Explore the vast range of titles available.

PurposePatients with spinal muscular atrophy (SMA) are often treated with growth friendly devices such as vertical expandable prosthetic titanium rib(VEPTR) and magnetically controlled growing rods(MCGR) to correct spinal deformity and improve pulmonary function. There is limited data on this topic, and the purpose of this study was to assess the effect of these constructs and the addition of chest wall support (CWS) on spinal deformity, thorax morphology and pulmonary outcomes.MethodsThis is a retrospective analysis of prospectively collected data. We included patients with chest wall deformity and scoliosis secondary to SMA who were treated with growth friendly interventions and had two-year follow-up. Descriptive statistics and univariate analyses were performed.ResultsThis study included 66 patients (25% MCGR, 73% VEPTR, 2% unknown). Approximately 23% of constructs included CWS. The average Cobb angle improved from 67° (SD: 27°) to 50° (SD: 26°) at 2 years in patients with CWS (p = 0.02), and from 59° (SD: 20°) to 46° (SD: 15°) at 2 years in patients without CWS (p < 0.01). Hemithorax height improved in patients treated with and without CWS (p = 0.01), but hemithorax width only improved in patients with CWS (p = 0.01). One patient with CWS and two patients without CWS required additional respiratory support at 2 years. The rates of postoperative complications were not significantly different in patients treated with and without CWS (p = 0.31).ConclusionsGrowth friendly constructs improve spinal deformity and may be effective in altering the progression toward respiratory failure in patients with SMA. Patients treated with CWS have significant improvements in thorax morphology compared to patients without CWS.

Study designIt is a retrospective cohort study.ObjectivesTo compare the radiographic and clinical outcomes of serial body casting for infantile idiopathic scoliosis (IIS) with versus without the use of general anesthesia (GA).Summary of background dataSerial body casting for IIS has traditionally been performed under GA. However, reports of neurotoxic effects of anesthetics in young children have prompted physicians to consider instead performing these procedures while patients are awake and distracted by electronic devices.MethodsPatients from a multicenter registry who underwent serial casting for IIS were included. The patients were divided into asleep (GA) and awake (no GA) cohorts. Comparisons were made between pre-casting, first in-cast, and post-casting radiographic measures in each cohort. The rates of successful casting (≥ 10° major CA improvement), curve progression, and incidence of casting abandonment for surgical intervention were also compared.ResultsOne-hundred and twenty-one patients who underwent serial casting for IIS were included. Ninety-two (76%) patients were asleep during casting procedures, while 29 (24%) were awake. Patients in the awake cohort were older (p < 0.01), had a lower BMI (p = 0.03), and more severe curve magnitudes (p < 0.01) at baseline. Patients in the awake cohort experienced greater first-in-cast correction of the major curve (p = 0.01) and improvement in thoracic spine height (p < 0.01). The rate of casting success was higher in the awake cohort (72%) as compared to the asleep cohort (48%) (p = 0.02), although the rate of curve progression (worsening) was similar (p = 0.880). Lastly, there was a lower rate of conversion to surgery at 2 years post-initiation of casting, although this was not statistically significant (0% vs. 8%; p = 0.126).ConclusionsPatients who underwent awake serial casting had similar radiographic outcomes as compared to those who were under general anesthesia during the procedures. Thus, awake casting may provide a safe and effective alternative to the use of general anesthesia in patients with idiopathic infantile scoliosis.Level of evidenceIII.

ObjectivesThe aim of this study is to determine risk factors for infection among EOS patients treated by rib-based distraction instrumentation, and to further assess the incidence of infection among C-EOS categories and sub-types.Summary of background dataDespite the heterogonous nature of early onset scoliosis, the classification of early onset scoliosis (C-EOS) has proven to have excellent reliability across its major categories. C-EOS’s reliability has been verified; however, little data exist on the utility of this categorization in clinical decision-making and risk assessment.MethodsAfter institutional review board approval, data for EOS patients treated by rib-based distraction instrumentation were collected between 2013 and 2017 in a single institution. Data collection included: major categories of early onset scoliosis classification (etiology, major curve and kyphosis), BMI, height, weight, procedure type, site of procedure, presence of tracheostomy, and bowel/urinary incontinence.Results156 EOS patients underwent 843 rib-based distraction instrumentation procedures. 22.4% of patients (35/156 patients, 42 procedures) developed infections, 30/35 requiring irrigation and debridement. Type of procedure was significantly associated with infection rate, with rib-based distraction instrumentation insertion corresponding with the highest incidence of infection, as compared to instrumentation revisions or expansions (p = 0.006). Infection rates were also more common in shorter and lighter weight children (p = 0.001 and 0.03; respectively). Patients with a neuromuscular etiology had the highest rate of infection in comparison to congenital, syndromic, and idiopathic (5.7% vs, 4.9%, 4.7%, and 2.6%; respectively). Notably, high infection rates occurred neuromuscular hyper-kyphotic subjects (M+), occurring in all major curve C-EOS subgroups and at a rate of 8.3% for all procedures.ConclusionNeuromuscular, larger magnitude major curve, and larger magnitude kyphotic angle C-EOS categories appear to be at a higher risk of infection. Such information potentiates the usefulness of C-EOS in surgical decision-making and in the informed consent process.Level of evidenceLevel III therapeutic.

Psoralen is a family of naturally occurring photoactive compounds found in plants that acquire potential cytotoxicity when activated by specific frequencies of electromagnetic waves. Psoralens penetrate the phospholipid cellular membranes and insert themselves between the pyrimidines of deoxyribonucleic acid (DNA). Psoralens are initially biologically inert and acquire photoreactivity when exposed to certain classes of electromagnetic radiation, such as ultraviolet light. Once activated, psoralens form mono- and di-adducts with DNA, leading to marked cell apoptosis. This apoptotic effect is more pronounced in tumor cells due to their high rate of cell division. Moreover, photoactivated psoralen can inhibit tyrosine kinase signaling and influence the immunogenic properties of cells. Thus, the cytotoxicity of photoactivated psoralen holds promising clinical applications from its immunogenic properties to potential anti-cancer treatments. This narrative review aims to provide an overview of the current understanding and research on psoralen and to explore its potential future pharmacotherapeutic benefits in specific diseases.

Novel binary gene expression tools like the LexA-LexAop system could powerfully enhance studies of metabolism, development, and neurobiology in Drosophila. However, specific LexA drivers for neuroendocrine cells and many other developmentally relevant systems remain limited. In a unique high school biology course, we generated a LexA-based enhancer trap collection by transposon mobilization. The initial collection provides a source of novel LexA-based elements that permit targeted gene expression in the corpora cardiaca, cells central for metabolic homeostasis, and other neuroendocrine cell types. The collection further contains specific LexA drivers for stem cells and other enteric cells in the gut, and other developmentally relevant tissue types. We provide detailed analysis of nearly 100 new LexA lines, including molecular mapping of insertions, description of enhancer-driven reporter expression in larval tissues, and adult neuroendocrine cells, comparison with established enhancer trap collections and tissue specific RNAseq. Generation of this open-resource LexA collection facilitates neuroendocrine and developmental biology investigations, and shows how empowering secondary school science can achieve research and educational goals.