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Background and purpose: Two novel enzyme replacement therapies (ERTs), studied in phase 3 trials in late‐onset Pompe patients, reached marketing authorization by the European Medicines Agency in 2022 and 2023. The European Pompe Consortium (EPOC) updates and extends the scope of the 2017 recommendations for starting, switching and stopping ERT. Methods: The European Pompe Consortium consists of 25 neuromuscular and metabolic experts from eight European countries. This update was performed after an in‐person meeting, three rounds of discussion and voting to provide a consensus recommendation. Results: The patient should be symptomatic, that is, should have skeletal muscle weakness or respiratory muscle involvement. Muscle magnetic resonance imaging findings showing substantial fat replacement can support the decision to start in a patient‐by‐patient scenario. Limited evidence supports switching ERT if there is no indication that skeletal muscle and/or respiratory function have stabilized or improved during standard ERT of 12 months or after severe infusion‐associated reactions. Switching of ERT should be discussed on a patient‐by‐patient shared‐decision basis. If there are severe, unmanageable infusion‐associated reactions and no stabilization in skeletal muscle function during the first 2 years after starting or switching treatment, stopping ERT should be considered. After stopping ERT for inefficacy, restarting ERT can be considered. Six‐monthly European Pompe Consortium muscle function assessments are recommended. Conclusions: The triple‐S criteria on ERT start, switch and stop include muscle magnetic resonance imaging as a supportive finding and the potential option of home infusion therapy. Six‐monthly long‐term monitoring of muscle function is highly recommended to cover insights into the patient's trajectory under ERT. [ABSTRACT FROM AUTHOR]

Subsurface drainage plays a crucial role in excess water removal through perforated pipes buried in the soil. The Red River Valley of eastern North Dakota and west central Minnesota often requires pumped drainage outlets due to its flat topography, with many modern systems incorporating variable frequency drives (VFD) for improved efficiency. VFDs adjust pump speeds to match drainage needs, but this dynamic operation complicates accurate flow rate estimation for edge-of-field monitoring. This project aimed to devise an automated method for calculating flow rate estimates from VFD pumping systems involving tank geometry, operational water levels, and pump duty cycling. Linear regressions with calibration data from a transit-time ultrasonic flowmeter produced mixed outcomes. Some linear regressions revealed errors in calculated versus measured flow rate of =14%, while other regressions showed errors nearing 40%. To address this, future efforts should explore methods incorporating the pump's electrical characteristics. This approach would provide a more accurate representation of the unstable and intricate pumping operations during transient conditions. [ABSTRACT FROM AUTHOR]

Deuterium metabolic imaging (DMI) is an emerging magnetic resonance technique, for non‐invasive mapping of human brain glucose metabolism following oral or intravenous administration of deuterium‐labeled glucose. Regional differences in glucose metabolism can be observed in various brain pathologies, such as Alzheimer's disease, cancer, epilepsy or schizophrenia, but the achievable spatial resolution of conventional phase‐encoded DMI methods is limited due to prolonged acquisition times rendering submilliliter isotropic spatial resolution for dynamic whole brain DMI not feasible. The purpose of this study was to implement non‐Cartesian spatial‐spectral sampling schemes for whole‐brain 2H FID‐MR Spectroscopic Imaging to assess time‐resolved metabolic maps with sufficient spatial resolution to reliably detect metabolic differences between healthy gray and white matter regions. Results were compared with lower‐resolution DMI maps, conventionally acquired within the same session. Six healthy volunteers (4 m/2 f) were scanned for ~90 min after administration of 0.8 g/kg oral [6,6′]‐2H glucose. Time‐resolved whole brain 2H FID‐DMI maps of glucose (Glc) and glutamate + glutamine (Glx) were acquired with 0.75 and 2 mL isotropic spatial resolution using density‐weighted concentric ring trajectory (CRT) and conventional phase encoding (PE) readout, respectively, at 7 T. To minimize the effect of decreased signal‐to‐noise ratios associated with smaller voxels, low‐rank denoising of the spatiotemporal data was performed during reconstruction. Sixty‐three minutes after oral tracer uptake three‐dimensional (3D) CRT‐DMI maps featured 19% higher (p =.006) deuterium‐labeled Glc concentrations in GM (1.98 ± 0.43 mM) compared with WM (1.66 ± 0.36 mM) dominated regions, across all volunteers. Similarly, 48% higher (p =.01) 2H‐Glx concentrations were observed in GM (2.21 ± 0.44 mM) compared with WM (1.49 ± 0.20 mM). Low‐resolution PE‐DMI maps acquired 70 min after tracer uptake featured smaller regional differences between GM‐ and WM‐dominated areas for 2H‐Glc concentrations with 2.00 ± 0.35 mM and 1.71 ± 0.31 mM, respectively (+16%; p =.045), while no regional differences were observed for 2H‐Glx concentrations. In this study, we successfully implemented 3D FID‐MRSI with fast CRT encoding for dynamic whole‐brain DMI at 7 T with 2.5‐fold increased spatial resolution compared with conventional whole‐brain phase encoded (PE) DMI to visualize regional metabolic differences. The faster metabolic activity represented by 48% higher Glx concentrations was observed in GM‐ compared with WM‐dominated regions, which could not be reproduced using whole‐brain DMI with the low spatial resolution protocol. Improved assessment of regional pathologic alterations using a fully non‐invasive imaging method is of high clinical relevance and could push DMI one step toward clinical applications. [ABSTRACT FROM AUTHOR]

Objective: To assess the oncological and functional outcomes of focal high‐intensity focused ultrasound (HIFU) in treating localised prostate cancer (PCa), a 3‐year prospective study was undertaken using periodic post‐ablation saturation biopsies. Patients and Methods: Men with two or fewer lesions of grade group (GG) ≤3 PCa were eligible for participation. Additional criteria included a prostate‐specific antigen (PSA) level of ≤15 ng/mL, clinical T1c‐T2, and a life expectancy of ≥10 years. The primary endpoint was failure‐free survival (FFS), defined as absence of clinically significant PCa (csPCa) in‐ or out‐of‐field on protocol‐mandated saturation biopsy, no whole‐gland or systemic salvage treatment, PCa metastasis, or PCa‐related death. Results are reported using two distinct definitions of csPCa: (i) the presence of any GG ≥2 and (ii) any GG ≥3 or core involvement of ≥6 mm. Secondary endpoints were functional patient‐reported outcome measures addressing urinary, sexual, and bowel function. Results: A total of 91 patients were included: six (7%) with GG1 and 85 (93%) with GG ≥2. In all, 83 (91%) underwent at least one follow‐up biopsy. Biopsy attendance at 6, 12, and 36 months was 84%, 67%, and 51%, respectively. The FFS at these time points for any GG ≥2 PCa was 79% (95% confidence interval [CI] 80–88%), 57% (95% CI 48–69%) and 44% (95% CI 34–56%), respectively. Using the second definition, FFS were 88% (95% CI 81–95%), 70% (95% CI 61–81%) and 65% (95% CI 55–77%), respectively. The 3‐year cancer‐specific survival was 100%, and freedom from metastasis was 99%. Magnetic resonance imaging (MRI) (negative predictive value of up to 89%, 95% CI 84–93%) and relative decrease of PSA values (P = 0.4) performed poorly in detecting residual disease. Urinary and bowel assessment returned to baseline questionnaire scores within 3 months. In all, 17 (21%) patients reported meaningful worsening in erectile function. A significant decrease of PCa related anxiety was observed. Conclusions: Focal HIFU treatment for localised PCa shows excellent functional outcomes with half of the patients remaining cancer‐free after 3 years. Whole‐gland treatment was avoided in 81%. Early follow‐up biopsies are crucial to change or continue the treatment modality at the right time, while the use of MRI and PSA in detecting PCa recurrence is uncertain. [ABSTRACT FROM AUTHOR]