Griscelli syndrome type 2: The first reported case of compound heterozygous mutation in RAB 27 A gene from India

Griscelli syndrome (GS) is a rare autosomal recessive disorder resulting in pigmentary dilution of the skin and hair with variable phenotypes depending upon the underlying genetic mutation. Mutations in 3 distinct genes MYO5A, RAB27A, MLPH are responsible for 3 subtypes (GS1, GS2, and GS3) of GS respectively. Griscelli syndrome type 2 (GS-2) is a rare autosomal recessive disease. It commonly presents with hemophagocytic lymphohistiocytosis (HLH) and recurrent infections due to immunodeficiency. We describe a 3-year-old female with pyrexia, silvery hair, lymphadenopathy, hepatosplenomegaly, and hemophagocytosis. We found a novel compound heterozygous mutation in the RAB 27A gene. She succumbed despite being on dexamethasone and septran prophylaxis. This case spreads awareness about this rare potentially fatal disease, as a high index of suspicion is required for prompt diagnosis and treatment. Early bone marrow transplant is the only curative treatment for GS-2

Interrelationship between cloud cover and sensible heat flux over land during MONTBLEX-1990

Micro-meteorological tower observations of MONTBLEX (Monsoon Trough Boundary Layer Experiment)- 1990, combined with routine surface observations at Jodhpur in the dry convective sector of Indian summer monsoon trough are used to examine the interrelationship between total cloud cover (TCC) and surface sensible heat flux (SHF) during the summer monsoon of 1990. A significant inverse relationship between TCC and SHF is found during various Intensive Observation Periods of the experiment. This relationship holds for the various methods of estimation of SHF. © Printed in India

Thermoelastic stress analysis of a functionally graded transversely isotropic hollow cylinder in elliptical coordinates

This paper is concerned with the axisymmetric thermoelastic problem to investigate the influence of nonlinear heat conduction equation, displacement functions and thermal stresses of a functionally graded transversely isotropic hollow cylinder that is presented in the elliptical coordinate system. The method of integral transform technique is used to produce an exact solution of the heat conduction equation in which sources are generated according to a linear function of the temperature. An explicit exact solution of the governing thermoelastic equation is proposed when material properties are power-law functions with the exponential form of the radial coordinate. Numerical calculations are also carried out for Material I with the nearly isotropic feature, along with Material II as an anisotropic material and illustrated graphically. The validity of the solution is demonstrated by comparing with the previous results

Efficacy of standard treatment protocol in recently diagnosed Lupus Nephritis at our tertiary care teaching hospital

Introduction: Lupus Nephritis occurred in approximately 50% of Systemic Lupus Erythematosus patients at some point during their illness and is associated with a poor prognosis. Material and Method: A prospective observational study of 50 newly diagnosed LN cases was conducted to investigate the response of standard treatment protocol (Cyclophosphamide -NIH protocol and Mycophenolate Mofetil-MMF). Results: Of the 50 newly diagnosed cases of LN, 94 % (n=47) were females, and 6 % (n=3) were males, with class IV LN accounting for the majority of patients 69.39 % (n=34). At six months, 36.7 % (n=11) of patients in the cyclophosphamide (CYP) group had a complete response. Only 27.3 % of patients in the MMF group had a complete response; however, this difference was not statistically significant. At the end of one year, only 56.7 % of the CYP group and 81.8 % of the MMF group had a complete response; however, this difference was not statistically significant (p=0.282). Although the initial response with CYP was better and later in the MMF group, these differences were not significant statistically. Tuberculosis or its reactivation was the most common complication during treatment, either with MMF or CYP. One patient died due to latent tuberculosis reactivation, another as a result of severe disease activity at presentation (proteinuria was 20 gm/24 hours in that patient), and the third as a result of pneumonia with septicemia. Conclusion: Treatment with either CYP or MMF is equally effective, but underlying infection, particularly tuberculosis, should be ruled out before initiating therapy

Graphene boosts thermoelectric performance of a Zintl phase compound

The concept of nanocomposites derived by incorporating a second minor phase in bulk thermoelectric materials has established itself as an effective paradigm for optimizing high thermoelectric performance. In this work, this paradigm is for the first time extended to bulk Zintl phase Mg3Sb2 and its isoelectronically Bi-doped derivative Mg3Sb1.8Bi0.2 system. Herein, we report the synthesis, microstructural details, electronic structure and thermoelectric properties of (Mg3Sb2, Mg3Sb1.8Bi0.2)/ graphene nanosheet (GNS) nanocomposites with different mass ratios. Field emission scanning electron microscopy (FE-SEM) and transmission electron microscopy (TEM) investigation reveals that Mg3Sb2 nanoparticles are homogenously anchored on the surface of GNS. We demonstrate that Mg3Sb2-based materials incorporated with a small content of graphene outperform optimally, resulting in potential p-type thermoelectric materials. The present nanocomposite additive of GNS deriving such a novel nanocomposite of (Mg3Sb2, Mg3Sb1.8Bi0.2)/GNS, enhances the electrical conductivity significantly, thereby resulting in a substantially large increase in the power factor. The enhanced electrical conductivity of these nanocomposites is attributed to the increase in the carrier concentration and high carrier mobility owing to the ultra high mobility of graphene. X-ray photoelectron spectroscopy (XPS) core level spectra confirm weak bonding between GNS and Mg3Sb2. Increase in carrier concentration is reflected in XPS valence band spectra and change in spectral weight near valence band maxima is indicative of increased electrical conductivity in the nanocomposite material. The thermal conductivity of these nanocomposites is noted to be reduced at high temperature. These favorable conditions lead to enhanced thermoelectric figure-of-merit (ZT) = 0.71 at 773 K for Mg3Sb2/GNS and a ZT = 1.35 at 773 K for Mg3Sb1.8Bi0.2/GNS nanocomposites with the mass ratio of 80 : 1 which are similar to 170% and similar to 125% higher values compared to bare Mg3Sb2 and bare Mg3Sb1.8Bi0.2 respectively. We strongly believe that the present novel strategy of fabricating such a nanocomposite of a Zintl compound by utilizing GNS as a nanocomposite additive, may provide an emerging path for improving thermoelectric properties of various Zintl phase compounds

Wireless Spying Robot for Supervision

Robot is the most important invention in many walks of life. Robots are invented to reduce human efforts. These Robots are extensively used in defence areas, industries, medical and home applications. It reduces human efforts and time by carrying out many risky jobs that are not possible by humans. This paper presents a Wireless Spying Robot for Supervision which is basically used for defence purpose. The robot is powered by battery. It is operated using wireless signee technology. The controlling device of the whole system is a microcontroller. This robot has metal sensors for detecting mines or bombs along with diffusion facility, fire sensor with pump motor to extinguish fire, gas sensor with buzzer to detect gas, IR sensors for path finding and obstacle avoidance. The system provides continuous visual monitoring through the wireless camera attached to the robot and sends continuous data to the control unit

Synergistic effect on static and dynamic mechanical properties of carbon fiber-multiwalled carbon nanotube hybrid polycarbonate composites

Carbon fiber (CF) and multiwalled carbon nanotube (MWCNT)-reinforced hybrid micro-nanocomposites are prepared through melt mixing followed by injection moulding. The synergistic effect on both the static and dynamic mechanical properties with MWCNT/aMWCNT and CF reinforcement in a polycarbonate matrix is investigated by utilizing dynamic mechanical analysis, and flexural and tensile measurements. The enhancement in the flexural modulus and strength of the composite specimens as compared to pure PC for maximum loading of CF is 128.40% and 39.90%, respectively, which further improved to 142.94% and 42.60%, respectively, for CF-functionalized MWCNTs. Similarly, the storage modulus of the composite specimens reinforced with a maximum loading of CF and CF-functionalized MWCNTs show an increment of 176.57% and 203.33%, respectively over pure PC at 40 degrees C. Various types of parameter such as the coefficient C factor, degree of entanglement and adhesion factor have been calculated to analyze the interaction between fillers and the polymer matrix. Composite specimens containing 2 wt% of functionalized MWCNTs show a lower C value than the as-synthesized MWCNTs, which is indicative of a higher effectiveness of functionalized MWCNT-containing composite specimens. These results are well supported by optical microscopy and Raman spectroscopy by confirming the distribution of reinforcement

Assessment of job stress among non-teaching staff: A cross-sectional study at teaching hospital, Central India

Introduction: Job stress plays an important role in the maintenance of organizational performance. A key aspect of job stress is increased workload, role conflict, poor job control, lack of support from coworkers and interpersonal conflict. Most studies in the field of job stress have focused on private sectors. The objective of this study was to assess the job stress among non-teaching staff at government teaching hospital. Methods: The present cross-sectional study was carried out in a government teaching hospital in central India between Jan 2020 to March 2020 among 90 participants purposively chosen for the study. New Job Stress Scale was used to assess the extent of stress among the participants. Approval from Institutional Ethical Committee was obtained before commencing the study. Data was analyzed by SPSS version 20 for percentages, mean and standard deviation. Results: A moderate level of stress affects 47.78 percent of participants. The present study found that most problematic dimensions of job stress variables were dimension of role conflict with as many as 51% of participants had experience role expectation conflict. Conclusion: We can infer from this finding that we need to have formal job description written for their non-teaching staff so that their role should be clear and unambiguous

Melt-Spun SiGe Nano-Alloys: Microstructural Engineering Towards High Thermoelectric Efficiency

Silicon-germanium (SiGe) alloys are prominent high-temperature thermoelectric (TE) materials used as a powering source for deep space applications. In this work, we employed rapid cooling rates for solidification by melt-spinning and rapid heating rates for bulk consolidation employing spark plasma sintering to synthesize high-performance p-type SiGe nano-alloys. The current methodology exhibited a TE figure-of-merit (ZT) approximate to 0.94 at 1123 K for a higher cooling rate of similar to 3.0 x 10(7) K/s. This corresponds to approximate to 88% enhancement in ZT when compared with currently used radioisotope thermoelectric generators (RTGs) in space flight missions, approximate to 45% higher than pressure-sintered p-type alloys, which results in a higher output power density, and TE conversion efficiency (eta) approximate to 8% of synthesized SiGe nano-alloys estimated using a cumulative temperature dependence (CTD) model. The ZT enhancement is driven by selective scattering of phonons rather than of charge carriers by the high density of grain boundaries with random orientations and induced lattice-scale defects, resulting in a substantial reduction of lattice thermal conductivity and high power factor. The TE characteristics of synthesized alloys presented using the constant property model (CPM) and CTD model display their high TE performance in high-temperature regimes along with wide suitability of segmentation with different mid-temperature TE materials