British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease.

Sjögren disease (SD) is a chronic, autoimmune disease of unknown aetiology with significant impact on quality of life. Although dryness (sicca) of the eyes and mouth are the classically described features, dryness of other mucosal surfaces and systemic manifestations are common. The key management aim should be to empower the individual to manage their condition-conserving, replacing and stimulating secretions; and preventing damage and suppressing systemic disease activity. This guideline builds on and widens the recommendations developed for the first guideline published in 2017. We have included advice on the management of children and adolescents where appropriate to provide a comprehensive guideline for UK-based rheumatology teams.

Prevalence of FSH-R Asn680Ser and Ala307Thr receptor polymorphism and their correlation with ART outcomes among infertile Indian-Asian women-a prospective cohort study.

The present prospective cohort study evaluated the prevalence of FSH-R receptor Asn680Ser and Ala307Thr among infertile Indian women and the correlation of these polymorphisms with ART outcomes. Total 804 infertile and 209 fertile controls were enrolled for FSH-R analysis. Correlation of different genotypes with ovarian reserve markers, IVF parameters, and cumulative live birth rates (CLBR) was done among women undergoing IVF. In fertile controls, at 680 position GG (Ser/Ser) was the most common genotype; but among infertile women, all the genotypes were equally distributed. There was no significant difference in ovarian response parameters, oocyte yield, and CLBR among the three genotype groups. Empty follicle syndrome (EFS) was highest in women with AA or AG type at both positions. On categorisation of unexpected poor responders according to POSEIDON stratification; GG genotype at both positions had the lowest risk ratio of low-oocyte yield in ART cycles, but these differences were not statistically significant. This is the largest study from Indian ethnicity showing GG (Ser/Ser) genotype is most common among fertile women. The effect of FSH-R genotypes is very marginal on IVF parameters and is not reflected in CLBR. More prospective data may be required on the correlation of these genotypes with genuine EFS, thus stratifying the next cycles with self or donor oocytes. Routine genetic testing of FSH-R polymorphism should not be done except in a research setting. As both 680 and 307 positions are in linkage disequilibrium, only 680 position analysis may be done in a research setting.

Prevalence of vascular thrombosis in patients with liver abscess.

Thrombotic complications in liver abscess are less commonly identified in the literature. In this prospective observational study, vascular thrombosis was detected in 21.9% cases. However, there was no significant difference in outcome with or without vascular thrombosis, properly treated.

Severe Hemolytic Anemia After Mothball Ingestion Complicated by an Undiagnosed Enzyme Deficiency: A Case Report.

Ingestion of mothballs containing naphthalene or paradichlorobenzene is known to cause hemolysis and methemoglobinemia secondary to severe oxidative stress, affecting the oxygen delivery system of the body. The gradual accumulation of oxidizing radicals in the setting of restricted glutathione availability leads to the oxidization of hemoglobin and other body proteins, ultimately causing cell destruction. In the setting of glucose-6-phosphate dehydrogenase deficiency (G6PDD), more pronounced symptoms and poor prognosis are anticipated as adequate nicotinamide adenine dinucleotide phosphate is not generated to protect red blood cells from oxidative injury, potentiating the hemolytic process further. Here, we report the case of a young male with mothball ingestion whose presentation and management were complicated by underlying undiagnosed G6PDD.

Defining anthropometric thresholds (mid-arm circumference and calf circumference) in older adults residing in the community: a cross-sectional analysis using data from the population representative Longitudinal Aging Study in India (LASI DAD).

OBJECTIVES: To identify factors associated with malnutrition (undernutrition and overnutrition) and determine appropriate cut-off values for mid-arm circumference (MAC) and calf circumference (CC) among community-dwelling Indian older adults. DESIGN: Data from the first wave of harmonised diagnostic assessment of dementia for Longitudinal Ageing Study in India (LASI-DAD) were used. Various sociodemographic factors, comorbidities, geriatric syndromes, childhood financial and health status were included. Anthropometric measurements included body mass index (BMI), MAC and CC. SETTING: Nationally representative cohort study including 36 Indian states and union territories. PARTICIPANTS: 4096 older adults aged >60 years from LASI DAD. OUTCOME MEASURES: The outcome variable was BMI, categorised as low (<18.5 kg/m2), normal (18.5-22.9 kg/m2) and high (>23 kg/m2). The cut-off values of MAC and CC were derived using ROC curve with BMI as the gold standard. RESULTS: 902 (weighted percentage 20.55%) had low BMI, 1742 (44.25%) had high BMI. Undernutrition was associated with age, wealth-quintile and impaired cognition, while overnutrition was associated with higher education, urban living and comorbidities such as hypertension, diabetes and chronic heart disease. For CC, the optimal lower and upper cut-offs for males were 28.1 cm and >31.5 cm, respectively, while for females, the corresponding values were 26 cm and >29 cm. Similarly, the optimal lower and upper cut-offs for MAC in males were 23.9 cm and >26.9 cm, and for females, they were 22.5 cm and >25 cm. CONCLUSION: Our study identifies a high BMI prevalence, especially among females, individuals with higher education, urban residents and those with comorbidities. We establish gender-specific MAC and CC cut-off values with significant implications for healthcare, policy and research. Tailored interventions can address undernutrition and overnutrition in older adults, enhancing standardised nutritional assessment and well-being.

Association Between Cognitive Performance and Nutritional Status: Analysis From LASI-DAD.

Malnutrition in low- and middle-income countries causes cognitive decline and other health problems. Harmonized Diagnostic Assessment of Dementia for Longitudinal Aging Study in India (LASI DAD) is an extensive study on late-life cognition and dementia. This study examines the link between nutrition and cognitive abilities in older adults using data from the LASI DAD. We conducted descriptive statistics on nutritional parameters (body-mass index, waist-hip ratio, and Mini-Nutritional Assessment), cognitive functions, and socio-demographic variables in 2,892 adults aged ≥60 years. Cognitive domains assessed included delayed recall, orientation, language, executive function, abstract reasoning, and attention. Cognitive impairment was defined as impaired performance in two or more domains. Mean age was 69.3 ± 7.1 years, 52.9% were female, and 57.5% were illiterate. Low body-mass index (adjusted OR: 1.88, p < .001), at risk of malnutrition (adjusted OR: 1.89, p < .001) and malnourished (adjusted OR: 2.86, p < .001) on Mini-Nutritional Assessment were associated with the presence of cognitive impairment. Better cognitive performance was associated with increased body mass index (adjusted OR: 0.74, p-.03), hemoglobin (adjusted OR: 0.91, p-.006), and serum albumin (adjusted OR: 0.38, p < .001). This study shows that nutritional status assessed by anthropometric measures and blood markers is strongly linked to cognitive performance in older adults.

The association between social media use and body dysmorphic symptoms in young people.

Introduction: Social media use (SMU) is highly prevalent amongst young people and previous research suggests an association with mental health problems, including poor body image. However, the potential relationship between SMU and body dysmorphic disorder (BDD) has received little attention. Furthermore, little is known about the factors that moderate the potential association between SMU and body dysmorphic symptoms. The current study tested the associations between three facets of SMU and body dysmorphic symptoms and explored perfectionism as a moderator in a non-clinical sample. Method: Two-hundred and nine 16-18-year-olds (mean age = 16.5 years, 37% male) recruited from schools in London completed an online survey measuring aspects of SMU, including: frequency of image-and text-based SMU; motivations for SMU (appearance, popularity, connection or values and interests); and active and passive SMU. Participants additionally completed validated measures of body dysmorphic symptoms, perfectionism, and anxiety/depressive symptoms. Linear regression models tested the association of body dysmorphic symptoms with different facets of SMU, with and without adjustment for age, sex and anxiety/depressive symptoms. Results: Frequency of use of image-based, but not text-based, platforms was significantly and positively associated with body dysmorphic symptoms, and this association remained significant in the adjusted models. Appearance-based motivation for SMU was the only motivator uniquely associated with body dysmorphic symptoms across the unadjusted and adjusted models. Passive, not active, SMU was associated with body dysmorphic symptoms in unadjusted models, but this association became non-significant in the adjusted models. Self-oriented perfectionism moderated the association between frequency of image-based SMU and body dysmorphic symptoms. Discussion: Image-based SMU, and appearance-based motivations for SMU, are positively associated with body dysmorphic symptoms. Self-oriented perfectionism may amplify the relationship between SMU and body dysmorphic symptoms. Our findings highlight the importance of a nuanced approach to examining SMU, and the need for further research to determine whether specific facets of SMU contribute to the development and/or maintenance of body dysmorphic symptoms.

Brucellosis Masquerading as Disseminated Tuberculosis: A Clinical Case Report.

Human brucellosis is a common zoonotic disease in certain regions of the world, especially in rural populations, yet it is often neglected. The index patient presented with prolonged fever and headache. Examination revealed hepatosplenomegaly, and investigations revealed transaminitis with bicytopenia with serological evidence positive for Brucella. The radiological picture suggested granulomatous disease with miliary nodules, and a bone marrow examination revealed a noncaseating granuloma. The case was unique and challenging, as the clinical picture was consistent with epidemiologically prevailing tuberculosis, which is also seen in brucellosis-endemic countries. Brucellosis mimicked disseminated tuberculosis, making the case intriguing to diagnose and treat. The case demonstrated that physicians need to have a high index of suspicion for brucellosis, which should be actively investigated in cases with prolonged fever. It also illustrated the fundamental importance of teamwork and the centrality of the multidisciplinary team in the planning and prioritization of treatment.

Role of N-acetylcysteine in liver injury due to dengue fever.

Dengue fever (DF) is a common mosquito-borne viral infection which is endemic in Southeast Asia. Liver involvement may vary from asymptomatic elevation of liver enzymes to fulminant hepatitis. Although the valuable effects of N-acetylcysteine (NAC) in paracetamol toxicity and non-paracetamol liver failure have been extensively studied, its use in DF-associated hepatitis remains unclear. We made a literature search in an online format from libraries such as PubMed, Google Scholar, and EMBASE, and selected 33 articles including original research articles, case reports, and systemic analyses. The majority of the articles reviewed had a positive outcome but treatment strategies involved NAC together with supportive care. Hence, data on sole use of NAC from large randomised control trials remain unclear.

Congenital Bleeding Disorders: Managing Central Nervous System Bleeding in an Adult Hemophiliac.

Congenital bleeding disorders remain a challenge to healthcare in the developing world. Despite the initiation of gene therapy almost five decades ago, the natural history of hemophilia remains the same. The cost of concentrated plasma factors, the development of a high titer of inhibitors in severe hemophilia A (HA), and the associated enhanced propensity of ICH make advancements in disease management questionable. Severe cases of hemophilia die young due to spontaneous central nervous system bleeds due to the lack of standard guidelines for plasma concentrate replacement and the limited availability of products due to the associated economic burden. Monoclonal antibodies, although a promising option as a standardized prophylactic treatment, remain underutilized due to availability and accessibility issues. Here, we report the case of a 28-year-old male with HA who presented to the emergency with a progressively worsening headache, nausea, and elevated blood pressure. He had an intracerebral hemorrhage (ICH) successfully managed with decongestants and factor VIII supplementation.

Deep phenotyping and genomic data from a nationally representative study on dementia in India.

The Harmonized Diagnostic Assessment of Dementia for the Longitudinal Aging Study in India (LASI-DAD) is a nationally representative in-depth study of cognitive aging and dementia. We present a publicly available dataset of harmonized cognitive measures of 4,096 adults 60 years of age and older in India, collected across 18 states and union territories. Blood samples were obtained to carry out whole blood and serum-based assays. Results are included in a venous blood specimen datafile that can be linked to the Harmonized LASI-DAD dataset. A global screening array of 960 LASI-DAD respondents is also publicly available for download, in addition to neuroimaging data on 137 LASI-DAD participants. Altogether, these datasets provide comprehensive information on older adults in India that allow researchers to further understand risk factors associated with cognitive impairment and dementia.

Prevalence of dementia in India: National and state estimates from a nationwide study.

INTRODUCTION: Prior estimates of dementia prevalence in India were based on samples from selected communities, inadequately representing the national and state populations. METHODS: From the Longitudinal Aging Study in India (LASI) we recruited a sample of adults ages 60+ and administered a rich battery of neuropsychological tests and an informant interview in 2018 through 2020. We obtained a clinical consensus rating of dementia status for a subsample (N = 2528), fitted a logistic model for dementia status on this subsample, and then imputed dementia status for all other LASI respondents aged 60+ (N = 28,949). RESULTS: The estimated dementia prevalence for adults ages 60+ in India is 7.4%, with significant age and education gradients, sex and urban/rural differences, and cross-state variation. DISCUSSION: An estimated 8.8 million Indians older than 60 years have dementia. The burden of dementia cases is unevenly distributed across states and subpopulations and may therefore require different levels of local planning and support. HIGHLIGHTS: The estimated dementia prevalence for adults ages 60+ in India is 7.4%. About 8.8 million Indians older than 60 years live with dementia. Dementia is more prevalent among females than males and in rural than urban areas. Significant cross-state variation exists in dementia prevalence.

Intracranial Calcifications in Systemic Lupus Erythematosus.

We present an unusual case of a 37-year-old woman diagnosed with systemic lupus erythematosus presenting with right-sided weakness and altered mentation. On computed tomography and magnetic resonance imaging, marked intracranial calcifications were seen. These localized calcifications are speculated to be secondary to the necrotic focus of repeated episodes of vessel inflammation. However, the pathogenesis of cerebral calcifications is largely unknown.

Mean platelet volume and glycaemic control in patients with new-onset Type 2 diabetes mellitus.

INTRODUCTION: Type 2 diabetes mellitus (T2DM) is a metabolic disorder associated with vascular complications that are attributable to dysregulated platelet reactivity as measured by mean platelet volume (MPV). This study aimed at determining a relationship between MPV and glycaemic control in new-onset T2DM. METHODS: This was a prospective study conducted on 236 new-onset T2DM patients divided in two groups as group A, glycosylated haemoglobin A1c (HbA1c < 7.9%; n = 70) and group B, HbA1c ⩾ 8% (n = 107) who were followed up for 6 months for change in platelet and glycaemic parameters. RESULTS: At 6-month follow-up, there was a significant decrease in HbA1c and MPV (group A (HbA1c: 7.40 ± 0.40 vs 7.03 ± 0.23%, p < 0.03; MPV: 9.65 fl ± 0.74 vs 9.46 fl ± 0.71, p < 0.001) and group B (HbA1c: 10.59 ± 1.89% vs 9.29 ± 1.50%, p < 0.001; MPV: 10.89 fL ± 1.29 vs. 10.23 fL ± 1.40, p< 0.001)). The percentage decline in HbA1c was more in group B (11.81 ± 5.87 vs 4.76 ± 4.58, p < 0.01). There was a positive correlation between ΔMPV and ΔHbA1c in group B; however, we did not observe significant correlation for group A. CONCLUSION: We interpret that in people with diabetes with baseline HbA1c ⩾ 8%, with improvement in glycaemic control, there is a significant decrease in MPV. We propose that a routine MPV testing can be used as a potential marker for glycaemic control in T2DM.

The Long and Short of Summative Assessment in Competency Based Medical Education: Time to Raise the Bar?

BACKGROUND: The Competency-based medical education (CBME) has been introduced for MBBS programme in India from 2019. Reorganization of the assessment system is required to meet the challenges imposed by this new framework. OBJECTIVES: An evaluation of the university summative assessments held prior to the introduction of CBME-based curriculum was carried out to analyze the pattern, relevance and distribution of questions. METHODS: Five sets of annual and supplementary summative examination papers from three universities, State (SU), Private (PU) and Medical (MU) were evaluated. The analysis included format i.e. Structured and Modified Essay questions, Short notes, other formats; marks distribution; terminology-based level of cognitive domain; subject-based relevance and topic-based distribution of questions. RESULTS: A total of 352 questions were analyzed. The maximum number of questions were from the state university (140, 39.7%).The contribution of short notes in the theory papers was 65.8% (PU), 87.1% (SU) and 88.9% (MU).Only the PU had Modified-Essay Questions (10.5%) and Modified short notes(4.7%). Terminology addressing higher cognitive domains was low as the questions assessed mainly the knowledge level (80-96.8%), comprehension 3.1%-6.4%. Only the PU had problem solving questions comprising 11.7% of total questions. Majority of questions had moderate or high relevance and only 2.1%-8.2% were of low-relevance. Inter-university differences in the topics were observed for dermatology, psychiatry, and infections. CONCLUSIONS: The present evaluation of high-stakes assessment in three universities indicates minor differences in the format of questions. Questions were predominantly in the form of short notes, structured essays and modified essays were a rarity; few questions targeted higher cognitive levels although majority had high-moderate relevance.

Scrub typhus manifesting as electrocardiographic disturbance: A case report and review of literature.

Scrub typhus (ST) has wide organ system involvement, but cardiac involvement is paramount in this spectrum due to early hemodynamic compromise. Various forms of cardiac involvement have been described in the literature, but we are describing rare electrocardiographic changes in the ST infection. A young male presented in our emergency department with complaints of restlessness and vomiting of 2 days' duration. The patient was having stable vitals on presentation, but his electrocardiogram (ECG) demonstrated second-degree atrioventricular block type 1 (also known Mobitz 1) and Osborn wave in the precordial leads. Further, he was having thrombocytopenia and eventually diagnosed with ST. For this, appropriate antibiotic treatment was given, which led to considerable symptomatic improvement and reversal of the ECG changes.

Mumps Infection With Symptoms of Parotitis, Pancreatitis, and Orchitis Concurrently in an Adolescent Male.

Mumps is a highly contagious childhood infectious disease caused by the mumps virus. Clinical symptoms of mumps infection among vaccinated young adults are rarely seen. We present an unusual case of a vaccinated young male who presented with a clinical picture suggestive of mumps infection with symptoms of parotitis, pancreatitis, and orchitis. The waning of vaccine-induced immunity and low efficacy of the mumps component of the measles, mumps, and rubella (MMR) vaccine could be the reasons for the same. Our patient was managed with supportive measures for the complications and made an uneventful recovery. It has been postulated that antigenic differences between the vaccine and strain-causing illness may result in a deficient immune response conferred by the vaccine. This case highlights the concerns regarding the effectiveness of the live attenuated vaccine currently in use.

Therapist-supported online cognitive therapy for post-traumatic stress disorder (PTSD) in young people: protocol for an early-stage, parallel-group, randomised controlled study (OPTYC trial).

INTRODUCTION: Post-traumatic stress disorder (PTSD) is a disabling psychiatric condition that affects a significant minority of young people exposed to traumatic events. Effective face-to-face psychological treatments for PTSD exist. However, most young people with PTSD do not receive evidence-based treatment. Remotely delivered digital interventions have potential to significantly improve treatment accessibility. Digital interventions have been successfully employed for young people with depression and anxiety, and for adults with PTSD. However, digital interventions to treat PTSD in young people have not been evaluated. The Online PTSD Treatment for Young People & Carers (OPTYC) trial will evaluate the feasibility, acceptability and initial indications of clinical efficacy of a novel internet-delivered Cognitive Therapy for treatment of PTSD in young people (iCT-PTSD-YP). METHODS AND ANALYSIS: This protocol describes a two-arm, parallel-groups, single-blind (outcome assessor), early-stage randomised controlled trial, comparing iCT-PTSD-YP with a waiting list (WL) comparator. N=34 adolescents (12-17 years old), whose primary problem is PTSD after exposure to a single traumatic event, will be recruited from 14 NHS Child and Adolescent Mental Health Services in London and southeast England, from secondary schools and primary care in the same region, or via self-referral from anywhere in the UK using the study website. Individual patient-level randomisation will allocate participants in a 1:1 ratio, randomised using minimisation according to sex and baseline symptom severity. The primary study outcomes are data on feasibility and acceptability, including recruitment, adherence, retention and adverse events (AEs). The primary clinical outcome is PTSD diagnosis 16 weeks post-randomisation. Secondary clinical outcomes include continuous measures of PTSD, anxiety and depression symptoms. Regression analyses will provide preliminary estimates of the effect of iCT-PTSD-YP on PTSD diagnosis, symptoms of PTSD, anxiety and depression relative to WL. Process-outcome evaluation will consider which mechanisms mediate recovery. Qualitative interviews with young people, families and therapists will evaluate acceptability. ETHICS AND DISSEMINATION: The study was approved by a UK Health Research Authority Research Ethics Committee (19/LO/1354). For participants aged under 16, informed consent will be provided by carers and the young person will be asked for their assent; participants aged 16 years or older can provide informed consent without their parent or caregiver's involvement. Findings will be disseminated broadly to participants, healthcare professionals, the public and other relevant groups. Study findings will be published in peer-reviewed journals. TRIAL REGISTRATION NUMBER: ISRCTN16876240.

Gemcitabine-Induced Hemolytic Uremic Syndrome in Lung Cancer: A Case Report.

Gemcitabine is a broad-spectrum anti-metabolite drug that is widely used in the therapy of numerous advanced cancers such as pancreatic, breast, ovary, lung, and bladder cancer. Gemcitabine has been reported to cause hemolytic uremic syndrome (HUS), but the underlying mechanism is not elucidated. The outcome of gemcitabine-induced HUS is often poor and associated with high mortality. We present a case report of a patient who was on chemotherapy for lung cancer and presented with the concerns of decreased urine output and shortness of breath. He was investigated and found to have HUS. He was managed with plasmapheresis, which resulted in partial recovery. This case report describes HUS caused by gemcitabine in patients with lung carcinoma and the management implemented and also aims to highlight the importance of early and timely recognition and treatment to improve clinical outcomes in these patients.

Early cardiovascular involvement in dengue fever: A prospective study with two-dimensional speckle tracking echocardiography.

Cardiac abnormalities in dengue infection have been conventionally identified by clinical manifestations. The primary objective of our prospective observational study was to assess true cardiovascular involvement and early myocarditis in 150 hospitalised, confirmed cases of dengue fever, through myocardial strain detection using two-dimensional speckle tracking echocardiography. Myocarditis was defined on the basis of European Society of Cardiology (ESC) 2013 criteria. Cardiac biomarkers, namely, creatine phosphokinase myocardial band was elevated in 28.6% and Troponin-T in 23.3% patients. Electrocardiography was abnormal in 64.6% while 6% patients had two-dimensional structural echocardiographic abnormalities. Myocardial dysfunction was suspected in 27.3% based on ESC criteria and strain analysis. The severe dengue group had lower longitudinal strain [-16.4 (6.3)] and circumferential strain [-15.7 (6.7)]. Two-dimensional speckle tracking echocardiography was found to be useful in improving the understanding of early myocardial mechanics in dengue fever.