Dreams of 'shooting out': hip-hop music production in Bishkek in the age of streaming

Digitalised music production and the rise of music streaming platforms shape music markets worldwide. Whereas in Western Europe and North America, the age of streaming has also been criticised for a casualisation of musical labour, in peripheral music economies like Kyrgyzstan it participates in structuring a market for music makers. At the same time, digitalisation also tends to reproduce global inequalities. Based on the case of hip-hop music production in Bishkek, Kyrgyzstan, the present working paper examines how the 'age of streaming' affects the musical production and the dreams and ambitions of music workers

Reflections from the Jury Box: Improving Evidence Based Practice through a Comparison with Our Legal System

Background: An experience serving jury duty prompted reflection on the parallels between evidenced based medicine and our legal system. Findings: The steps of the legal system can be tied to each step of the practice of evidenced based medicine. Implications: Patients should be included in evidence based decisions. Pharmacists can act as resources for other providers practicing evidenced based medicine. Educators can use this analogy to teach evidence based medicine. Conflict of Interest We declare no conflicts of interest or financial interests that the authors or members of their immediate families have in any product or service discussed in the manuscript, including grants (pending or received), employment, gifts, stock holdings or options, honoraria, consultancies, expert testimony, patents and royalties.   Type: Commentar

A Comprehensive Review of Pegvaliase, an Enzyme Substitution Therapy for the Treatment of Phenylketonuria

Objective: To review the pharmacology, pharmacokinetics, efficacy, safety, and place in therapy of a phenylalanine-metabolizing enzyme indicated to reduce blood phenylalanine concentrations, pegvaliase injection. Data Sources: Searches of MEDLINE (1946-September 1, 2018) were conducted using the terms pegvaliase and phenylalanine ammonia lyase (PAL). Additional data were obtained from the prescribing information, the product dossier obtained from the manufacturer, and Clinicaltrials.gov. Study Selection and Data Extraction: All English language articles related to pharmacology, pharmacokinetics, efficacy, or safety of the combination therapy in human subjects were reviewed. Data Synthesis: Pegvaliase is a pegylated PAL enzyme that converts phenylalanine to ammonia and trans-cinnamic acid. Blood phenylalanine levels were reduced by approximately 50% to 70% in patients receiving therapeutic doses of pegvaliase. However, most patients experienced adverse events. Conclusions and Relevance: The mainstay of therapy in phenylketonuria (PKU) has historically consisted of dietary restriction of phenylalanine. Pegvaliase injection is the first Food and Drug Administration (FDA)-approved enzyme substitution therapy for patients with PKU. The therapy may be a viable option for patients with documented blood phenylalanine \u3e 600 micromol/L who have failed existing management strategies

Reflections from the Jury Box: Improving Evidence Based Practice through a Comparison with Our Legal System

Background: An experience serving jury duty prompted reflection on the parallels between evidenced based medicine and our legal system. Findings: The steps of the legal system can be tied to each step of the practice of evidenced based medicine. Implications: Patients should be included in evidence based decisions. Pharmacists can act as resources for other providers practicing evidenced based medicine. Educators can use this analogy to teach evidence based medicine. Conflict of Interest We declare no conflicts of interest or financial interests that the authors or members of their immediate families have in any product or service discussed in the manuscript, including grants (pending or received), employment, gifts, stock holdings or options, honoraria, consultancies, expert testimony, patents and royalties.   Type: Commentar

La Polynésie française pourra-t-elle demeurer un pays heureux ?

On ne devrait pouvoir parler de développement humain que lorsque les hommes grandissent en humanité et qu’ils deviennent plus épanouis et plus satisfaits des relations qu’ils ont avec les autres hommes. Un tel développement a donc deux composantes : l’une individuelle et l’autre sociale. Quelles sont les chances d’un tel développement en Polynésie française ?We should not talk about human development until mankind grows in humanity and becomes more fulfilled and more satisfied with the relationship it has with other men. Such a development is made of two componens: one is personal and the other social. What are the chances of such a development in French Polynesia

An Oncology, Lifestyle Medicine, and Cardiac Rehabilitation Partnership in the Development of a Group Medical Visit Model for Cancer Survivors

Methods/Approach: An oncology and cardiology team developed an oncology provider survey in REDCap designed to assess attitudes regarding perspectives on cardio-oncology, rehabilitation, and Lifestyle Medicine. The 27-item multiple choice and Likert-style survey was distributed via email on three separate occasions to MaineHealth Cancer Care Network providers (n=66) in spring, 2022.https://knowledgeconnection.mainehealth.org/lambrew-retreat-2023/1004/thumbnail.jp

Childhood-Onset Epileptic Encephalopathy Associated With Isolated Focal Cortical Dysplasia and a Novel TSC1 Germline Mutation

Tuberous sclerosis complex (TSC) is an autosomal-dominant inheritable neurocutaneous disease due to mutations within the TSC1 and TSC2 genes. Many patients present with West syndrome, a severe epilepsy syndrome characterized by the triad of infantile spasms, an interictal electroencephalogram (EEG) pattern termed hypsarrhythmia (continuous slow activity with an amplitude higher than 300 mu V and multiregional spikes/polyspikes/sharp waves) and developmental regression. In this study, we report on a previously healthy patient with positive family history of epilepsy with new-onset epileptic encephalopathy at the age of 9 years. Clinical signs alone were not sufficient to establish the diagnosis of TSC but epilepsy panel screening revealed a novel frameshift mutation (c.90delA;p.Glu31Argfs*12) within the TSC1 gene. Segregation gene analysis detected the same mutation in the mother. Cranial magnetic resonance imaging (MRI) studies from the index patient and his mother revealed a similar pattern of isolated subcortical white matter lesions resembling most likely focal cortical dysplasia (FCD) type IIb. In summary, in these 2 related patients, a novel TSC1 frameshift mutation was associated with an isolated FCD type IIb in the absence of further CNS abnormalities usually encountered in patients with TSC, fostering our understanding of the broad mutation spectra in the TSC1 gene and the close relationship between cortical tubers and FCD type IIb

Combined Scintigraphy and Tumor Marker Analysis Predicts Unfavorable Histopathology of Neuroblastic Tumors with High Accuracy

Objectives Our aim was to improve the prediction of unfavorable histopathology (UH) in neuroblastic tumors through combined imaging and biochemical parameters. Methods I-123-MIBG SPECT and MRI was performed before surgical resection or biopsy in 47 consecutive pediatric patients with neuroblastic tumor. Semi-quantitative tumor-to-liver count-rate ratio (TLCRR),MRI tumor size and margins, urine catecholamine and NSE blood levels of neuron specific enolase (NSE) were recorded. Accuracy of single and combined variables for prediction of UH was tested by ROC analysis with Bonferroni correction. Results 34 of 47 patients had UH based on the International Neuroblastoma Pathology Classification (INPC). TLCRR and serum NSE both predicted UH with moderate accuracy. Optimal cut-off for TLCRR was 2.0, resulting in 68% sensitivity and 100% specificity (AUC-ROC 0.86, p < 0.001). Optimal cut-off for NSE was 25.8 ng/ml, resulting in 74% sensitivity and 85% specificity (AUC-ROC 0.81, p = 0.001). Combination of TLCRR/NSE criteria reduced false negative findings from 11/9 to only five, with improved sensitivity and specificity of 85% (AUC-ROC 0.85, p < 0.001). Conclusion Strong I-123-MIBG uptake and high serum level of NSE were each predictive of UH. Combined analysis of both parameters improved the prediction of UH in patients with neuroblastic tumor. MRI parameters and urine catecholamine levels did not predict UH

Three-Dimensional High-Resolution Black-Blood Magnetic Resonance Imaging for Detection of Arteritic Anterior Ischemic Optic Neuropathy in Patients With Giant Cell Arteritis

Objectives: Arteritic anterior ischemic optic neuropathy (A-AION) caused by inflammatory occlusion of the posterior ciliary arteries is the most common reason for irreversible vision loss in patients with giant cell arteritis. Atypical clinical presentation and negative funduscopy can delay systemic high-dose corticosteroid therapy to prevent impending permanent blindness and involvement of the contralateral eye. The purpose of this study was to assess the diagnostic accuracy of 3-dimensional (3D) high-resolution T1-weighted black-blood magnetic resonance imaging (T1-BB-MRI) for the detection of posterior ciliary artery involvement in patients with giant cell arteritis and funduscopic A-AION. Materials and Methods: After institutional review board approval and informed consent, 27 patients with suspected giant cell arteritis and vision disturbances were included in this monocentric prospective cohort study. Giant cell arteritis was diagnosed in 18 patients according to the diagnostic reference standard (6 men, 73.8 [69.0-78.0] years);14 of those were positive for A-AION. Precontrast and postcontrast 3D T1-BB-MRI was performed in all 27 patients. Two radiologists separately assessed image quality and local fat suppression (4-point scale), visual contrast enhancement (3-point scale), and diagnostic confidence (5-point scale) regarding arteritic posterior ciliary artery involvement. Magnetic resonance imaging findings were assessed in comparison to funduscopy. Statistical analysis included accuracy parameters and interrater agreement. Results: Sensitivity of 3D T1-BB-MRI was 92.9% (95% confidence interval, 66.1%-99.8%) and specificity was 92.3% (95% confidence interval, 64.0%-99.8%) for detection of A-AION-positive patients. Image quality and local fat suppression were assessed with 3.2 +/- 0.8 (median 3) and 3.8 +/- 0.5 (median 4). Visual contrast enhancement with 2.3 +/- 0.8 (median 3) and diagnostic confidence was rated at 4.7 +/- 0.5 (median 5). Interrater agreement was high (kappa = 0.85, P < 0.001). Three-dimensional T1-BB-MRI displayed bilateral findings in 50% of the cases, whereas only unilateral A-AION was detected in funduscopy as a possible indication for the contralateral eye at risk. Conclusions: Three-dimensional T1-BB-MRI allows accurate detection of arteritic posterior ciliary artery involvement in patients with A-AION. Further, 3D T1-BB-MRI seems to display arteritic involvement of the posterior ciliary arteries earlier than funduscopy and might, therefore, display "vision-at-risk" in patients with visual impairment and suspected giant cell arteritis but unremarkable funduscopy