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With the advent of the Internet of Things, billions of objects or devices are inserted into the global computer network, generating and processing data at a volume never imagined before. This paper proposes a way to collect and process local data through a data fusion technology called summarization. The main feature of the proposal is the local data fusion, through parameters provided by the application, ensuring the quality of data collected by the sensor node. In the evaluation, the sensor node was compared when performing the data summary with another that performed a continuous recording of the collected data. Two sets of nodes were created, one with a sensor node that analyzed the luminosity of the room, which in this case obtained a reduction of 97% in the volume of data generated, and another set that analyzed the temperature of the room, obtaining a reduction of 80% in the data volume. Through these tests, it has been proven that the local data fusion at the node can be used to reduce the volume of data generated, consequently decreasing the volume of messages generated by IoT environments.

Introduction Maple Syrup Urine Disease (MSUD) is a rare metabolic disorder requiring lifelong restriction of branched-chain amino acids (BCAAs), and targeted supplementation with valine and isoleucine to maintain metabolic stability. Methods A prospective, open-label, observational study was conducted over 56 days in 5 paediatric patients mean age 8.8 years (range 3–14) with classical MSUD. In Phase 1 (Days 0–28), participants received the ready to use (RTU) liquid valine formulation, while continuing their usual powdered isoleucine supplement. In Phase 2 (Days 29–56), participants returned to powdered valine and changed to the ready to use RTU liquid isoleucine supplement. Weekly plasma BCAAs were monitored. Acceptability, gastrointestinal tolerance, adherence, and dietary intake were assessed through questionnaires and food diaries. Results RTU liquid valine and isoleucine supplements were well tolerated and accepted by all participants. Plasma leucine decreased when taking both liquid valine (mean leucine 253 µmol/L) and isoleucine (mean leucine 219 µmol/L) compared to baseline (mean leucine 356 µmol/L). Required doses of valine and isoleucine decreased by 30% and 18%, respectively, suggesting improved adherence or enhanced efficacy. Palatability and ease of use were rated more favourably for the RTU products, with no adverse effects or adherence issues reported. Dietary intake remained stable throughout the study. Discussion These preliminary findings suggest that RTU valine and isoleucine formulations may offer potential advantages in MSUD management, simplifying administration, and enhancing user satisfaction. While limited by sample size and short-term evaluation, their practicality and patient-centered design warrant further investigation in larger, more diverse populations to establish long-term efficacy and broader applicability.

Background Effective management of phenylketonuria (PKU) requires consistent blood phenylalanine (Phe) control. However, patients, particularly those with classical PKU, often experience diurnal fluctuations, particularly with early morning Phe levels after an overnight fast. These fluctuations have been linked to neurocognitive and psychological impairments. This 4-week, randomized, controlled crossover study evaluated the efficacy of a prolonged-release amino acid (PR-AA) compared to a conventional free amino acid-based protein substitute (AA) in reducing early morning blood Phe levels in patients with classical PKU. Methods Participants on a low-Phe diet supplemented with protein substitutes were randomly assigned to one of two treatment arms: Arm A, participants received the PR-AA as a single evening dose for 7 days, followed by a 2-week washout period, then crossed over to receive their usual AA protein substitute for all daily doses for another 7 days. Arm B, the order was reversed. All other daily protein substitute doses remained unchanged, using the patients’ usual Phe-free L-AA protein substitute throughout the study. Blood Phe and tyrosine (Tyr) levels, Phe/Tyr ratio, branched-chain amino acids (BCAAs), urinary nitrogen and creatinine excretion, tolerability, and patient acceptability were assessed. Results From 16 eligible patients, 13 children with classical PKU ( n= 8 females, 62%) with a mean age of 11.8 ± 3.2 years completed the study. Administration of the PR-AA significantly reduced early morning Phe levels compared to AA (mean difference from baseline to the end of treatment with PR-AA: 63.6 ± 28.93 µmol/L; −17.8%; p =  0.0484; with AA: 95.5 ± 28.9 µmol/L; +27.6%; p =  0.0063). Subgroup analyses indicated greater benefit in patients < 12 years and in those with higher baseline Phe level (>360 μmol/L). PR-AA also significantly increased blood Tyr levels, and improved the Phe/Tyr ratio. No significant differences were observed for other parameters. PR-AA was well tolerated, with high adherence. Conclusions PR-AA demonstrated improved metabolic control, particularly in reducing early morning Phe levels. PR-AA may be a useful alternative protein substitute for patients with classical PKU, particularly those with poor metabolic control. Further long-term studies are warranted to assess sustained efficacy, adherence, and neurocognitive outcomes.

The goal of this work is to present a systematic literature mapping (SLM) identifying algorithms for the search for data, determining the best path and types of communication between the local server and the drone, as well as possible simulators to validate proposed solutions. The concept, here considered as IoT Off-Grid, is characterized by being an environment without commercial electrical infrastructure and without communication connected to the internet. IoT equipment generates data to be stored on a local server. It collects these data through a drone that searches each local server for later integration with the commercial internet environment. As a result, we have algorithms to determine the best path based on the TSP—travelling salesman problem. Different types of communication between the drone and the server contain the data, predominantly WiFi 802.11. As a simulator, OMNeT++ stands out.

Several market sectors are attracted by the potential of unmanned aerial vehicles (UAVs), such as delivery, agriculture, and cinema, among others. UAVs are becoming part of Internet of Things (IoT) networks in the development of autonomous and scalable solutions. However, these vehicles are gradually becoming attractive targets for cyberattacks. This study proposes the development of an efficient platform based on the Message Queuing Telemetry Transport (MQTT) protocol for UAV control and Denial-of-Service (DoS) detection embedded in the UAV system. For the efficiency test, latency, network and memory consumption on the platform were measured, in addition to the correlation between payload and delay time. The results of efficiency tests were collected for the three levels of quality of service (QoS). A strong correlation greater than 90% was found between delay and data size for all QoS levels, showing almost a linear proportion. In DoS detection, the best results were a true positive rate (TPR) of 0.97 with 16 features from the AWID2 dataset using LightGBM with Bayesian optimization and data balancing. Unlike other studies, the built platform shows efficiency for UAV control and guarantees security in the communication with the broker and in the Wi-Fi UAV network.

Background In phenylketonuria (PKU), attending multidisciplinary clinic reviews is an important aspect of life-long care. Since the COVID-19 pandemic, video and telephone clinics are used as alternative methods for people with PKU to have contact with their care team. There is limited research concerning patient preference, experience and perceptions of alternative types of clinic review. Individuals from the UK with PKU and their caregivers were invited to complete an online questionnaire, hosted on the National Society for PKU (NSPKU) website and social media platform. Results Data was available from 203 respondents. Forty one per cent of respondents ( n  = 49/119) preferred in-person clinics; 41% ( n  = 49) a hybrid of in-person, video and telephone clinics; 9% ( n  = 11) video clinics only, 6% ( n  = 7) telephone only and 3% ( n =  3) were unsure. The main respondent obstacles to in-person clinics were costs, travel and time, but this was balanced by the benefits of a physical examination and better patient engagement/motivation. Twenty one per cent ( n  = 36/169) of respondents were uncomfortable with the number of healthcare professionals (HCPs) in a clinic room. Patients were less likely to consult with a doctor on video (64%, n  = 91/143) or phone (50%, n =  59/119) reviews compared to in-person (80%, n  = 146/183). Issues with video and telephone reviews included the shorter time length of review, distractions, technical issues and poor patient engagement. Conclusions Online video and telephone clinic platforms were effective in overcoming the challenging circumstances in management, monitoring and treatment of patients with PKU during the COVID-19 pandemic. However, in-person clinics remain the preferred respondent option. It is important that HCPs are flexible, enabling people with PKU a choice of clinic options according to their individual clinical need and circumstances.

Background Phenylalanine-free infant formula is an essential source of safe protein in a phenylalanine restricted diet, but its efficacy is rarely studied. We report a multicentre, open, longitudinal, prospective intervention study on a phenylalanine-free infant formula (PKU Start: Vitaflo International Ltd.). Results This was a 2-part study: part I (28 days short term evaluation) and part II (12 months extension). Data was collected on infant blood phenylalanine concentrations, dietary intake, growth, and gastrointestinal tolerance. Ten infants ( n  = 8 males, 80%), with a median age of 14 weeks (range 4–36 weeks) were recruited from 3 treatment centres in the UK. Nine of ten infants completed the 28-day follow-up (one caregiver preferred the usual phenylalanine-free formula and discontinued the study formula after day 14) and 7/9 participated in study part II. The phenylalanine-free infant formula contributed a median of 57% (IQR 50–62%) energy and 53% (IQR 33–66%) of total protein intake from baseline to the end of the part II extension study. During the 12-month follow-up, infants maintained normal growth and satisfactory blood phenylalanine control. Any early gastrointestinal symptoms (constipation, colic, vomiting and poor feeding) improved with time. Conclusion The study formula was well tolerated, helped maintain good metabolic control, and normal growth in infants with PKU. The long-term efficacy of phenylalanine-free infant formula should continue to be observed and monitored.

IoT encompasses various objects, technologies, communication standards, sensors, actuators in powered environments, and networked communication. The concept adopted here, IoT off-grid, considers an environment without commercial electricity and commercial internet. Managing various utilities with IoT and collecting the relevant information from this environment is the purpose of this project. It uses machine learning to select relevant data. These data are collected safely using a drone that travels through the off-grid stations. A systematic literature mapping is presented, identifying the state of the art. The result is a software architecture proposal with configurations in the drone and off-grid stations that contemplate data collection from the IoT off-grid environment. The results are also presented with different selection algorithms used in machine learning and final execution in the prototype.

Background In children with phenylketonuria (PKU), transitioning protein substitutes at the appropriate developmental age is essential to help with their long-term acceptance and ease of administration. We assessed the parental experiences in transitioning from a second stage to third stage liquid or powdered protein substitute in patients with PKU. Results Sixteen interviews (23 open-ended questions) were carried out with parents/caregivers of children with PKU (8 females, 50%) with a median age of 8 years (range 5–11 years), continuously treated with diet, and on a third stage protein substitute. Parents/caregivers identified common facilitators and barriers during the third stage protein substitute transition process. The main facilitators were: child and parent motivation, parent knowledge of the transition process, a role model with PKU, low volume and easy preparation of the third stage protein substitute (liquid/powder), anticipation of increasing child independence, lower parent workload, attractive packaging, better taste and smell, school and teacher support, dietetic plans and guidance, PKU social events, child educational materials and written resources. The main barriers were child aversion to new protein substitutes, poor child behaviour, child aged > 5 years, parental fear of change, the necessity for  parental time and persistence, loss of parental control, high product volume, different taste, smell, and texture of new protein substitutes, and peer bullying. Conclusion A stepwise, supportive approach is necessary when transitioning from second to third stage protein substitutes in PKU. Future studies are needed to develop guidance to assist parents/caregivers, health professionals, and teachers during the transition process.

Protein is the most satiating macronutrient, increasing secretion of gastrointestinal hormones and diet induced thermogenesis. In phenylketonuria (PKU), natural protein is restricted with approximately 80% of intake supplied by a synthetic protein source, which may alter satiety response. Casein glycomacropeptide (CGMP-AA), a carbohydrate containing peptide and alternative protein substitute to amino acids (AA), may enhance satiety mediated by its bioactive properties. Aim: In a three-year longitudinal; prospective study, the effect of AA and two different amounts of CGMP-AA (CGMP-AA only (CGMP100) and a combination of CGMP-AA and AA (CGMP50) on satiety, weight and body mass index (BMI) were compared. Methods: 48 children with PKU completed the study. Median ages of children were: CGMP100; (n = 13), 9.2 years; CGMP50; (n = 16), 7.3 years; and AA (n = 19), 11.1 years. Semi-quantitative dietary assessments and anthropometry (weight, height and BMI) were measured every three months. Results: The macronutrient contribution to total energy intake from protein, carbohydrate and fat was similar across the groups. Adjusting for age and gender, no differences in energy intake, weight, BMI, incidence of overweight or obesity was apparent between the groups. Conclusion: In this three-year longitudinal study, there was no indication to support a relationship between CGMP and satiety, as evidenced by decreased energy intake, thereby preventing overweight or obesity. Satiety is a complex multi-system process that is not fully understood.