Headache as the sole manifestation of Wilson’s disease

Wilson’s disease (WD) is an inborn error of copper metabolism caused by a mutation to the copper-transporting gene ATP7B. In the first decade of life, hepatic involvement predominates but neurological manifestations occur in the third or fourth decades. Studies showed Indian children with neuro WD present with behavior abnormality, speech and cognitive impairment, sub-clinical affection of visual pathway, and autonomic function. As a treatable disease, WD should be detected early in the course of the disease by any health professional at any care level, but the rare prevalence of the disease explains the lack of awareness of this disease. Even a high index of suspicion about this entity gets more difficult when the rare and atypical symptom is the only presentation of the disease. Here, we present the case of a 15-year-old girl with worsening headache for the past 3 months as the only neurological manifestation of WD, and that also without any evidence of hepatic involvement.

Incidence, Diagnosis and Treatment of Otorhinolaryngologicl, Head and Neck Tuberculosis: A Prospective Clinical Study

Abstract Introduction Tuberculosis is a disease of diversified presentation. It affects almost all organs in the body, and otorhinolaryngological, head and neck involvement is not an exception. Objective To increase awareness about the different clinical presentations of otorhinolaryngological, head and neck tuberculosis, the techniques employed to diagnose it, and to assess the response to the treatment. Methods We conducted a prospective study of 114 patients who presented primarily with otorhinolaryngological, head and neck tuberculosis. Routine blood investigations, chest radiographs, the tuberculin test, and sputum examination for the presence of acid-fast bacilli were performed in all cases. Site-specific investigations were performed in relevant cases only. The patients were treated according to the antitubercular treatment (ATT) regimen recommended by the Indian Ministry of Health and Family Welfare's National Tuberculosis Elimination Program (NTEP), and they were followed up clinically two and six months after starting the ATT. Results Tubercular cervical lymphadenopathy was the most common clinical presentation (85.96%), followed by deep neck abscess (5.27%). Fine-needle aspiration cytology proved to be a reliable tool for the diagnosis of tubercular lymphadenopathy. Improvement at the end of 2 and 6 months of the ATT was observed in 90.35% and 96.50% of the cases respectively. Conclusion The diagnosis of otorhinolaryngological, head and neck tuberculosis requires a high index of clinical suspicion, and the ATT proved to be very effective in reducing the severity of the disease.

National guidelines for data quality in surveys: An overview

Good quality health, nutrition and demographic survey data are vital for evidence-based decision-making. Existing literature indicates system specific, data collection and reporting gaps that affect quality of health, nutrition and demographic survey data, thereby affecting its usability and relevance. To mitigate these, the National Data Quality Forum (NDQF), under the Indian Council of Medical Research (ICMR) - National Institute of Medical Statistics (NIMS) developed the National Guidelines for Data Quality in Surveys delineating assurance mechanisms to generate standard quality data in surveys. The present article highlights the principles from the guidelines for informing survey researchers/organizations in generating good quality survey data. It describes the process of development of the national guidelines, principles for each of the survey phases listed in the document and applicability of them to data user for ensuring data quality. The guidelines may be useful to a broad-spectrum of audience such as data producers from government and non-government organizations, policy makers, research institutions, as well as individual researchers, thereby playing a vital role in improving quality of health, nutrition and demographic data ecosystem.

A novel biodegradable polymer scaffold for in vitro growth of corneal epithelial cells

The shortage of donor corneal tissue worldwide has led to extensive research for alternate corneal equivalents utilizing tissue engineering methods. We conducted experiments using Poly D, L lactic acid polymer along with a copolymer (Eudragit) in varying concentrations to create a biodegradable scaffold suitable for in vitro growth of corneal epithelial stem cells. It was found that stable, spherical, and porous microparticles can be prepared by combining PDLLA and Eudragit RL100 polymers in the ratio of 90:10 and 70:30. The microparticles can then be fused to form scaffold membranes with porous architecture and good water retention capacity at room temperature using methanol, which can withstand handling during transplantation procedures. The scaffolds made using a 70:30 ratio were found to be suitable for the promotion of growth of laboratory corneal epithelial stem cell lines (SIRC cell lines). This innovation can pave way for further developments in corneal stem cell research and growth, thus providing for viable laboratory-derived corneal substitutes.

Histological Variants of Leiomyoma: A Great Impersonator posing a Diagnostic Challenge - Three year Study in Delhi NCR

Background: Objectives: Uterine smooth muscle tumours are diagnostically challenging as well as scientifically intriguing with leiomyoma being the most common. On review of literature there are numerous studies on audit of hysterectomy specimens worldwide, however, studies focusing on detailed histopathologi cal analysis of leiomyomas are limited. Thereby, this study was conducted to identify the secondary changes as well as histological variants of leiomyoma, their frequency of occurrence and other associated clinicopathological factors Methods: A retrospecti ve audit of all the hysterectomy and myomectomy specimens was conducted over a period of 3 years (Jan 2016 to Dec 2018) comprising of a total of 155 cases with 388 leiomyomas (LM). The clinical details were retrieved, and histopathological slides reviewed for cellularity, mitosis, atypia, necrosis, any secondary changes or specific variants. Results: On histopathology, 19.1% LM showed degenerative changes and histologic variants were observed in 6.18% LM. Hyaline change was the most frequent degenerative ph enomenon in 82.43% (60/74) while cellular leiomyoma was the most common variant in 37.5% (9/24) LM. Conclusion: Certain histological variants make the differentiation from malignant tumors challenging. A thorough tissue sampling and strict adherence to histologic criteria coupled with a ncillary techniques like immunohistochemistry can help in excluding malignancy in most of the cases. Awareness regarding these is very important amongst youn g pathologists to avoid misdiagnosis and overtreatment leading to unwarranted stress to the patient.

Modified protocol to overcome in vitro recalcitrance of premature micro-shoots abscission by silver supplementation in Kinnow mandarin (Citrus nobilis L. × Citrus deliciosa T.)

The present study focused on alleviating the issue of premature abscission of micro-shoots emerged from the cultured nodal segments in Kinnow mandarin. To resolve the problem, explants of different maturity stages, culture media, growth regulator combinations and ethylene inhibitors (silver compounds) were evaluated. Hardwood stage was observed to be the most efficient explant maturity stage to boost the vigorous early shoot emergence on MS (Murashige and Skoog) medium. Addition of GA3 (Gibberellic acid) 10 mg L?1 resulted in delayed abscission. Further reduction in abscission rate and healthy shoot growth were observed when subculturing of micro-shoots was done on MS medium supplemented with BAP 2.5 mg L?1, GA3 10 mg L?1 and silver thiosulfate 5 mg L?1. This modified procedure could be effectively utilized for mass multiplication as well as crop improvement in Kinnow mandarin.

Clinical and Genetic Profile of Children With Short Stature Presenting to a Genetic Clinic in Northern India

Objective: To define the spectrum of genetic disorders in patients with short stature visiting the genetic out-patient department in a tertiary care hospital. Methods: A chart review was done for 455 individuals (10 months-16 yrs) with short stature, who were evaluated at the genetic clinic from 1 January, 2017 upto 31 October, 2018. 226 patients who needed detailed evaluation, the spectrum of genetic diagnosis is presented. Results: Proportionate short stature was identified in 63% individuals (n=142) of which 93 (65%) were recognizable syndromes such as Turner syndrome, and William syndrome, and RASopathies. In clinically undefined syndromes (39, 27%), a diagnosis could be made by karyotype (n=3/10), chromosomal microarray (6/12) and exome sequencing (1/6). In the 84 children in the disproportionate short stature group (37%), lysosomal storage disorders (LSDs) (45%, n=38) were identified by enzyme analysis in 86.8% and skeletal dysplasias (44%, n=37) identified by skeletal survey in 89% cases. Conclusions: In undefined syndromic short stature, chromosomal microarray may be the first investigation of choice if phenotyping is not suggestive of a specific genetic syndrome. Exome sequencing can be useful in identifying newer genes among idiopathic and familial short stature cohorts.

Neuromyelitis optica spectrum disorder post-COVID-19 infection: A rare case report from Northeast India

Multiple sclerosis and neuromyelitis optica spectrum disorder may be seen in the acute setting of coronavirus disease 2019 (COVID-19) infection or even post-recovery. Such patients may present with optic neuropathy along with weakness in the back and lower limbs. Ascending paralysis can present with respiratory distress in acute COVID-19 infection and may even prove to be fatal. We report a unique case of a 16-year-old female with past history of COVID-19 infection having optic neuropathy, and radioimaging showing demyelinating plaques in the central nervous system with spinal cord edema. Serology showed positivity for rheumatoid arthritis, and the patient was managed with steroids and rituximab.

SOCIAL AND ECONOMIC FACTORS THAT IMPACT CRYPTOCURRENCY

This research paper revolves around several factors that affect cryptocurrency and its efficiency shortly. Many great scholars have talked differently about this concept. To find where cryptocurrency is bound to be, a survey was conducted with necessary questions and a total of 200 responses were received which in turn, helped us to analyze and interpret various economic and social factors impacting its standing in the business sector. Certain limitations and suggestions were generated at the end of the research analysis, followed by the conclusion. It also explores the users' confidence in dealing with cryptocurrency in a time when the usage of such virtual cash was not fully managed and regulated. Besides, the paper is aimed to measure the spread of cryptocurrency use to have a clear photo from the practical view. The paper additionally analyses how certain remarkable international locations have responded in terms of recommendations

Introduction of SDL in Department of Anatomy: Evaluation of Learning and SDL Readiness

Background: Self-directed learning (SDL) is a process in which individuals assume the responsibility of identifying their learning needs, setting goals, locating sources, developing and implementing proper strategies, and evaluating the outcomes of learning both individually and collectively. The advantages of SDL include, but not limited to, gaining more autonomy in learning, having higher motivation for it, acquiring lifelong learning skills, and developing greater self-control, self-confidence, and self-management. SDL has become popular in medical curricula and has been advocated as an effective learning strategy for medical students to develop competence in knowledge acquisition. Aim and Objectives: To introduce self directed learning in the Department of Anatomy, to encourage faculty members to accept SDL as a Teaching Learning method and encouraging students to develop an attitude of self directed learning & to become lifelong learners. Methodology: After proper sensitization of the faculty & students regarding SDL, two sessions were conducted during the Anatomy Dissection Hall timings of the First Year students. The performance of the students, in the SDL topics was assessed using the MCQ & SAQ tests. Feedback regarding the SDL methodology was taken both from the students as well as the faculty. Also the Questionnaires were given to students applying Fischer’s Self Directed Learning Readiness Scale (SDLRS) & Jefferson Scale of Life Long learning –Health Profession Version (JeffSLL-HSP) & the results were statistically analyzed. Results: A total of 126 MBBS students of the first semester participated in SDL session and most students scored more than mean score in the tests after SDL sessions, there was a fourfold increase in the number of students scoring more than 80% in the two SDL tests. Also participating students showed high readiness towards SDL and were oriented to become Life Long Learners. Conclusions: SDL can be effectively implemented in the Department of Anatomy and it motivates students to become life long learners.

Spontaneous intracranial hypotension associated with posterior reversible encephalopathy syndrome

Spontaneous intracranial hypotension (SIH) is usually due to cerebrospinal fluid (CSF) leak, resulting in loss of CSF volume. Posterior reversible encephalopathy syndrome (PRES) is the inability of the posterior circulation of the brain to autoregulate in response to significant variation in blood pressure.Altered perfusion with associated blood-brain barrier disruption may lead to vasogenic edema, usually without infarction, commonly in the parieto-occipital regions. Magnetic resonance (MR) imaging of the brain and spine and cerebral MR venography are essential tools for diagnosing clinically suspected SIH and PRES. We present 18-year-young gentleman with clinical and radiological signs of SIH, which later evolved toward PRES.

Difference of sociodemographic characteristics among the disabled population in Cambodia: a cross-sectional study of the demographic and health survey data / Journal of Rural Medicine

Objective: There is an urgent need to raise awareness of the significance of the social security system for vulnerable populations in developing countries and identify the widening disparities among people with disabilities. This study determined the sociodemographic characteristics of people with disabilities in Cambodia.Materials and Methods: This was a cross-sectional study. Data from the Cambodia Demographic and Health Survey were used to determine the association between disability and sociodemographic characteristics such as age, gender, number of family members, residence (rural/urban), and economic status.Results: The results showed that the proportion of people with disabilities greatly increased with age. The rural-urban residence difference affected the disability proportion in univariate analysis; however, the effect was not significant after adjusting for covariables in multivariate analysis. The odds of having a disability were 0.85 times lower for the high economic status group than for the low economic status group.Conclusion: Raising awareness to expand the capacity of social support for older adults with disabilities, especially those who do not receive care from their families, may be an urgent issue in Cambodia. Therefore, a well-designed and disease-specific study is required. This study was the first to determine the sociodemographic disparities among people with disabilities in Cambodia.

The effect and correlation of smoking with platelet indices, neutrophil lymphocyte ratio and platelet lymphocyte ratio

ABSTRACT Introduction: Smoking is associated with the occurrence and progression of cardiovascular diseases, inflammatory disorders and malignancies. Objective: To study the platelet indices, neutrophil lymphocyte ratio (NLR) and platelet lymphocyte ratio (PLR) in smokers and their correlation with smoking pack-years. Method: A total of 110 smokers and 110 non-smokers were included. The smokers were grouped into three groups: mild (<5 pack-years), moderate (5-10 pack-years) and heavy (>10 pack-years). The platelet count, plateletcrit (PCT), mean platelet volume (MPV) and platelet distribution width (PDW) were noted. The NLR and PLR were calculated and the statistical analysis was made using the Student's T-test, Analysis of Variance (ANOVA) and Spearman's correlation coefficient. Results: The platelet count, PCT and PDW were significantly higher with mean values: 218.56 ± 121.31 vs 203.23 ± 80.35 (p-value = 0.038), 0.27 ± 0.10 vs 0.26 ± 0.10 (p-value = 0.041) and 12.54 ± 1.45 vs 11.99 ± 1.70 (p-value = 0.001) in smokers and non-smokers, respectively. The PLR differed significantly with mean values: 119.40 ± 84.81 in smokers and 181.99 ± 313.09 in non-smokers, with a p-value of 0.045. A significant positive correlation was found between pack-years of smoking and platelet count and PLR with the Pearson correlation coefficient of 0.250 and 0.198 and p-values, 0.008 and 0.037, respectively. The Platelet Count, PCT, MPV and PDW varied significantly between mild, moderate and heavy smoker groups, with p-values of 0.045, 0.010, 0.015 and 0.017, respectively. Conclusion: The platelet indices and inflammatory markers NLR and PLR are derived from routine blood investigations, which are easily available and inexpensive. The monitoring of platelet indices, along with the PLR, can be used as early predictors of morbidity in smokers.

Associations between countertransference reactions towards patients with borderline personality disorder and therapist experience levels and mentalization ability

Abstract Objective This exploratory study locates countertransference as a pan-theoretical concept, comprising of thoughts, feelings, and behaviors expressed or experienced by therapists toward their patients. It aims to understand the patterns of countertransference experienced in working with borderline personality disorder. Associations between countertransference reactions and therapist-related variables of experience and mentalization ability are also examined. Method Psychotherapists (n = 117) completed the Therapist Response Questionnaire to assess patterns of countertransference experienced with a representative patient diagnosed with borderline personality disorder. They also completed a measure of mentalization ability that examined self-related mentalization, other-related mentalization, and motivation to mentalize. Results The profile of responses across eight countertransference dimensions is discussed, with the most strongly endorsed reactions being positive/satisfying, parental/protective, and helpless/inadequate. More experienced therapists reported less negative countertransference reactions in select dimensions. Therapists' self-reported ability to reflect on and understand their own mental states was negatively correlated with a range of difficult countertransference experiences. There were few associations between their ability to make sense of others' mental states, the motivation to mentalize, and the strength of their countertransference reactions. Conclusion The implications for countertransference management as well as therapist training and development are highlighted.

Gut microbiome and checkpoint inhibitor colitis

Immune checkpoint inhibitor therapies such as ipilimumab, are increasingly being used as a treatment option for a variety of cancers, including metastatic melanoma and have demonstrated effectively a prolonged survival. These agents have an immunological mode of action that predisposes patients to a number of immune-related adverse events, colitis being one of the most commonly encountered complications. The pathogenesis for the development of colitis is unclear, and there is a growing consensus that the ecosystem of the gastrointestinal microbiota plays a significant role. Based on this suspected connection, studies are being carried out to explore the changes in the microbiota in patients on these medications who develop colitis. Conceivably, the modulation of the gut microbiota could offer a therapeutic benefit. Fecal microbiota transplantation is one therapeutic option that is currently being investigated, though there are still more data needed to evaluate its efficacy. In this review, we recapitulate the mechanisms of action of immune checkpoint inhibitors, their adverse events, with a focus on colitis and the role gut microbiota are suspected to play, and finally discuss the microbiota modulation therapies being investigated.

In Silico Screening of DNA Gyrase B Potent Flavonoids for the Treatment of Clostridium difficile Infection from PhytoHub Database

Abstract Clostridium difficile infection (CDI) is the most common hospital acquired diarrheal disease with its increasing incidence and mortality rate globally. DNA Gyrase B (GyrB) is a key component of DNA replication process across all bacterial genera; thus, this offers a potential target for the treatment of CDI. In the present study, several virtual screening approaches were employed to identify a novel C. difficile GyrB inhibitor. The 139 known metabolites were screened out from the 480 flavonoids in PhytoHub database. Molinspiration and PROTOX II servers were used to calculate the ADME properties and oral toxicity of the metabolites, whereas mutagenicity, tumorigenicity, irritant, and reproductive effect were predicted using DataWarrior program. The binding mode and the binding efficiency of the screened flavonoids against the GyrB were studied using FlexX docking program. From virtual screening of 139 metabolites, we found 25 flavonoids with no mutagenicity, tumorigenicity, irritant, and reproductive effect. Docking study suggested that flavonoids 1030 ((-)-epicatechin 3'-O-sulfate), 1032 ((-)-epicatechin 4'-O-sulfate), 1049 (3'-O-methyl-(-)-epicatechin 4-O-sulfate), 1051 (3'-O-methyl-(-)-epicatechin 7-O-sulfate), 1055 (4'-O-methyl-(-)-epicatechin 7-O-sulfate) and 1317 (quercetin sulfate) have significantly higher binding affinity than the known GyrB inhibitor novobiocin. The results from molecular dynamics simulation and free energy calculations based on solvated interaction energy suggested that (-)-epicatechin 3'-O-sulfate could be a potential drug candidate in the management of CDI.

Systemic Scleroderma with Complete Heart Block

For patients with Systemic Sclerosis (SSc), cardiac involvement (CI) is directly caused by myocardial fibrosisor ischemia or is secondary to pulmonary arterial hypertension1 Here we present a similar case of a femalepatient aged 45 yr old female who presented with complaint of recurrent syncopal attacks, and difficulty inswallowing food and liquids. She had typical CREST Syndrome manifestations with complete heart block(CHB). For which pacemaker implantation was done.