Pediatric erythromelalgia from multidisciplinary perspectives: a scoping review.

Erythromelalgia is a rare, chronic pain disorder characterized by the triad of intense burning sensation, warmth, and redness, primarily involving the hands and feet, and usually alleviated by cold and worsened by heat. The objective of this scoping review was to: 1) map the existing literature on erythromelalgia in youth, 2) identify knowledge gaps, and 3) inform directions for future research in pediatric erythromelalgia. One hundred and sixty-seven studies reporting 411 cases of childhood-onset erythromelalgia were identified.

RSK1 is an exploitable dependency in myeloproliferative neoplasms and secondary acute myeloid leukemia.

Myeloid malignancies are heterogenous disorders characterized by distinct molecular drivers but share convergence of oncogenic signaling pathways and propagation by ripe pro-inflammatory niches. Here, we establish a comprehensive transcriptional atlas across the spectrum of myeloproliferative neoplasms (MPN) and secondary acute myeloid leukemia (sAML) through RNA-sequencing of 158 primary samples encompassing CD34+ hematopoietic stem/progenitor cells and CD14+ monocytes. Supported by mass cytometry (CyTOF) profiling, we reveal aberrant networks of PI3K/AKT/mTOR signalling and NFκB-mediated hyper-inflammation.

Mode of Delivery and Subsequent Motor Function in Children With Myelomeningocele Without In Utero Repair.

Objective: To assess the association between mode of delivery and 2-year motor function in children with prenatal diagnosis of myelomeningocele.

Methods: A multisite retrospective cohort study of children with myelomeningocele across 14 NAFTNet (North American Fetal Therapy Network) centers born between 2007 and 2020 who had a physical examination available at 2 years of life. Exclusion criteria were in utero myelomeningocele repair, postnatal myelomeningocele diagnosis, missing data on fetal presentation at delivery, and contraindications to labor.

Social, Emotional, and Behavioral Functioning in Adolescents with Klinefelter Syndrome.

Objective: Klinefelter syndrome (KS) is a common genetic condition in males associated with an extra X chromosome (i.e., 47,XXY).

Voltage-dependent anion channel 1 oligomerization regulates PANoptosis in retinal ischemia-reperfusion injury.

Ischemia-reperfusion injury is a common pathophysiological mechanism in retinal degeneration. PANoptosis is a newly defined integral form of regulated cell death that combines the key features of pyroptosis, apoptosis, and necroptosis. Oligomerization of mitochondrial voltage-dependent anion channel 1 is an important pathological event in regulating cell death in retinal ischemia-reperfusion injury.

Success of Bone Ossification After Posterior Vault Distraction Osteogenesis in Patients With Craniosynostosis.

Background: Posterior vault distraction osteogenesis (PVDO) is an effective treatment for multisuture and syndromic craniosynostosis. It remains unclear how well the calvarium ossifies after PVDO, especially in older patients when they have plateaued in their cranial growth phase. The purpose of this study is to report outcomes associated with PVDO across a wide range of ages at our institution.

Dermatofibrosarcoma Protuberans, Version 1.2025, NCCN Clinical Practice Guidelines In Oncology.

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue sarcoma and affects an estimated 1,500 people annually in the United States. DFSP frequently exhibits extensive local infiltration. Initial treatment is through surgical excision, and care should be taken to ensure that negative margins are achieved to minimize recurrence.

The association between physician sex and patient outcomes: a systematic review and meta-analysis.

Background: Some prior studies have found that patients treated by female physicians may experience better outcomes, as well as lower healthcare costs than those treated by male physicians. Physician-patient sex concordance may also contribute to better patient outcomes. However, other studies have not identified a significant difference.

Utilization of Telemedicine for Patients Receiving In-center Hemodialysis in the United States.

Background: In March 2020, responding to the COVID-19 pandemic, federal emergency waivers in the United States enabled kidney care providers (nephrologists and advanced practice providers) to substitute face-to-face in-center hemodialysis visits with telemedicine encounters. We examined whether the frequency of kidney care provider visits and hospitalizations were associated with telemedicine use in hemodialysis care.

Methods: We used Medicare claims to identify US patients receiving in-center hemodialysis during the first 16 months of the COVID-19 pandemic.

NCCN Guidelines® Insights: Lung Cancer Screening, Version 1.2025.

The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Lung Cancer Screening provide criteria for selecting individuals for screening and offer recommendations for evaluating and managing lung nodules detected during initial and subsequent annual screening. These NCCN Guidelines Insights focus on recent updates to the NCCN Guidelines for Lung Cancer Screening.

Responsiveness to transcutaneous spinal stimulation for upper extremity recovery following spinal cord injury: A case series exploration of midsagittal tissue bridges.

Context: Transcutaneous spinal stimulation (TSS), applied to the cervical spine, is able to improve voluntary upper extremity strength and function in individuals with cervical spinal cord injury (SCI). While most respond and improve in the presence of TSS, others do not respond as favorably. Midsagittal tissue bridges, adjacent to the lesion, can be observed and measured using T-weighted magnetic resonance imaging (MRI), and both ventral and dorsal tissue bridges are associated with recovery following SCI.

Distinct myeloid-derived suppressor cell populations in human glioblastoma.

The role of glioma-associated myeloid cells in tumor growth and immune evasion remains poorly understood. We performed single-cell RNA sequencing of immune and tumor cells from 33 gliomas, identifying two distinct myeloid-derived suppressor cell (MDSC) populations in isocitrate dehydrogenase-wild-type (IDT-WT) glioblastoma: an early progenitor MDSC (E-MDSC) population with up-regulation of metabolic and hypoxia pathways and a monocytic MDSC (M-MDSC) population. Spatial transcriptomics demonstrated that E-MDSCs geographically colocalize with metabolic stem-like tumor cells in the pseudopalisading region.

Protein-Based Degraders: From Chemical Biology Tools to Neo-Therapeutics.

The nascent field of targeted protein degradation (TPD) could revolutionize biomedicine due to the ability of degrader molecules to selectively modulate disease-relevant proteins. A key limitation to the broad application of TPD is its dependence on small-molecule ligands to target proteins of interest. This leaves unstructured proteins or those lacking defined cavities for small-molecule binding out of the scope of many TPD technologies.

Risk of cardiovascular disease following degarelix versus gonadotropin-releasing hormone agonists in patients with prostate cancer: a systematic review and meta-analysis.

Background: Prostate cancer treatment involves hormonal therapies that may carry cardiovascular risks, particularly for long-term use. Gonadotropin-releasing hormone (GnRH) antagonists, such as degarelix, may offer advantages over agonists, but comprehensive comparative cardiovascular outcomes are not well established. This study aimed to systematically review and analyze the cardiovascular safety profiles of degarelix compared to those of traditional GnRH agonists, providing critical insights for optimizing treatment strategies.

Reconstructive techniques for chest and breast deformities in Poland syndrome: An up-to-date systematic review.

Background: Poland syndrome (PS) is a congenital abnormality defined as aplasia or hypoplasia of the unilateral pectoralis muscle and breast tissue that may be accompanied by limb or thoracic deformities. Reconstruction of deformities associated with PS is challenging owing to the spectrum of differences. We aimed to evaluate the trends in surgical management of chest and breast anatomical anomalies associated with PS.