Citations

Summary Amyloid light‐chain (AL) amyloidosis is a systemic clonal plasma cell disorder characterized by the production and deposition of misfolded immunoglobulin light chains (LCs), resulting in multiorgan dysfunction. Due to its intricate molecular mechanisms and diverse organ involvement, the disease poses significant diagnostic and therapeutic challenges. This review explores the molecular landscape of AL amyloidosis, emphasizing genetic, transcriptomic and proteomic alterations. Key findings include chromosomal abnormalities, somatic mutations, aberrant gene expression, disrupted protein folding pathways and the role of cytokine and chemokine secretion. These factors collectively drive the overproduction and destabilization of amyloidogenic LCs, leading to organ‐specific amyloid deposition, clinical heterogeneity and variable patient outcomes. Despite therapeutic advancements, the disease's complexity challenges the development of effective biological models. Progressing towards personalized therapies requires the development of preclinical models and the identification of biomarkers and molecular data to design targeted interventions. This review highlights the importance of integrating DNA, RNA and protein‐level analyses to deepen the understanding of AL amyloidosis pathogenesis. Such insights are pivotal for improving diagnostics, prognostics and therapeutic strategies, ultimately advancing precision medicine for this challenging disease.

Ewing sarcoma and osteosarcoma constitute 36% of all primary bone cancers. However, these 2 subtypes represent the most commonly diagnosed bone cancer types in the pediatric and adolescent population. Although still largely unknown, certain genetic mutations, rearrangements, and/or predisposition syndromes likely play a role in the pathogenesis of bone cancer. Osteosarcoma may also develop as a direct result of the long-term side effects of radiation therapy. With the implementation of a multimodality approach to treatment, including multiagent neoadjuvant and adjuvant chemotherapy regimens, targeted therapy options, surgery, and radiation, individuals with Ewing sarcoma and osteosarcoma are showing higher cure rates and improved overall survival. The NCCN Guidelines for Bone Cancer provide a consensus and evidence-based framework for the workup, management, and surveillance of local and recurrent/metastatic disease.

ABSTRACTBackgroundAs a component of goal‐oriented care, goal‐setting is an important strategy to improve self‐management and reduce the economic and personal burden of chronic illness. Primary care nurses, as integral members of the healthcare team, play a pivotal role in providing self‐management support to patients with multiple chronic illnesses. Yet, little is known about their perspectives on partnering with patients to set meaningful goals within self‐management support programmes.AimTo explore perspectives of primary care nurses about their practice of goal‐setting with patients to improve self‐management.MethodsFor this qualitative exploratory study, we conducted semi‐structured interviews with 28 primary care nurses who provided self‐management support to patients with multiple chronic illnesses. Data were analysed using inductive thematic analysis. This research was approved by the Mayo Clinic Institutional Review Board.ResultsKey themes from interviews focused on: (1) goal‐setting practices; (2) challenges encountered in goal‐setting; and (3) the perceived value‐add of using a patient‐centred goal‐setting approach. Nurses described practices that empower patients to take charge of goal‐setting, emphasising that the opportunity to create personal goals enhances motivation and enables the development of realistic action plans. Key challenges faced were patients' lack of familiarity with personal goal‐setting, competing life stressors and patients' reluctance to change.ConclusionOur findings contribute valuable insights about how primary care nurses approach goal‐setting within a self‐management support program. Notably, unlike previous studies where clinicians reported that time‐limited appointments and institutional expectations hindered patient‐centred goal‐setting, our participants practicing in an institutionally supported goal‐oriented patient‐centred model of care did not report time constraints or pressures to prioritise clinical goals over patient goals. Instead, they described patient‐centred goal‐setting practices that are associated with positive patient and clinical outcomes. To potentially corroborate and expand upon our findings and inform practice and training, future research should include qualitative and survey studies to explore primary care RNs perspectives about goal‐setting and observational research to evaluate nurse–patient goal‐setting interactions.

ABSTRACTObjectiveTo explore pre–hematopoietic stem cell transplant (HSCT) demographic, disease, and psychological factors predictive of future transplant regret and to determine post‐HSCT variables associated with regret.Patients and MethodsHSCT candidates participated in a prospective cohort study (June 2008–October 2013) examining health behaviors and HSCT outcomes, including completion of standardized surveys at pre‐HSCT (baseline) and 1‐year post‐HSCT. Cases were participants that endorsed regret at 1‐year post‐HSCT follow‐up, and controls were participants without regret at 1 year, matched on age, sex, and transplant type. For cases and controls, pre‐HSCT psychosocial evaluations were abstracted from the electronic health record and coded to determine the Psychosocial Assessment of Candidates for Transplantation score, psychosocial stressors, and mental health diagnoses. The association of selected factors with regret was estimated with odds ratios and 95% confidence intervals from conditional logistic regression models.ResultsAt post‐HSCT, 49 participants of 638 endorsed transplant regret (8%) and formed the case group; 98 controls were matched from remaining participants. Cases and controls were well matched on age (56.6 vs. 57.2 years), sex (both groups 34.7% female), and transplant type (both groups 81.6% autologous). After controlling for the number of hospitalizations and active treatment status, conditional logistic regression revealed that patients who endorsed regret were 3.7 times (95% CI = 1.37–9.69, p = 0.008) more likely to not be in remission compared to controls at 1‐year post‐HSCT.ConclusionMatched case–control analyses revealed that no pre‐HSCT variables collected during the pre‐HSCT evaluation period were predictive of transplant regret, while poorer outcomes at 1‐year after transplant were associated with regret.

Pediatric regional anesthesia offers significant benefits, yet its adoption faces barriers, including perceived overcomplexity. This study aimed to identify and establish a set of core, high-value, low-complexity nerve blocks to improve perioperative pain management in pediatric patients. A four-round modified Delphi consensus study was conducted with an international panel of pediatric and regional anesthesia experts. An initial long list of regional techniques was compiled by the Steering Committee and refined through iterative input. Panelists rated each technique on a 10-point Likert scale for importance. Consensus was defined as ≥75% of panelists assigning a mean importance score of ≥8. Techniques receiving 50%–74% agreement were categorized as having strong agreement and considered for inclusion. The final selection was confirmed through a virtual roundtable discussion. Thirty-three experts representing 12 pediatric and regional societies participated. Consensus was reached on six regional techniques, with strong agreement (*) on two additional techniques, identifying eight core pediatric regional anesthesia blocks: supraclavicular brachial plexus block, rectus sheath block, transverse abdominis plane block*, suprainguinal fascia iliaca block*, femoral nerve block, adductor canal block, popliteal sciatic nerve block, and landmark-based caudal block. This consensus-driven framework defines a core set of pediatric regional anesthesia techniques that balance clinical effectiveness, feasibility, and accessibility. These findings provide a practical entry point for practitioners looking to incorporate pediatric regional anesthesia into their practice, regardless of prior experience. Future efforts should focus on standardized training, implementation research, and policy initiatives to support widespread adoption and improve perioperative pain management in children globally.