A case of chronic recurrent multifocal osteomyelýtýs successfully treated with etanercept

PubMe

MEFV mutations in systemic JIA

Background: Systemic form of juvenile idiopathic arthritis (JIA) is regarded as an autoinflammatory disease. Certain genetic polymorphisms in genes coding inflammatory proteins have been associated with the disease. On the other hand mutations of the MEFV gene cause a monogenic autoinflammatory disease, Familial Mediterranean Fever (FMF). In a previous study in adult rheumatoid arthritis 3 out of the 25 British patients who developed secondary amyloidosis had a mutation/polymorphism in the MEFV gene. Aim: To analyse whether mutaions in the MEFV gene had an association with systemic JIA. Patients and methods: MEFV mutations were screened in a total of 32 systemic JIA patients. All had been classified as systemic JIA according to the Durban JIA criteria. None had disease characteristics that met the Tel Hashomer criteria for the diagnosis of FMF. Results: 2 carrier for M694V and two patients who were homozygote for MEFV mutations. Both of these patients were among the most severe patients in the group. One had an excellent response to etanercept whereas the other was resistant to anti-TNF and other conventional treatments and had only a partial response to thalidomide. Although the number of severe mutations were increased in this small group of patients with systemic JIA the difference with the Turkish population did not reach statistical significance, but the disease causing mutation (M694V) was significantly high in the patients with systemic JIA(p = 0.02). Conclusion: However, the severe disease course in the aforementioned patients suggest that MEFV mutations may be a modifying genetic factor in systemic JIA.PubMe

Evolución de los ácidos grasos en el mesocarpo del níspero (Mespilus germanica. L.) a diferentes estados de maduración

The fatty acid composition of medlar (Mespilus germanica L.) varied significantly among the ripening stages sampled at 157, 172 and 187 DAFs (days after full bloom). Twenty-one different fatty acids were detected in preclimacteric fruit and 17 when the climacteric began. Principal fatty acids, determined in medlar fruit harvested from October (157 and 172 DAFs) to November (187 DAF) were mainly palmitic acid (16:0), linoleic acid (18:2n-6), and a-linolenic acid (18:3n-3). While the content of saturated fatty acids [palmitic acid (16:0) and stearic acid (18:0)] increased, the content of the essential polyunsaturated fatty acids [linoleic acid (18:2n-6) and linolenic acid (18:3n-3)] decreased through ripening, in parallel with pulp darkening. The percentage of linoleic acid and a-linolenic acid in ripe, hard fruits was 60.0 and 13.5 % of dry wt at 157 DAF which decreased throughout ripening, remaining at 28.7 and 5.6 % of dry wt, respectively, in the fully softened and darkened pulp. A marked decreases in the double bond index, percentage of unsaturation and the ratio of unsaturation/saturation were also seen throughout the medlar ripening. The contribution of unsaturated fatty acid to the total fatty acid content decreased markedly as the medlar fruit became progressively softer and darkened.La composición en ácidos grasos del níspero (Mespilus germanica L.) varió significativamente entre los estados de maduración muestreados a los 157, 172 y 187 DAFs (días después de la floración). Veinte y un ácidos grasos diferentes fueron detectados en el fruto preclimatérico y 17 cuando comenzó el climaterio. Los ácidos grasos principales encontrados en nísperos, recolectados desde Octubre (157 y 172 DAFs) hasta Noviembre (187 DAF), fueron principalmente ácido palmítico (16:0), ácido linoléico (18:2n-6), y ácido a-linolénico (18:3n-3). En tanto que el contenido en ácidos grasos saturados (ácido palmítico (16:0) y ácido esteárico (18:0)) aumentó, el contenido en ácidos grasos esenciales (ácido linoleico (18:2n-6) y ácido linolénico (18:3n-6)) disminuyó durante la maduración, en paralelo con el oscurecimiento de la pulpa. El porcentaje de ácido linoleico y de ácido a-linolénico en frutos maduros sin reblandecer fue de 60.0 y 13.5 % del peso seco a 157 DAF, disminuyendo durante la maduración, y permaneciendo a 28.7 y 56 % del peso seco, respectivamente, en la pulpa completamente blanda y oscura. También se observó durante la maduración del níspero una marcada disminución en el número de dobles enlaces, en el tanto por ciento de instauración y en la relación instauración / saturación. La contribución de los ácidos grasos insaturados al contenido de ácidos grasos totales disminuyó marcadamente cuando el níspero comenzó progresivamente a reblandecerse y oscurecerse

Growth Pattern and Morphological Variation of Labeo calbasu Found in Indus River, Sindh-Pakistan

The present work reports the length–weight relationships (LWR) and condition factor relationships for Labeo calbasu collected from Upstream (Matyari) Kotri barrage at, River Indus, Pakistan, because stock assessment helps the fisheries managers to conserve the commercially important fish. Morphological characters of fish as well as Length-weight relationship are an important tool for fishery management. The results of LWR (W= aLb), for L calbasu.  Representing negative allometric growth pattern. LWRs and condition factor relationships were found significantly correlated. A total of 200 and 190 specimens from upstream and downstream were collected, respectively. The assessed values of length-weight correlation and condition factor were calculated as Kn=39.663 (LeCren), and K=11.915 (Fulton) for upstream and Kn=44.066 and K=13.872 for downstream. Length-weight was found with a strong correlation of n= 2.892, a=0.0235 with r2=0.934 for upstream population then the downstream population.  The results of this work would be beneficial for sustainable management as well as fishery managers. 

Ultracompact microinterferometer-based fiber Bragg grating interrogator on a silicon chip

We report an interferometer-based multiplexed fiber Bragg grating (FBG) interrogator using silicon photonic technology. The photonic-integrated system includes the grating coupler, active and passive interferometers, interferometers, a 12-channel wavelength-division-multiplexing (WDM) filter, and Ge photodiodes, all integrated on a 6x8 mm2 silicon chip. The system also includes optical and electric interfaces to a printed board, which is connected to a real-time electronic board that actively performs the phase demodulation processing using a multitone mixing (MTM) technique. The device with active demodulation, which uses thermally-based phase shifters, features a noise figure of σ  =  0.13 pm at a bandwidth of 700 Hz, which corresponds to a dynamic spectral resolution of 4.9 fm/Hz1/2. On the other hand, the passive version of the system, based on a 90º-hybrid coupler, features a noise figure of σ  =  2.55 pm at a bandwidth of 10 kHz, also showing successful detection of a 42 kHz signal when setting the bandwidth to 50 kHz. These results demonstrate the advantage of integrated photonics, which allows the integration of several systems with different demodulation schemes in the same chip and guarantees easy scalability to a higher number of ports without increasing the dimensions or the cost

The Glial Regenerative Response to Central Nervous System Injury Is Enabled by Pros-Notch and Pros-NFκB Feedback

Organisms are structurally robust, as cells accommodate changes preserving structural integrity and function. The molecular mechanisms underlying structural robustness and plasticity are poorly understood, but can be investigated by probing how cells respond to injury. Injury to the CNS induces proliferation of enwrapping glia, leading to axonal re-enwrapment and partial functional recovery. This glial regenerative response is found across species, and may reflect a common underlying genetic mechanism. Here, we show that injury to the Drosophila larval CNS induces glial proliferation, and we uncover a gene network controlling this response. It consists of the mutual maintenance between the cell cycle inhibitor Prospero (Pros) and the cell cycle activators Notch and NFκB. Together they maintain glia in the brink of dividing, they enable glial proliferation following injury, and subsequently they exert negative feedback on cell division restoring cell cycle arrest. Pros also promotes glial differentiation, resolving vacuolization, enabling debris clearance and axonal enwrapment. Disruption of this gene network prevents repair and induces tumourigenesis. Using wound area measurements across genotypes and time-lapse recordings we show that when glial proliferation and glial differentiation are abolished, both the size of the glial wound and neuropile vacuolization increase. When glial proliferation and differentiation are enabled, glial wound size decreases and injury-induced apoptosis and vacuolization are prevented. The uncovered gene network promotes regeneration of the glial lesion and neuropile repair. In the unharmed animal, it is most likely a homeostatic mechanism for structural robustness. This gene network may be of relevance to mammalian glia to promote repair upon CNS injury or disease

Precariousness, gender, resistance and consent in the face of global production network’s ‘Reforms’ of Pakistan’s garment manufacturing industry

This case study of the restructuring of Pakistan’s garment manufacturing industry explores how attempts to increase capital’s control over the labour process intersect with local patriarchal structures and trigger workers’ reflexivity and agency causing unanticipated consequences. Using Archer’s notion of agency, the article examines the theoretical space where capitalism meets patriarchy, and both are reproduced. The focus on reflexivity, anchored between objective contexts and agents’ personal concerns, helps theorize capital–labour–gender relations in global supply chains and explains workers’ impactful resistance to protect a supposedly precarious work regime. Our findings challenge the notion that globalization reduces workers’ agency and their potential for impactful resistance

Artificial agents among us: Should we recognize them as agents proper?

In this paper, I discuss whether in a society where the use of artificial agents is pervasive, these agents should be recognized as having rights like those we accord to group agents. This kind of recognition I understand to be at once social and legal, and I argue that in order for an artificial agent to be so recognized, it will need to meet the same basic conditions in light of which group agents are granted such recognition. I then explore the implications of granting recognition in this manner. The thesis I will be defending is that artificial agents that do meet the conditions of agency in light of which we ascribe rights to group agents should thereby be recognized as having similar rights. The reason for bringing group agents into the picture is that, like artificial agents, they are not self-evidently agents of the sort to which we would naturally ascribe rights, or at least that is what the historical record suggests if we look, for example, at what it took for corporations to gain legal status in the law as group agents entitled to rights and, consequently, as entities subject to responsibilities. This is an example of agency ascribed to a nonhuman agent, and just as a group agent can be described as nonhuman, so can an artificial agent. Therefore, if these two kinds of nonhuman agents can be shown to be sufficiently similar in relevant ways, the agency ascribed to one can also be ascribed to the other-this despite the fact that neither is human, a major impediment when it comes to recognizing an entity as an agent proper, and hence as a bearer of rights

Molecular epidemiology of hcv among health care workers of khyber pakhtunkhwa

<p>Abstract</p> <p>Background</p> <p>Studies of the molecular epidemiology and risk factors for hepatitis C virus (HCV) in health care workers (HCWs) of Peshawar, Khyber Pakhtunkhwa region are scarce. Lack of awareness about the transmission of HCV and regular blood screening is contributing a great deal towards the spread of hepatitis C. This study is an attempt to investigate the prevalence of HCV and its possible association with both occupational and non-occupational risk factors among the HCWs of Peshawar.</p> <p>Results</p> <p>Blood samples of 824 HCWs, aged between 20-59 years were analysed for anti-HCV antibodies, HCV RNA and HCV genotypes by Immunochromatographic tests and PCR. All relevant information was obtained from the HCWs with the help of a questionnaire. The study revealed that 4.13% of the HCWs were positive for HCV antibodies, while HCV RNA was detected in 2.79% of the individuals. The most predominant HCV genotype was 3a and 2a.</p> <p>Conclusion</p> <p>A program for education about occupational risk factors and regular blood screening must be implemented in all healthcare setups of Khyber Pakhtunkhwa province in order to help reduce the burden of HCV infection.</p