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Mucopolysaccharidosis VI (MPS VI) is an autosomal recessive inborn error of metabolism caused by mutations in the arylsulfatase B gene (ARSB) and consequent deficient activity of ARSB, a lysosomal enzyme. We present here the results of a study undertaken to identify the mutations in ARSB in MPS VI patients in India. Around 160 ARSB mutations, of which just 4 are from India, have been reported in the literature. Our study covered nine MPS VI patients from eight families. Both familial mutations were found in seven families, and only one mutation was found in one family. Seven mutations were found — four novel (p.G38_G40del3, p.C91R, p.L98R and p.R315P), two previously reported from India (p.D53N and p.W450C), and one reported from outside India (p.R160Q). One mutation, p.W450C, was present in two families, and the other six mutations were present in one family each. Analysis of the molecular structure of the enzyme revealed that most of these mutations either cause loss of an active site residue or destabilize the structure of the enzyme. The only previous study on mutations in ARSB in Indian MPS VI patients, by Kantaputra et al. 2014 [1], reported four novel mutations of which two (p.D53N and p.W450C) were found in our study as well. Till date, nine mutations have been reported from India, through our study and the Kantaputra study. Eight out of these nine mutations have been found only in India. This suggests that the population studied by us might have its own typical set of mutations, with other populations equally likely to have their own set of mutations.

Introduction: Propofol and isoflurane are commonly used in general anaesthesia. Both the drugs are neither neuroprotective nor neurotoxic. In clinical settings, inhaled anaesthetics like isoflurane are frequently used. But, it has been claimed that isoflurane anaesthesia would be a factor inducing cognitive impairment. Propofol is metabolised quickly, primarily in the liver, and its by products are inert. After the initial dose, the half-life of propofol is 2 to 8 minutes, and even with prolonged infusions, propofol promotes quick recovery. Aim: To evaluate the effects of propofol and isoflurane on postoperative recovery patterns in patients receiving general anaesthesia and to determine how they affect cognitive function and memory. Materials and Methods: A double-blinded randomised clinical study was conducted at the Department of Anaesthesia, RL Jalappa Hospital and Research Centre, Tamaka, Kolar, Karnataka, India, during the period from January 2022 to March 2022. In the present study, 60 patients of between age 50-90 years were included. Patients were split into two groups: group A received an intravenous infusion of propofol, and group B received isoflurane. Patients in both groups had their cognitive ability and memory tested before surgery. In the present study, baseline Mean Atrial Pressure (MAP), Heart Rate (HR), Pulse Oximetry (SpO2) and Ramsay Sedation score were comparable in both groups. The novel variables, such as surgery types, duration, and medications were evaluated in both groups. Mini Mental State Examination (MMSE) and Montreal Cognitive Assessment (MoCA) were assessed one hour before and four hours after surgery. Comparison of a continuous variable across the groups was performed using the Student's t-test or Mann-Whitney U test, depending on the normality of the distribution. A comparison of categorical variables across the two study groups was made using the Chi-square test. A p-value of <0.05 was considered statistically significant. Results: Age group of 50-60 years were more in both group A (66.7%), and group B (70%). In Group A, most participants were females (56.7%), and in group B, majority were males (60%). In group A, the postoperative assessment showed a mean MoCA score of 25.6±1.52; in group B postoperative assessment showed a mean MoCA score of 24.17±1.46 with a p-value of 0.001, which was statistically significant. The postoperative assessment showed a mean MMSE score of 26.3±1.58 in group A and in group B, the postoperative assessment showed a mean MMSE score of 24.9±1.4 with a p-value of 0.001, which was statistically significant. Conclusion: The current results imply that postoperative delirium is more frequently present after isoflurane anaesthesia than after propofol anaesthesia. [ABSTRACT FROM AUTHOR]

Multilevel inverters have emerged as a viable alternative for various power electronic applications. It offers significant features in terms of reduced total harmonic distortion (THD) due to more number of output voltage levels, lower filter size, and voltage stress and reduced switching losses when compared to conventional two-level inverters. The classical topologies such as neutral point clamped, flying capacitor and cascaded H-bridge (CHB) are very popular for industrial applications. All these topologies have limitations in terms of more component count, capacitor voltage balancing for increased number of voltage levels. In this regard, multi-level DC link (MLDCL) inverter has been introduced as an improvement with respect to CHB MLI in terms of reduced switch count and is considered here for the study. This paper presents a single-phase MLDC link inverter with a modified carrier-based level-shifted phase disposition sinusoidal pulse width modulation (LS-PD-SPWM) technique. The proposed novel carrier PWM is compared with the conventional modulation techniques in terms of THD. The performance of the proposed modulation technique is analyzed through simulation studies in MATLAB software. A laboratory prototype model is developed in order to validate the experimental results. [ABSTRACT FROM AUTHOR]

Back ground: Humerus is the long bone located in the arm. The Nutrient foramen is an opening in the bone shaft. It allows the passage of nutrient artery which gives nourishment to the bone. The nutrient artery is the principal blood supply for long bones. Supratrochlear foramen is a small hole located at the distal epiphysis of the humerus. The supratrochlear foramen communicates the olecranon fossa with the coronoid fossa & is present over the trochlea of lower end of humerus. The main aim of the study is to determine the location, number and direction of nutrient foramen in relation to the growing end of adult humeri and also to observe the morphological features like incidence and shape of the supratrochlear foramen. Material and Methods: A total number 100 adult humeri were used for this study. The bones were observed for direction, location and number of nutrient foramen and incidence and shape of the supra trochlear foramen. The collected data was analyzed by using the SPSS software and the results were tabulated. Results: In the present study 95% of bones have single, 4% have double and 1% have triple nutrient foramina respectively. The location of nutrient foramina is found to be antero- medial surface in majority of the bones 94%, posterior surface in 4%, anterolateral surface in 1% and absent in 1% of bones. The direction of nutrient foramen is towards the elbow joint. In majority of the bones 91% nutrient foramina were located in the mid third of shaft of humerus, 8% in lower third and 1% in upper third. The supra trochlear foramen is seen in 18% of the bones. Among the 18 bones supra trochlear foramen is round in 10 humeri and oval in 8. Conclusion: The chief blood supply to the shaft of humerus is by a nutrient artery passing through the nutrient foramen. The knowledge of the location and direction of nutrient foramen is useful in surgical procedures done over humerus such as open reduction of fracture humerus, in newer transplantation and bone grafting techniques being attempted by orthopedic surgeons. The incidence of supratrochlear foramen is varies from one population to other. Knowing the incidence in this population will be helpful to the orthopedic surgeons and radiologists for operative procedures and diagnosis. [ABSTRACT FROM AUTHOR]

Introduction: The heart is supplied by right and left coronary arteries. Coronary arteries are physiological end arteries but not anatomical. Coronary arteries are normally epicardial in position except in some cases where a segment of coronary artery is overlapped by a bunch of cardiac muscle fibres. This pattern of arrangement of cardiac fibres over a segment of coronary artery is known as myocardial bridging. Presence of myocardial bridges causes atherosclerotic changes in the pre bridged segment and bridged segment is spared from atherosclerosis. Material and Methods: A total number of 200 heart specimens were observed for this study. A fresh non MLC specimens were included for scanning electron microscopic study. Results: Pre bridged segment of coronary vessel presented the thick tunica intima with atherosclerotic formation. The Polygonal epithelial cells were seen on the surface. In tunica intima plenty of elastic fibres were observed. Tunica media presented a clear muscular component with loose connective. Our present results are correlating with previous histology studies. Conclusion: Myocardial bridges are not an uncommom condition. Many histological studies are available in the review. In our study also we found the same conclusions as histological studies. The pre bridged segment is more prone for atherosclerotic changed than the bridged segment of coronary artery. [ABSTRACT FROM AUTHOR]

Purpose: In view of the raising rate of adverse birth outcomes (ABOs) across the globe, this study was conducted to assess the impact of medical conditions and medications received during pregnancy on ABOs. Materials and Methods: A prospective case–control study was conducted at the Department of Obstetrics and Gynecology of a tertiary care hospital over a period of 3 years from July 2015 to June 2018. Liveborn and stillborn neonates included in the study were categorized into cases and controls based on the presence or absence of composite ABOs, respectively. Binary logistic regression analysis was used to identify the risk factors for ABOs among medical conditions and medications received by mothers during their current pregnancy. Results: Among 1214 neonates included in the study, 556 (45.8%) were identified with composite ABOs, the majority were low birth weight (320 [26.4%]) and preterm birth 300 (24.7%). After adjusting for confounding factors, it was identified that hypertension (adjusted odds ratio [aOR] 7.3), oligohydramnios (aOR 3.9), anemia (aOR 3.2), nifedipine (aOR 10.0), nicardipine (aOR 5.3), and magnesium sulfate (aOR 5.3) were the risk factors for overall and specific ABOs like preterm birth and low birth weight. It was also identified that the early detection and management of hypertension with antihypertensives like labetalol and methyldopa can reduce the risk of preterm birth by 93% and 88%, respectively. Conclusion: Medical conditions such as hypertension, oligohydramnios, and anemia and medications such as nifedipine, nicardipine, and magnesium sulfate during pregnancy were identified as the risk factors for overall and specific ABOs like preterm birth and low birth weight. [ABSTRACT FROM AUTHOR]