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Radical nephrectomy combined with contemporary chemotherapeutic and radiation therapy protocols has drastically improved outcomes for children with Wilms tumor. Patients with bilateral disease and a syndrome predisposing to tumor development have necessitated the use of nephron-sparing surgery in select cases. Success in managing these patients has increased the indication for partial nephrectomy, although current guidelines for unilateral Wilms tumor are limited. Given that children are being cured with increasing success, recent focus has shifted to long-term health outcomes in addition to tumor treatment. Specifically, renal function has an impact on long-term cardiovascular health and events. Adult outcomes with partial nephrectomy provide a guideline for a paradigm shift in the management of children with Wilms tumor, particularly with advances in imaging and adjuvant therapy. The data are limited for children undergoing partial nephrectomy for unilateral Wilms tumor and outcomes for larger tumors will need to be studied closely in future trials. Increased utilization of neoadjuvant chemotherapy could further expand the number of patients eligible for partial nephrectomy.

Telemedicine utilization rapidly expanded throughout the coronavirus disease 2019 (COVID-19) pandemic and yielded numerous patient benefits. However, its future remains uncertain, limiting further institutional investments in telemedicine. We aimed to evaluate trends in the volume of telemedicine visits throughout the pandemic and patient factors associated with telemedicine usage to inform the future role of telemedicine in pediatric urology. An Institutional Review Board (IRB)-approved registry of pediatric urology outpatient visits was queried from June 2019 to November 2021 at a single institution. Variables of interest included patient demographics, travel distance for care, insurance status, primary visit diagnosis, and visit type (in-person visits, IPVs; or video visits, VVs). IPVs and VVs were further categorized as new patient visits (NPVs), return patient visits (RPVs), and post-operative visits (post-ops). Monthly trends in descriptive variables were summarized. A total of 51,605 pediatric urology outpatient visits occurred during the study period. Patients had a median age of 5 years [interquartile range (IQR), 1–11] and were predominantly male (71.6%), white (61.6%), and held private insurance (69%). VVs increased substantially from 0% in February 2020 to 100% in April 2020 and then subsequently declined through November 2021, although total visit volumes were increasing throughout the period. As the pandemic progressed, compared to IPVs, VVs were more likely to have an RPV visit type (80% vs. 50%–60%) and longer median travel distance (30+ vs. 14 miles). VVs were less frequently covered by subsidized insurance (20%–30% vs. 50%–70%). In summary, this study found that telemedicine usage has declined since the peak of the COVID-19 pandemic, although its use remains higher than pre-pandemic levels and appears to be associated with RPVs, longer travel distance, and private insurance coverage. Utilization has stabilized as we have emerged from peak pandemic restrictions. Further work is required to elucidate the optimal role of telemedicine and its effects on access to care in pediatric urology.

We sought to use deep learning to extract anatomic features from postnatal kidney ultrasounds and evaluate their performance in predicting the risk and timing of chronic kidney disease (CKD) progression for boys with posterior urethral valves (PUV). We hypothesized that these features would predict CKD progression better than clinical characteristics such as nadir creatinine alone.We performed a retrospective cohort study of boys with PUV treated at two pediatric health systems from 1990 to 2021. Features of kidneys were extracted from initial postnatal kidney ultrasound images using a deep learning model. Three time-to-event prediction models were built using random survival forests. The Imaging Model included deep learning imaging features, the Clinical Model included clinical data, and the Ensemble Model combined imaging features and clinical data. Separate models were built to include time-dependent clinical data that were available at 6 months, 1 year, 3 years, and 5 years.Two-hundred and twenty-five patients were included in the analysis. All models performed well with C-indices of 0.7 or greater. The Clinical Model outperformed the Imaging Model at all time points with nadir creatinine driving the performance of the Clinical Model. Combining the 6-month Imaging Model (C-index 0.7; 95% confidence interval [CI] 0.6, 0.79) with the 6-month Clinical Model (C-index 0.79; 95% CI 0.71, 0.86) resulted in a 6-month Ensemble Model that performed better (C-index 0.82; 95% CI 0.77, 0.88) than either model alone.Deep learning imaging features extracted from initial postnatal kidney ultrasounds may improve early prediction of CKD progression among children with PUV. A higher resolution version of the Graphical abstract is available as Supplementary information.

Chronic autoimmune demyelinating neuropathies are a group of rare neuromuscular disorders with complex, poorly characterized etiology. Here we describe a phenotypic, human-on-a-chip (HoaC) electrical conduction model of two rare autoimmune demyelinating neuropathies, chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN), and explore the efficacy of TNT005, a monoclonal antibody inhibitor of the classical complement pathway. Patient sera was shown to contain anti-GM1 IgM and IgG antibodies capable of binding to human primary Schwann cells and induced pluripotent stem cell derived motoneurons. Patient autoantibody binding was sufficient to activate the classical complement pathway resulting in detection of C3b and C5b-9 deposits. A HoaC model, using a microelectrode array with directed axonal outgrowth over the electrodes treated with patient sera, exhibited reductions in motoneuron action potential frequency and conduction velocity. TNT005 rescued the serum-induced complement deposition and functional deficits while treatment with an isotype control antibody had no rescue effect. These data indicate that complement activation by CIDP and MMN patient serum is sufficient to mimic neurophysiological features of each disease and that complement inhibition with TNT005 was sufficient to rescue these pathological effects and provide efficacy data included in an investigational new drug application, demonstrating the model's translational potential.