1,744 research outputs found

    Thoracic cord compression caused by disk herniation in Scheuermann’s disease: A case report and review of the literature

    Get PDF
    We present the case of a 14-year-old male with Scheuermann’s disease and significant neurological deficit due to thoracic disk herniation at the apex of kyphosis. He was treated with an anterior decompression, anterior and posterior fusion in the same setting using plate, cage and a segmental instrumentation system. The patient had an excellent outcome with complete neurological recovery

    Recommendations for the detection and diagnosis of Niemann-Pick disease type C: An update.

    Get PDF
    PURPOSE OF REVIEW: Niemann-Pick disease type C (NP-C) is a neurovisceral disorder that may be more prevalent than earlier estimates. Diagnosis of NP-C is often delayed; a key aim for clinical practice is to reduce this delay. Recently, substantial progress has been made in the field of NP-C screening and diagnosis, justifying an update to the existing recommendations for clinical practice. RECENT FINDINGS: New biomarker profiling and genetic analysis technologies are included as first-line diagnostic tests for NP-C. Most diagnoses can now be confirmed by combination of biomarker and genetic analyses. Filipin staining may facilitate diagnosis in uncertain cases. Recommendations are provided for psychiatrists, neuro-ophthalmologists, and radiologists, and on screening within specific at-risk patient cohorts. The NP-C diagnostic algorithm has been updated and simplified. SUMMARY: This publication provides expert recommendations for clinicians who may see patients presenting with the signs and symptoms of NP-C, including general practitioners, pediatricians, neurologists, and psychiatrists

    Bilateral sternoclavicular joint septic arthritis secondary to indwelling central venous catheter: a case report

    Get PDF
    <p>Abstract</p> <p>Introduction</p> <p>Septic arthritis of the sternoclavicular joint is rare, comprising approximately 0.5% to 1% of all joint infections. Predisposing causes include immunocompromising diseases such as diabetes, HIV infection, renal failure and intravenous drug abuse.</p> <p>Case presentation</p> <p>We report a rare case of bilateral sternoclavicular joint septic arthritis in an elderly patient secondary to an indwelling right subclavian vein catheter. The insidious nature of the presentation is highlighted. We also review the literature regarding the epidemiology, investigation and methods of treatment of the condition.</p> <p>Conclusion</p> <p>SCJ infections are rare, and require a high degree of clinical suspicion. Vague symptoms of neck and shoulder pain may cloud the initial diagnosis, as was the case in our patient. Surgical intervention is often required; however, our patient avoided major intervention and settled with parenteral antibiotics and washout of the joint.</p

    Childhood Predictors of Desistance and Level of Persistence in Offending in Early Onset Offenders

    Get PDF
    Childhood predictors of adolescent offending careers were studied in 310 boys from the longitudinal Pittsburgh Youth Study who started offending prior to age 12. Three main groups were distinguished: serious persisters (n = 95), moderately serious persisters (n = 117), desisters (n = 63), and an intermittent group (n = 35). Group membership was predicted using risk and promotive factors measured in childhood. Serious and moderately serious persisters could be distinguished well from desisters (29.2% and 32.3% explained variance). Distinction between the two persister groups proved somewhat more difficult (20.9% explained variance). More serious persisters than desisters showed disruptive behavior, while moderately serious persisters fell in between. Further, more moderately serious persisters were marked by social disadvantage. Family involvement, small family and positive peer relationships were promotive of desistance. Concluding, early onset offenders show considerable heterogeneity in their adolescent offending careers which seem to some extent to be predicted by different sets of risk and promotive factors

    Expression of High-Affinity IgE Receptor on Human Peripheral Blood Dendritic Cells in Children

    Get PDF
    BACKGROUND: In a mouse model of viral induced atopic disease, expression of FcεRI on dendritic cells is critical. While adult human conventional (cDC) and plasmacytoid (pDC) dendritic cells have been shown to express FcεRI, it is not known if this receptor is expressed in childhood and how its expression is governed by IgE. METHODS: Following informed consent of subjects (n = 27, aged 12-188 months), peripheral blood was stained for surface expression of CD19, ILT7, CD1c, IgE, FcεRI and analyzed by flow cytometry (cDC: CD19(-) ILT7(-) CD1c(+); pDC: CD19(-) ILT7(+) CD1c(-)). Total and specific serum IgE levels to food and inhalant allergens were determined by ImmunoCAP, and the relationship between FcεRI expression on dendritic cells and sensitization, free IgE, cell bound IgE, and age was determined. RESULTS: Independent of sensitization status, FcεRI expression was noted on cDC and pDC as early as 12 months of age. Serum IgE level correlated with expression of FcεRI on cDC, but not pDC. Based on the concentration of IgE, a complex relationship was found between surface bound IgE and expression of FcεRI on cDC. pDC exhibited a linear relationship of FcεRI expression and bound IgE that was consistent through all IgE concentrations. CONCLUSIONS: In children, FcεRI expression on cDC and pDC is modulated differently by serum and cell bound IgE. IgE governance of FcεRI expression on cDC depends upon a complex relationship. Further studies are needed to determine the functional roles of FcεRI on cDC and pDC

    BDNF Facilitates L-LTP Maintenance in the Absence of Protein Synthesis through PKMζ

    Get PDF
    Late-phase long term potentiation (L-LTP) is thought to be the cellular basis for long-term memory (LTM). While LTM as well as L-LTP is known to depend on transcription and translation, it is unclear why brain-derived neurotrophic factor (BDNF) could sustain L-LTP when protein synthesis is inhibited. The persistently active protein kinase ζ (PKMζ) is the only molecule implicated in perpetuating L-LTP maintenance. Here, in mouse acute brain slices, we show that inhibition of PKMζ reversed BDNF-dependent form of L-LTP. While BDNF did not alter the steady-state level of PKMζ, BDNF together with the L-LTP inducing theta-burst stimulation (TBS) increased PKMζ level even without protein synthesis. Finally, in the absence of de novo protein synthesis, BDNF maintained TBS-induced PKMζ at a sufficient level. These results suggest that BDNF sustains L-LTP through PKMζ in a protein synthesis-independent manner, revealing an unexpected link between BDNF and PKMζ
    corecore