTLIVING tool for the prediction of the post-transplant recipient and living donor outcomes in a European cohort.

A living donor kidney transplant (LDKT) is the best treatment for ESRD. A prediction tool based on clinical and demographic data available pre-KT was developed in a Norwegian cohort with three different models to predict graft loss, recipient death, and donor candidate's risk of death, the iPREDICTLIVING tool. No external validations are yet available. We sought to evaluate its predictive performance in our cohort of 352 pairs LKDT submitted to KT from 1998 to 2019. The model for censored graft failure (CGF) showed the worse discriminative performance with Harrell's C of .665 and a time-dependent AUC of .566, with a calibration slope of .998. For recipient death, at 10 years, the model had a Harrell's C of .776, a time-dependent AUC of .773, and a calibration slope of 1.003. The models for donor death were reasonably discriminative, although with a poor calibration, particularly for 20 years of death, with a Harrell's C of .712 and AUC of .694 with a calibration slope of .955. These models have moderate discriminative and calibration performance in our population. The tool was validated in this Northern Portuguese cohort, Caucasian, with a low incidence of diabetes and other comorbidities. It can improve the informed decision-making process at the living donor consultation joining clinical and other relevant information.

Surgical complications and technical failure of simultaneous pancreas-kidney transplantation: A 22-year experience from a single center.

Simultaneous pancreas-kidney transplantation (SPKT) is the best treatment for selected individuals with type 1 diabetes mellitus and end-stage renal disease. Despite advances in surgical techniques, donor and recipient selection, and immunosuppressive therapies, SPKT remains a complex procedure with associated surgical complications and adverse consequences. We conducted a retrospective study that included 263 SPKT procedures performed between May 2000, and December 2022. A total of 65 patients (25%) required at least one relaparotomy, resulting in an all-cause relaparotomy rate of 2.04 events per 100 in-hospital days. Lower donor body mass index was identified as an independent factor associated with reoperation (OR .815; 95% CI:  .725-.917, p = .001). Technical failure (TF) occurred in 9.9% of cases, primarily attributed to pancreas graft thrombosis, intra-abdominal infections, bleeding, and anastomotic leaks. Independent predictors of TF at 90 days included donor age above 36 years (HR 2.513; 95% CI 1.162-5.434), previous peritoneal dialysis (HR 2.503; 95% CI 1.149-5.451), and specific pancreas graft reinterventions. The findings highlight the importance of carefully considering donor and recipient factors in SPKT. The incidence of TF in our study population aligns with the recent series. Continuous efforts should focus on identifying and mitigating potential risk factors to enhance SPKT outcomes, thereby reducing post-transplant complications.

Longitudinal Trajectories of Estimated Glomerular Filtration Rate in a European Population of Living Kidney Donors.

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 a mean 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.

Smartphone-based inertial measurements during Chester step test as a predictor of length of hospital stay in abdominopelvic cancer postoperative period: a prospective cohort study.

BACKGROUND: Objective assessment of pre-operative functional capacity in cancer patients using the smartphone gyroscope during the Chester step (CST) test may allow greater sensitivity of test results. This study has investigated whether the CST is a postoperative hospital permanence predictor in cancer patients undergoing abdominopelvic surgery through work, VO2MAX and gyroscopic movement analysis. METHODS: Prospective, quantitative, descriptive and inferential observational cohort study. Fifty-one patients were evaluated using CST in conjunction with a smartphone gyroscope. Multivariate linear regression analysis was used to examine the predictive value of the CST. RESULTS: The duration of hospital permanence 30 days after surgery was longer when patients who performed stage 1 showed lower RMS amplitude and higher peak power. The work increased as the test progressed in stage 3. High VO2MAX seemed to be a predictor of hospital permanence in those who completed levels 3 and 4 of the test. CONCLUSION: The use of the gyroscope was more accurate in detecting mobility changes, which predicted a less favorable result for those who met at level 1 of the CST. VO2MAX was a predictor of prolonged hospitalization from level 3 of the test. The work was less accurate to determine the patient's true functional capacity.

Predicting factors of neurodevelopmental performance in children with phenylketonuria.

In phenylketonuria (PKU), high phenylalanine (Phe) levels hamper neurodevelopment impairing executive function later in life. While the second has been more studied, fewer data exist on predictors of PKU patients' development in specific populations. To contribute to the field, we performed a retrospective analysis of predictors of neurodevelopment in PKU patients in a Portuguese cohort. We analyzed the retrospective data on the metabolic control of 89 patients, as their health and familial features. Griffith's Mental Development Scale performance at age 6 (GMDS6) was used to assess neurodevelopment. Our cohort included 14 GMDS6low and 75 GMDS6high patients. In a multivariate analysis, the better predictors of neurodevelopment were the metabolic control at age 3 and year of birth (n = 87, ß0  = -121, ß1  = -1.77, ß2  = 0.06, LRchi2(2) = 13.61, Prob > chi2 = 0.001, Pseudo R2 = 0.1773). With this model, it was possible to define a safety cut-off of 7.8 mg/dL for the Phe level at age 3 (sensitivity = 72.6%, specificity = 78.6%), confirming the safety of the cut-off of 6 mg/dL already used in the clinical practice. Our study supports the relevance of metabolic control to predict the neurodevelopment of PKU patients, in the historical context of the disease management.

External Validation of the Toulouse-Rangueil Predictive Model to Estimate Donor Renal Function After Living Donor Nephrectomy.

A predictive model to estimate post-donation glomerular filtration rate (eGFR) and risk of CKD at 1-year was developed from a Toulouse-Rangueil cohort in 2017 and showed an excellent correlation to the observed 1-year post-donation eGFR. We retrospectively analyzed all living donor kidney transplants performed at a single center from 1998 to 2020. Observed eGFR using CKD-EPI formula at 1-year post-donation was compared to the predicted eGFR using the formula eGFR (CKD-EPI, mL/min/1.73 m2) = 31.71+ (0.521 × preoperative eGFR) - (0.314 × age). 333 donors were evaluated. A good correlation (Pearson r = 0.67; p < 0.001) and concordance (Bland-Altman plot with 95% limits of agreement -21.41-26.47 mL/min/1.73 m2; p < 0.001) between predicted and observed 1-year post-donation eGFR were observed. The area under the ROC curve showed a good discriminative ability of the formula in predicting observed CKD at 1-year post-donation (AUC = 0.83; 95% CI: 0.78-0.88; p < 0.001) with optimal cutoff corresponding to a predicted eGFR of 65.25 mL/min/1.73 m2 in which the sensibility and specificity to predict CKD were respectively 77% and 75%. The model was successfully validated in our cohort, a different European population. It represents a simple and accurate tool to assist in evaluating potential donors.

Excess Mortality in Kidney and Kidney-Pancreas Transplant Recipients in the COVID-19 Pandemic in Portugal-A Cohort Study.

The COVID-19 pandemic increased morbidity and mortality worldwide, particularly in the Kidney and Kidney-Pancreas Transplant Recipient (KTR/KPTR) population. Aiming at assessing the absolute and relative excess mortality (EM) in a Portuguese KTR/KPTR cohort, we conducted a retrospective observational study of two KTR/KPTRs cohorts: cohort 1 (P1; n = 2,179) between September/2012 and March/2020; cohort 2 (P2; n = 2067) between March/2020, and August/2022. A correlation between relative and absolute EM and age, sex, time from transplantation and cause of death was explored. A total of 145 and 84 deaths by all causes were observed in P1 and P2, respectively. The absolute EM in P2 versus P1 was 19.2 deaths (observed/expected mortality ratio 1.30, p = 0.006), and the relative EM was 1.47/1,000 person-months (95% CI 1.11-1.93, p = 0.006). Compared to the same period in the general population, the standardized mortality rate by age in P2 was 3.86 (95% CI 2.40-5.31), with a peak at 9.00 (95% CI 4.84-13.16) in P2C. The higher EM identified in this population was associated, mainly, with COVID-19 infection, with much higher values during the second seasonal COVID-19 peak when compared to the general population, despite generalized vaccination. These highlight the need for further preventive measures and improved therapies in these patients.

SARS-CoV-2 infection in kidney transplant recipients: Early report of five cases.

From December 2019 to March 2020, China was the epicenter of the SARS-CoV-2 infection pandemic, but from that moment on, Europe surpassed China in the number of new cases and deaths related to this novel viral respiratory infection. The emergence of this world pandemic is particularly important for solid organ transplant recipients, who might have an increased risk of mortality, not only due to their chronic immunosuppression status, but also to the cardiovascular risk that correlates with several years of chronic kidney disease. To the extent that there is still a lack of knowledge about the clinical characteristics, evolution, and prognosis of SARS-CoV-2 infection in kidney transplant recipients, we will report the first 5 cases diagnosed and followed in our transplant unit, as well as share the therapeutic strategies adopted.

HLA class II eplet mismatch load improves prediction of dnDSA development after living donor kidney transplantation.

HLA donor-specific antibodies developed de novo after transplant remain a major cause of chronic allograft dysfunction. Our study main purpose was to determine whether HLA MM, assessed traditionally and by HLA total and AbVer eplet mismatch load (EptMM and EpvMM) assessed with HLAMatchMaker, had impact on dnDSA development after living donor kidney transplantation (LDKT). We retrospectively analysed a cohort of 96 LDKT between 2008 and 2017 performed in Hospital Santo António. Seven patients developed dnDSA-II and EpvMM and EptMM were greater in dnDSA-II group compared to the no dnDSA-II (18.0 ± 8.7 versus 9.9 ± 7.9, p = .041 and 41.3 ± 18.9 versus 23.1 ± 16.7, p = .018), which is not observed for AgMM (2.29 versus 1.56; p = .09). In a multivariate analysis, we found that preformed DSA (HR = 7.983; p = .023), living unrelated donors (HR = 8.052; p = .024) and retransplantation (HR = 14.393; p = .009) were predictors for dnDSA-II (AUC = 0.801; 0.622-0.981). HLA-II EpvMM (HR = 1.105; p = .028; AUC = 0.856) showed to be a superior predictor of dnDSA-II, when compared to AgMM (HR = 1.740; p = .113; AUC = 0.783), when adjusted for these clinical variables. Graft survival was significantly lower within dnDSA-II patient group (36% versus 88%, p < .001). HLA molecular mismatch analysis is extremely important to minimize risk for HLA-II dnDSA development improving outcome and increasing chance of retransplant lowering allosensitization.

Lower donated kidney volume is associated with increased risk of lower graft function and acute rejection at 1 year after living donor kidney-a retrospective study.

Kidney volume has been proven to be a surrogate marker of nephron mass and renal function. We studied 190 donor and recipient pairs undergoing living donor kidney transplantation at our institution during 9 years. Different metrics of donor kidney volume (DKV) were explored: alone or indexed to recipient's anthropometry, as body surface area (BSA). DKV/BSA (min. 49.7; P33rd 77.7; P67th 95.3; max. 176 cm3 /m2 ) was chosen given its higher correlation with eGFR at 1 year, and recipients were divided according to its tertiles (T). The eGFR at 1 year was lower in T1, when compared with T2 (P = 0.015) and T3 (P < 0.001). In a multivariable model, a regression spline revealed that a DKV/BSA lower than 80 was significantly associated with an eGFR at 1 year <60. In the first 6 years, the overall annual eGFR slope was -0.90 ml/min/year. Acute rejection occurred in 19%, 11%, and 0% of patients in T1, T2, and T3, respectively (P < 0.001). DKV/BSA increased stepwise from cellular- (n = 12) to antibody-mediated (n = 7) AR cases and to those without AR (n = 171; P = 0.002; no AR versus cellular AR). Lower DKV/BSA ratio was associated with significantly worse graft function and higher incidence of AR. Hence, it can be a tool for better selection of donors in order to improve graft outcomes, particularly in the setting of multiple potential living donors or kidney paired exchange programs.

Remaining kidney volume indexed to weight as a strong predictor of estimated glomerular filtration rate at 1 year and mid-term renal function after living-donor nephrectomy - a retrospective observational study.

The donors' estimated glomerular filtration rate (eGFR) after living nephrectomy has been a concern, particularly in donors with smaller kindeys. Therefore, we developed this retrospective observational study in 195 donors to determine the ability remaining kidney volume indexed to weight (RKV/W) to predict eGFR at 1 year through multivariate linear regression and to explore this relationship between annual eGFR change from 1 to 4 years postdonation evaluated by a linear mixed model. Comparing RKV/W tertiles (T1, T2, T3), RKV/W was a good predictor of 1-year eGFR which was significantly better in T3 donors. Gender, predonation eGFR, and RKV/W were independent predictors of eGFR at 1-year. In a subgroup with predonation eGFR < 90mL/min/1.73 m2 , a significant prediction of eGFR < 60mL/min/1.73 m2 was detected in males with RKV/W ≤ 2.51cm3 /kg. Annual eGFR (ml/min/year) change from 1 to 4 years was + 0.77. RKV/W divided by tertiles (T1-T3) was the only significant predictor: T2 and T3 donors had an annual eGFR improvement opposing to T1. RKV/W was a good predictor of eGFR at 1 year, independently from predonation eGFR. A higher RKV/W was associated with improved eGFR at 1 year. A decline in eGFR on the four years after surgery was only noticeable in donors with RKV/W ≤ 2.13cm3 /kg.

Pancreas-Kidney transplantation: Impact of dialysis modality on the outcome.

It remains controversial whether dialysis modality prior to SPKT (simultaneous pancreas-kidney transplantation) affects the outcome. We analyzed outcomes in type 1 diabetic patients undergoing SPKT, comparing peritoneal dialysis (PD) and hemodialysis (HD) groups: 119 had been on HD; 39 on PD. They were comparable except regarding dialysis time, higher in HD patients (30 ± 23 vs. 21 ± 15 months, P = 0.003). Thrombosis-driven relaparotomy was more frequent in PD patients (12.8% vs. 1.7%, P = 0.014). Pancreas loss due to infection was higher in PD patients (12.8% vs. 3.4%, P = 0.042). Thrombosis-related kidney loss was more frequent in PD patients (5.1%, vs. 0% in HD patients, P = 0.058). Thirteen deaths occurred, more within the PD group (17.9% vs. 5%; P = 0.011), being infection the leading cause (13.5%, vs. 1.7% in HD patients, P = 0.010). Patient survival was inferior in PD patients. Besides PD, cardiovascular disease and graft failure were independent predictors of patient death. In conclusion, PD patients more frequently complicated with intra-abominal infection leading to pancreatic loss and with renal thrombosis, with adverse impact on survival. As a PD first strategy in end-stage renal disease patients is generally associated with good outcomes, these gloomier results after SPKT urge for careful adjustment of infection and thrombosis prophylactic protocols in PD patients.

The need for scientific-area-related indicators for effective energy planning in higher education institutions.

The quest for improving energy efficiency is transversal to all areas of society. Higher education institutions represent an important sector in this quest due to their high demand, but also for the role model that they can play in educating energy-efficient citizens and piloting new approaches and experiences. Thus, decreasing energy consumption in higher education institutions, in addition to reducing the carbon footprint, contributes to ameliorating countries' energy bills, and, most importantly, contributes to a more sustainable society. The purpose of the paper, based on the energy consumption of the University of Minho, Portugal, between 2007 and 2022, is threefold: first, to evaluate how energy consumption and associated carbon footprint indicators have performed under a sustainable strategy program, second to reflect on total energy and specific energy indicators, and lastly to emphasize the need to improve energy metering and planning systems to account for the distinctive needs of the different scientific area buildings. This is not only relevant but also rare to find in scientific literature. Findings suggest that UMinho's energy consumption is in line with the numbers reported in the literature. Moreover, detailed indicators, specified by scientific area building, show diverse patterns in energy use, demonstrating the limitations of an overall analysis of buildings in the university campi. The results show that energy efficiency improved as a result of the implemented action plan, and demonstrate the need for detailed and specific indicators that reflect the different needs of each scientific area. The results provided by this refinement call for the design of tailored initiatives to decrease energy consumption, since they allow the planning of specific measures and programs for different energy use patterns, and therefore improve their efficiency. Finally, the preliminary results of the analysis of building specific energy use point to the need for more detailed data on hourly and daily consumption and academic term given the relative contribution of users' behaviour.

Spontaneous Hemoperitoneum Due to Ruptured Uterine Artery: A Rare Complication During Pregnancy.

Spontaneous rupture of the uterine artery is a rare and life-threatening cause of hemoperitoneum in pregnancy, associated with high maternal and perinatal morbidity and mortality. We present a case of a 29-year-old woman, in the 36th week of gestation, with acute abdomen due to hemoperitoneum. Ultrasound revealed free fluid in the abdominal cavity, with no signs of fetal distress, and the patient was mildly hypotensive. Exploratory laparotomy and cesarean section were performed, and extensive blood clots on the upper abdominal quadrants were discovered, as well as a bleeding left uterine artery. We expect that this case raises awareness of the ruptured uterine artery as a possible etiology of hemoperitoneum during pregnancy.

Use of Hepatitis C Virus Antibody-Positive Donors in Kidney Transplantation.

Background The use of kidney donors with hepatitis C virus (HCV) has been arising as a possibility to increase the donor pool. It encompasses both the use of donors with positive and negative viremia, particularly since the advent of direct antiviral agents that produce sustained virologic response. Methodology We conducted a retrospective observational study to describe the experience of our transplantation center in the use of HCV antibody-positive (HCV-Ab+) kidneys. Results We performed five transplants with HCV-Ab+ donors. The median age of kidney recipients was 63 (interquartile range (IQR) = 54-71) years, and 60% (n = 3) were males. Two recipients received a second transplant. The median dialysis vintage was 1,414 (IQR = 1,103-2,806) days. The induction immunosuppression protocol was basiliximab in most patients (60%, n = 3), and all received maintenance immunosuppression with tacrolimus, mycophenolate mofetil, and prednisolone. One of the recipients had a personal history of cured HCV infection. Seroconversion occurred in half of the remaining patients, which was sustained during the follow-up. None of the patients developed HCV viremia. At the end of follow-up, mean creatinine and proteinuria were 1.45 ± 1.12 mg/dL and 0.099 ± 0.045 g/g, respectively. We did not observe any rejection episodes, need for dialysis, or recipient's death. Conclusions Our work aligns with the current literature that advocates that the use of these donors is safe and cost-effective and can be an effective strategy for expanding the donor pool and augmenting the transplantation volume. Seroconversion is a known risk whose mechanisms are not entirely understood, although it does not appear to be related to a higher transmission risk.

Renal Artery Stenosis in Living Donor Kidney Transplantation: A Rare Cause of "Flash Edema".

Transplant renal artery stenosis (TRAS) is a well-recognized vascular complication after kidney transplantation, with an incidence ranging from 1% to 23%. TRAS often presents with clinical features such as refractory hypertension, de novo hypertension, allograft dysfunction, and the presence of a bruit over the graft. A rare manifestation of TRAS is flash pulmonary edema. Here, we present a case of a 37-year-old male who received a living donor kidney. Four years after the transplant, he presented with acute kidney injury, hypertensive crisis, and flash pulmonary edema. Initially, methylprednisolone pulses were administered due to suspicion of acute rejection, which was later ruled out after a kidney graft biopsy. Computed tomography angiography showed findings suggesting stenosis or thrombus in the renal artery. The patient developed sudden acute pulmonary edema, requiring hemodialysis, with notable clinical improvement. Subsequently, stent placement was performed without complications, resulting in the complete recovery of renal function and effective blood pressure control. The incidence of renal artery stenosis is higher in living donor kidney transplantation, mainly due to technical complexities during surgery. Acute presentations, such as flash edema, are exceptionally rare but can occur years after transplantation. Prompt intervention can lead to favorable outcomes.

Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe: The Impact of Age and Phenylketonuria Severity.

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.

Blood Phenylalanine Levels in Patients with Phenylketonuria from Europe between 2012 and 2018: Is It a Changing Landscape?

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.

Early dietary treated patients with phenylketonuria can achieve normal growth and body composition.

BACKGROUND: In the past, overtreatment may have resulted in growth impairment in patients with phenylketonuria. OBJECTIVE: The paper aims to investigate height and body composition in early treated patients with phenylketonuria who were diagnosed between 1981 and 2008. DESIGN: A cross-sectional study of 89 patients with phenylketonuria and 78 controls aged (mean ± SD, in years) 14.4 ± 6.6 and 15.9 ± 7.1, respectively, was undertaken, including anthropometric and body composition evaluation using bioelectrical impedance. Median Phe concentrations in the last year before study enrollment were used as a measure of metabolic control. Natural protein and amino acid mixture intakes were recorded in patients. RESULTS: No statistically significant differences were found on height z-scores between patients and controls aged less than 19 years (p=0.301), although all patients with classical phenylketonuria revealed negative height z-scores, resulting in a mean ± SD of -0.65 ± 0.41. Among participants aged 19 years or more, median (p25-p75) of height was significantly higher in controls [168.0 cm (159.2-174.8)] than in patients [160.5 cm (151.9-167.5)] (p=0.017). No significant differences were found between patients and controls regarding fat mass, fat free mass, muscular mass, body cell mass index and phase angle. CONCLUSION: Our results suggest that early and continuously treated patients with phenylketonuria born after 1992 can achieve normal growth and body composition, although the negative height z-score in patients with classical phenylketonuria strengthens the continuous need to optimize the quality of their protein intake.

Influence of 3D Microstructure Pattern and Infill Density on the Mechanical and Thermal Properties of PET-G Filaments.

This study aims to evaluate the thermal and mechanical performances of PET-G thermoplastics with different 3D microstructure patterns and infill densities. The production costs were also estimated to identify the most cost-effective solution. A total of 12 infill patterns were analysed, including Gyroid, Grid, Hilbert curve, Line, Rectilinear, Stars, Triangles, 3D Honeycomb, Honeycomb, Concentric, Cubic, and Octagram spiral with a fixed infill density of 25%. Different infill densities ranging from 5% to 20% were also tested to determine the best geometries. Thermal tests were conducted in a hotbox test chamber and mechanical properties were evaluated using a series of three-point bending tests. The study used printing parameters to meet the construction sector's specific needs, including a larger nozzle diameter and printing speed. The internal microstructures led to variations of up to 70% in thermal performance and up to 300% in mechanical performance. For each geometry, the mechanical and thermal performance was highly correlated with the infill pattern, where higher infill improved thermal and mechanical performances. The economic performance showed that, in most cases, except for the Honeycomb and 3D Honeycomb, there were no significant cost differences between infill geometries. These findings can provide valuable insights for selecting the optimal 3D printing parameters in the construction industry.