Does the standard proteinuria cut-off for renal biopsy in lupus nephritis as per the current guidelines hold good for Asian population? A single-centre study from South India.

INTRODUCTION: Current rheumatology and nephrology society guidelines in lupus nephritis do not recommend renal biopsy for proteinuria of less than 500 mg/24 h. This might lead to a significant delay in the early diagnosis of lupus nephritis. AIM: The main aim of this study is to determine the nature of renal lesions in patients with low-grade proteinuria and to analyze the predictors for clinically significant lupus nephritis. METHODS: This was a single-center, retrospective study. All consecutive patients of lupus nephritis, with low-grade proteinuria (200 mg to 500 mg/24 h) undergoing renal biopsy were enrolled in this study. The renal biopsies were classified into significant lesions (Class III/IV/V) and non-significant lesions (Class I and II). Treatment naïve groups and treatment-modified groups were analyzed separately. Predictive factors for significant renal lesions were determined by univariate and multivariate analysis. RESULTS: We identified 183 patients of lupus with proteinuria between 200 and 500 mg / 24 h. Mean (SD) age was 30.2 (11.39) years with 167 (91.2%) of them being females. The mean (SD) baseline proteinuria was 351.03 (98.1) mg/24 h 85 patients (46.5%) had proliferative lupus nephritis where whereas 17 patients (9.3%) had membranous nephropathy. Crescents and fibrinoid necrosis were seen in 10 (5.46%) and 24 (13.11 %) patients respectively. Isolated proteinuria without any other sediments was seen in 95 patients (51.9%) of which 29 patients had proliferative lupus nephritis. Elevated Anti-double stranded DNA (anti-dsDNA), low C3, low C4 and the presence of urinary sediments were significantly associated with significant renal lesions in biopsy. CONCLUSION: Significant renal lesions were seen in around half of the patients with low-grade proteinuria underscoring the importance of performing a renal biopsy in this set of patients. Low C3 and C4, urinary sediments, and elevated anti-dsDNA were predictors for significant renal lesions.

Multiple Tubercular Intestinal Perforations: A Case Report.

Intestinal tuberculosis (TB) is a frequently encountered pathology by surgeons all over India. There exists a vast body of knowledge about this disease; however, a detailed understanding of its presentation as well as surgical management is essential for every Indian surgeon, given its rampant nature. This report discusses the case of a 28-year-old female presenting with severe left upper abdominal pain, non-bilious vomiting, and fever, who was ultimately diagnosed with small bowel TB leading to perforations. Despite a history of pulmonary TB treated a year prior, the patient exhibited significant clinical and imaging findings, including pneumoperitoneum and peritonitis. Exploratory laparotomy revealed multiple tubercular perforations in the mid-jejunum and a stricture causing proximal jejunal dilatation. Surgical intervention involved resection of the affected segment and end-to-end anastomosis. Histopathological analysis confirmed TB as the cause. This case underscores the importance of considering TB in the differential diagnosis of small bowel perforations and highlights the critical role of timely surgical intervention and comprehensive management in improving patient outcomes.

Early diagnosis of lupus: A possibilty. A multicentric study from SLE Special Interest Group (SIG) of Indian Rheumatology Association (IRA).

INTRODUCTION: Systemic Lupus Erythematosus (SLE) warrants an early diagnosis and prompt management. Delay in diagnosis can result in repeated flares, permanent damage, and even death. There is a large variability in the time taken to diagnose SLE across the world. We undertook this study to determine the time taken for diagnosis of SLE in India and to identify the factors associated. METHODS: Patients with SLE diagnosed within the previous 1 year as per Systemic Lupus Erythematosus International Collaborating Clinics criteria (SLICC) 2012 criteria were included in a cross-sectional multicentre questionnaire-based survey. Demographic profile, self-reported socioeconomic status as per Kuppuswamy classification of socioeconomic status (version 2022) (SES), and several healthcare related parameters including referral pattern were recorded. Median time taken for diagnosis was used to demarcate early or late diagnosis and associated factors were explored. RESULTS: We included 488 patients with SLE from 10 rheumatology centres. The median time to diagnosis was 6 months Interquartile Range (IQR 3,14.7) and within 3 months in about one third [150(30.7%)]. Very early diagnosis (<1 month) was established in 78(16.0%) patients. The mean SLE Disease Activity Index (SLEDAI) at diagnosis was 10.28+7.24. In univariate analysis, an older age, lower SES, non-southern state of residence and larger family size were significantly associated with late diagnosis. In the multivariate analysis, higher SES (AOR 0.95, 95% CI: 0.92-0.98), multiple organ system involvement at initial presentation (AOR1.75 95%CI: 1.08-2.84) and place of residence in south Indian states (AOR1.92 95%CI: 1.24-2.97) had lesser odds of being associated with late diagnosis. Distance from the closest medical centre/professional did not influence the time to diagnosis. Majority of patients had first consulted a medical graduate (42.5%) or postgraduate doctor (48.2%), and referral to rheumatologist was largely done by postgraduate (65%) doctors. More than half of our patients (61%) self-finance their treatment. CONCLUSION: Median time to diagnosis of SLE was 6 months, 1/3rd being diagnosed within 3 months and 78(16.0%) with 1 month of symptom onset. Delay in diagnosis was noted in those belonging to lower socioeconomic strata and those with single organ disease. Distance to the health care facility did not influence time to diagnosis.

Comparative Outcomes of Monopolar and Bipolar Transurethral Resection of the Prostate: An Institutional Perspective.

Objective This prospective comparative study aimed to highlight and compare two types of transurethral resection of the prostate (TURP), namely M-TURP (monopolar) and B-TURP (bipolar), in the endoscopic management of benign prostatic hyperplasia (BPH). Methods and materials This research was conducted between 2022 and 2023 at a tertiary care health center. Included in the investigation were 100 consenting study participants undergoing M-TURP and B-TURP at our center. All referred patients presenting with clinical, ultrasound, or uroflowmetry features of BPH and those with failed attempts at medical management were included in the study. Patients with carcinoma of the prostate were excluded from the study. Post-operatively, the endpoints for comparison included maximal urinary flow rate (Qmax), prostate volume, duration of hospital stay, duration of catheterization, drop in serum sodium concentration, and drop in hemoglobin levels. Descriptive statistics were computed to delineate the study sample. After the completion of data collection, data analysis was performed using SPSS for Windows, Version 16.0 (Released 2007; SPSS Inc., Chicago, IL, USA), and the correlations sought were achieved using the Chi-square test of significance. Results The peak incidence of BPH was seen in the sixth decade of life: the M-TURP group was 65.16 ± 7.07 years (mean ± standard deviation), while that in the B-TURP group was 62.32 ± 8.16 years (mean ± standard deviation). Nine percent of the study participants did not show any comorbidities. The most frequent symptom of BPH at presentation was a poor urinary stream (78%, n = 100), followed by nocturia (67%, n = 100). In our study, patients undergoing M-TURP had a mean serum prostate-specific antigen (PSA) level of 4.31 ± 1.03 ng/mL, while patients undergoing B-TURP had a mean serum PSA of 4.24 ± 0.99 ng/mL (p = 0.820; p > 0.05). The study found that patients undergoing M-TURP had a mean prostate size of 35.04 ± 3.57 cc, while those undergoing B-TURP had a mean prostate size of 35.72 ± 3.22 cc (p = 0.765). For the B-TURP group, the mean decrease in postoperative serum sodium concentration was 4.3 mEq/L, while for the M-TURP group, it was 6.4 mEq/L (p = 0.903). In the M-TURP group, there were three cases of transurethral resection (TUR) syndrome, while the B-TURP group had only one case. Conclusion BPH is a common problem affecting the quality of life of several male patients. Both M-TURP and B-TURP are comparable in their efficacy in treating BPH, with the exception of a higher incidence of hyponatremia and TUR syndrome in the M-TURP group.

Beyond paraneoplastic neurological syndromes: Anti-neuronal antibodies in neuropsychiatric systemic lupus erythematosus.

INTRODUCTION: Anti-neuronal antibodies target antigens produced by tumour cells and cells of nervous system. These antibodies are formed as a result of autoimmune response elicited by the underlying malignancy, when proteins restricted to immune privileged neurons are presented by the tumour. Previous studies have shown presence of anti-neuronal antibodies in systemic lupus erythematosus and neuropsychiatric lupus (NPSLE) but information on individual antibodies and their pathogenic role is lacking. AIMS/OBJECTIVE: To assess the frequency of anti-neuronal antibodies in our neuropsychiatric lupus cohort and to assess any significant association with specific neurological syndrome and to see if the antibodies were more likely to occur in active rather than inactive neuropsychiatric lupus. METHODOLOGY: This cross-sectional study was conducted in our center from 2019 to 2022. Neuropsychiatric manifestations were defined according to 1999 American College of Rheumatology (ACR) nomenclature and case definitions for neuropsychiatric lupus. Samples were taken from active or inactive NPSLE patients with their informed consent. Testing was done on an anti-neuronal antigen panel which consisted of [Amphiphysin, CV2, GAD 65, PNMA2 (Ma-2/Ta), Ri, Yo, Hu, recoverin, SOX1, titin, Zic, Tr)] by semi-quantitative Line immune assay. Association between the categorical variables and antibody positivity group was established using chi-square/Fisher's exact test as appropriate. RESULTS: 65 patients were recruited, of which 23 (35%) patients had active NPSLE at the time of sample collection. Anti-neuronal antibodies were positive in 13/65 (20%) patients with anti-Gad 65 antibodies having the highest frequency (6.2%) followed by anti CV 2 (3.1%), anti Sox1 (3.1%), anti Amphiphysin (3.1%) anti recoverin (1.5%), anti Yo (1.5%) and anti Zic (1.5%). The panel of anti-neuronal antibodies did not show any specific association with NPSLE features.However, an interesting finding was that, patients with active disease had higher odds of having anti-neuronal antibodies with an OR = 10 (95% CI:2.38 -42) (p < 0.001) than inactive disease. CONCLUSION: Anti-neuronal antibodies were more likely to be positive in active neuropsychiatric lupus patients, and these antibodies which are commonly used to diagnose paraneoplastic syndromes may have a potential role in the diagnosis of NPSLE.

Reevaluating Diagnosis of Sarcoidosis: Biopsy with Necrosis in Mycobacterial Endemic Areas.

BACKGROUND: Sarcoidosis is a multisystem inflammatory disease with a variable presentation. The most characteristic feature of sarcoidosis is nonnecrotizing granulomas. However, when sarcoidosis presents with rare organ involvement, and biopsy shows necrosis, the diagnosis becomes challenging. CASE PRESENTATION: Here, we present three cases of sarcoidosis with unusual organ involvement and biopsy findings of necrosis, leading to a delay in diagnosis and treatment. Case 1 was presented with lymphoreticular involvement within the intraparotid lymph node and genitourinary area. Biopsy from the epididymis showed necrosis, initially leading to treatment for tuberculosis (TB). Case 2 describes lymphoreticular involvement and cardiac symptoms. His cervical and bone marrow biopsies showed necrosis. Case 3's presentation was disseminated lymphadenopathy with hepatosplenomegaly, initially suspected as malignancy or TB. CONCLUSION: While biopsy plays a significant role in diagnosing sarcoidosis, the presence of necrosis alone should not lead to its exclusion.

Transient perivascular inflammation of the carotid artery (TIPIC) syndrome - a rare differential for anterior neck pain - Series of 3 cases and review of literature.

We report a series of 3 cases of transient perivascular inflammation of the carotid artery (TIPIC) syndrome in an otherwise healthy individual. We would also like to review this rare entity and compare it with other similar cases reported in the literature. Our first case was a young male with right-sided neck pain of 1-week duration with magnetic resonance imaging (MRI) showing right carotid perivascular inflammation which completely resolved after 2 weeks with anti-inflammatory drugs. In the second case, a young male presented with left-sided neck pain and odynophagia of 5 days duration with an MRI showing left carotid perivascular inflammation which completely resolved after 2 weeks with anti-inflammatory drugs. In the third case a young male presented with right-sided neck pain of 1-day duration with an MRI showing right common carotid perivascular inflammation near the bifurcation with complete resolution in pain but with residual wall thickening. We want to highlight the existence of this new entity by reporting these 3 case series with a brief review of the literature. The cause and pathogenesis of this rare entity remain unknown. It has been hypothesized to be autoimmune or viral-mediated inflammation which requires further understanding.

Comparison of rituximab efficacy in treatment-naive and refractory inflammatory myopathies: experiences from a tertiary care centre.

OBJECTIVES: To find out if Rituximab (RTX) is effective in "treatment naive" idiopathic inflammatory myopathies (IIM), and whether there could be differential treatment responses between the "treatment naive" and treatment "refractory" IIM. METHODS: Data obtained from a prospectively maintained database comprising patients with IIM treated with rituximab. Patient details were obtained at baseline, 3-months, 6-months intervals, and subsequent follow up visits. Treatment response was categorised as improved, worsening, or stable based on manual muscle testing (MMT8) scores, patient global and physician global improvement (PtGA and PGA) for skin and joint symptoms improvement and spirometry at 6 months. The time to clinical improvement and remission were noted and survival analysis curves were constructed. RESULTS: 60 patients with IIM (including 18 with anti-SRP myopathy) were included, out of which 33 who received RTX were treatment naïve. The remaining 27 were started on rituximab for refractory myopathy. Mean age was 39 years (SD12.58) in "treatment-naive" group and 43 years (SD 12.12) in "refractory" group. At 6 months of follow up, 48/55 (87%) patients showed response, 31/31 (100%) in "treatment-naive" and 17/24 (70%) in "refractory" cases, p 0.006*. In refractory group, 7 (29%) had stable disease. The mean changes in MMT8 were significantly more in the "treatment-naive" treatment group (13.41(SD 7.31) compared with "refractory" IIM 8.33 (SD 7.92) (p= 0.017*). Majority of patients were able to reduce dose below 5 mg/day before 6 months. No major adverse events were reported over the median follow-up of 24 (IQR 36) months. CONCLUSIONS: Rituximab is effective and safe across the spectrum of IIM. Early use in disease is associated with better outcomes.

Clinical Characteristics and Outcomes of Interstitial Lung Disease in Primary Sjögren's Syndrome: A Retrospective Cohort Study.

Objectives: To describe the characteristics of primary Sjögren's syndrome (pSS) patients with interstitial lung disease (ILD) and to assess treatment response. Methods: All patients of pSS from 2010 to 2019 were retrospectively identified. Lung function tests, high resolution computed tomography (HRCT) findings, and treatment outcomes were analysed. Results: Out of 550 patients with pSS, ILD was detected in 33 patients (frequency of 6 %). The mean(±SD) age at the diagnosis of pSS was 50 (± 9.3) years. 28/33(84.8%) were females. ILD onset preceded pSS diagnosis in 2 (6%) patients, simultaneously diagnosed in 21 (63.6%) patients and developed after pSS onset in 10 (30.3%) patients. 5 patients (15.15 %) were asymptomatic for ILD. Non-specific interstitial pneumonia (NSIP) accounted for the most frequent ILD subtype, in 15 patients (45.5%). Mycophenolate mofetil (MMF) was the most frequently used steroid sparing agent, in 25 patients (75.7%). 7 patients were lost to follow up. Response was seen in 22 patients, whereas 3 patients were non responders. There was one mortality due to lower respiratory tract infection-related sepsis. Presence of sicca symptoms [91.5% vs 8.7% (p<0.001)], NSIP pattern of ILD [90% vs 10% (p = 0.002)], and absence of Raynaud's phenomenon [91.7% vs 8.3% (p<0.001)] were significantly associated with responder status when compared to non-responders. Conclusion: ILD in primary Sjögren's syndrome is not an uncommon entity, and immunosuppression with steroids along with steroid-sparing agents led to good clinical outcomes of ILD in a majority of the patients in our cohort.

Hydroxychloroquine-Induced Phospholipidosis - A Forgotten Complication of a Common Drug.

Hydroxychloroquine (HCQ) has immunomodulatory and immunosuppressive properties and is used in many rheumatological conditions like systemic lupus erythematosus, rheumatoid arthritis, and Sjogren's syndrome. It is usually a widely used and well-tolerated DMARD (Disease Modifying Anti Rheumatic Drugs). Its most feared toxicities include retinopathy and, rarely, cardiomyopathy. Among its other reported side effects is drug-induced phospholipidosis. Here, we report two cases of HCQ-induced phospholipidosis based on renal biopsy electron microscopy. HCQ-induced phospholipidosis, although uncommon, must be considered as one of the differentials in a patient with persistent proteinuria.

Analysis of the Accuracy of the Modified CT Severity Index in Predicting Clinical Outcomes in Acute Pancreatitis: A Cross-Sectional Study.

OBJECTIVE: To evaluate the accuracy of the modified CT severity index (MCTSI) in predicting the severity of acute pancreatitis and to prognosticate the clinical outcomes. METHODS AND MATERIALS: The study was conducted at a tertiary health center between January 2021 and June 2023. A total of 150 consecutive patients with clinical/laboratory features suggestive of acute pancreatitis were included in the study and underwent a contrast-enhanced CT scan within 24 hours of admission. Based on their MCTSI score, these patients had conservative or surgical/endoscopic treatment. Clinical outcomes were assessed in terms of recovery, development of complications, or death. The receiver operating characteristic curve and descriptive statistics were computed to determine the sensitivity and specificity. The data were analyzed using SPSS version 16 software (SPSS Inc., Chicago, IL), and an attempt was made to evaluate the accuracy of MCTSI in predicting these clinical outcomes. RESULTS: The mean age of patients in our study was 49.21 ± 11.02 years. Out of the 150 included patients, 103 were men and 47 were women. Compared to 11.68% of severe acute pancreatitis patients who died, 88.32% recovered. The area under the curve was determined as 0.865, based on which the MCTSI score predicted acute pancreatitis clinical outcome with 64% sensitivity and 92% specificity. The MCTSI demonstrated value in predicting clinical outcomes with a p-value of 0.043 ± 0.012 (p < 0.05) in the recovered patients while p = 0.032 ± 0.012 for patients who succumbed. The p-value for MCTSI in predicting complications was p = 0.0012 ± 0.0008 (p < 0.05). CONCLUSION: Our study was able to demonstrate the high level of accuracy of the MCTSI score in predicting complications and clinical outcomes, especially in patients with severe acute pancreatitis. The MCTSI serves as a valuable asset in the preliminary evaluation of acute pancreatitis, thereby facilitating appropriate management.

An Endosomal Acid-Regulatory Feedback System Rewires Cytosolic cAMP Metabolism and Drives Tumor Progression.

Although suppressed cAMP levels have been linked to cancer for nearly five decades, the molecular basis remains uncertain. Here, we identify endosomal pH as a novel regulator of cytosolic cAMP homeostasis and a promoter of transformed phenotypic traits in colorectal cancer. Combining experiments and computational analysis, we show that the Na+/H+ exchanger NHE9 contributes to proton leak and causes luminal alkalinization, which induces resting [Ca2+], and in consequence, represses cAMP levels, creating a feedback loop that echoes nutrient deprivation or hypoxia. Higher NHE9 expression in cancer epithelia is associated with a hybrid epithelial-mesenchymal (E/M) state, poor prognosis, tumor budding, and invasive growth in vitro and in vivo. These findings point to NHE9-mediated cAMP suppression as a pseudostarvation-induced invasion state and potential therapeutic vulnerability in colorectal cancer. Our observations lay the groundwork for future research into the complexities of endosome-driven metabolic reprogramming and phenotype switching and the biology of cancer progression. IMPLICATIONS: Endosomal pH regulator NHE9 actively controls cytosolic Ca2+ levels to downregulate the adenylate cyclase-cAMP system, enabling colorectal cancer cells to acquire hybrid E/M characteristics and promoting metastatic progression.

Myofibroblast and pro-fibrotic cytokines in fibrosis of IgG4-related disease (IgG4-RD) patients from South Asia: preliminary data.

INTRODUCTION: Fibrosis is a typical pathological characteristic in IgG4-RD patients and often irreversible. There exists a lack of suitable markers for detection of earlier onset of fibrosis in various organs in IgG4-RD patients. Hence, this study aims at analysing ambispectively the myofibroblasts and the pro-fibrotic cytokines, IFN gamma and IL-33 involved in IgG4-RD associated fibrosis in South Asian patients. METHOD: Archived biopsy samples of definite/probable/possible cases of IgG4-RD, classified according to diagnostic criteria, taken from patients who attended the OPD and IPD of our tertiary care centre during January 2015-January 2020 were chosen for this study. The paraffin sections were examined qualitatively for fibrosis and the excessive collagen deposition by Hematoxylin & Eosin and Masson's Trichrome staining. Also, the presence of alpha-Smooth muscle actin (α-SMA) expressing myofibroblasts and the involvement of pro-fibrotic cytokines (IFN-gamma, IL-33) were assessed by Immunohistochemistry and scored semi-quantitatively (+mild, ++moderate, +++ severe). Serum IL-33 levels were analysed by indirect Elisa (R & D Systems). RESULTS: Myofibroblasts were present in 10/12 biopsy samples, in moderate levels in 4 (33%) and very high levels (+++) in 3 (25%) of the patients. IFN-gamma was expressed at low levels in 6 (50%) and absent in 6 (50%). All patients showed IL-33 expression with very high levels in tissue (6, 50%), as well as in serum samples. CONCLUSION: The findings of this study reinforce the role of myofibroblasts and profibrotic cytokines like IL-33 in fibrosis of Ig4-RD patients, pointing to their potential as earlier predictive markers of onset and extent of fibrosis.

Relation of Langerhans cell size to buccal carcinoma.

Oral cancer decreases quality of life despite timely medical management. The carcinogens in tobacco products and their role in tumorigenesis are well documented. Langerhans cells (LCs) are a subset of antigen-presenting cells (APCs) that monitor the tumor microenvironment and engulf carcinogens and foreign bodies. We investigated the distribution and size of LCs and their relation to the mode of tobacco consumption and clinical outcome in patients with buccal carcinoma. We recruited patients with oral cancer who were scheduled for tumor excision and men with urethral stricture undergoing substitution urethroplasty using buccal mucosa. Normal and tumor-adjacent tissues were stained with CD1a antibody. The distribution and mean diameter of 100 LCs/patient were determined. We found significantly smaller LCs in patients who chewed only tobacco compared to those who consumed tobacco by other means. The size of LCs decreased significantly with progressive stages of malignant disease. We found that patients with larger LCs survived longer than those with smaller LCs during an average follow-up of 24 months. We suggest a relation between the size of LCs and clinical outcomes in patients with buccal carcinoma.

Scaffold Proteins in Autoimmune Disorders.

Cells transmit information to the external environment and within themselves through signaling molecules that modulate cellular activities. Aberrant cell signaling disturbs cellular homeostasis causing a number of different diseases, including autoimmunity. Scaffold proteins, as the name suggests, serve as the anchor for binding and stabilizing signaling proteins at a particular locale, allowing both intra and intercellular signal amplification and effective signal transmission. Scaffold proteins play a critical role in the functioning of tight junctions present at the intersection of two cells. In addition, they also participate in cleavage formation during cytokinesis, and in the organization of neural synapses, and modulate receptor management outcomes. In autoimmune settings such as lupus, scaffold proteins can lower the cell activation threshold resulting in uncontrolled signaling and hyperactivity. Scaffold proteins, through their binding domains, mediate protein- protein interaction and play numerous roles in cellular communication and homeostasis. This review presents an overview of scaffold proteins, their influence on the different signaling pathways, and their role in the pathogenesis of autoimmune and auto inflammatory diseases. Since these proteins participate in many roles and interact with several other signaling pathways, it is necessary to gain a thorough understanding of these proteins and their nuances to facilitate effective target identification and therapeutic design for the treatment of autoimmune disorders.

Utility of peripheral blood monocyte subsets, circulating immune complexes and serum cytokines in assessment of SLE activity: an observational, cross-sectional study.

INTRODUCTION: SLE disease measurements by current standards are less than perfect. Monocytes and their subsets are part of innate immunity, and one of our objectives was to look at their role in SLE disease activity. We also looked at the common serum cytokines and the role of circulating immune complex (CIC) estimation in the assessment of disease activity. METHODS: We conducted a single-centre observational cross-sectional study of SLE patients with active and inactive disease as the comparison arms. Blood samples were collected for (a) peripheral blood monocyte separation and flowcytometric analysis of monocyte subsets based on CD14 and CD16 surface markers, and (b) ELISA for serum cytokines and CIC estimation. Results were analysed in terms of the difference in medians between the active and inactive disease groups using the Mann-Whitney U test (non-normally distributed data). RESULTS: The absolute monocyte count was lower in the active group than the inactive group (median (IQR) of 329 (228.5) vs. 628 (257)/microliter, p = 0.001). The frequency (%) of the intermediate monocyte subset showed a trend towards an increase in active disease (median (IQR) of 15.10% (9.65) vs. 11.85% (8.00), p = 0.09). It also had a significant positive correlation to the SLEDAI scores (r = 0.33, p = 0.046). The mean fluorescence intensity (MFI) of CD163, expressed primarily by intermediate subsets, was increased, and CD11c MFI was reduced in active disease. Serum TNF-a level was elevated in active disease (median (IQR) of 38 (48.5) pg/ml vs. 9 (48.5) pg/ml, p = 0.042). CIC ELISA at an optimal cut-off of 10 meq/ml provided an area under the curve (AUC) of 0.85 for detecting active SLE. CONCLUSION: Peripheral blood monocytes are depleted in active disease. The intermediate monocyte subset may have a role in disease activity. TNF-alpha correlated modestly with disease activity. CIC estimation by ELISA may be used in addition to or as an alternative to current standards of laboratory tests for the serological assessment of activity.

Ventriculoperitoneal Shunt Surgery in Brunei Darussalam: A Population-Based Perspective.

Objective Ventriculoperitoneal shunt surgery is commonly performed to treat hydrocephalus. We aimed to characterize the demographics, complications, and outcomes of patients who underwent ventriculoperitoneal shunt surgery in Brunei Darussalam. Materials and Methods We conducted a retrospective study on patients who underwent ventriculoperitoneal shunt surgery at the Neurosurgery Department of the Raja Isteri Pengiran Anak Saleha Hospital and Brunei Neuroscience Stroke and Rehabilitation Centre between January 2015 and June 2020. Statistical Analysis All statistical analyses were performed using the Statistical Package for the Social Sciences version 20 (IBM Corporation, Armonk, New York, United States). The χ 2 test, Student's t -test, and Mann-Whitney U test were performed for nominal, normally, and non-normally distributed variables, respectively. Multivariate logistic regression was used to assess the predictors of complications and shunt failure. Results Fifty-three patients with a median age of 33 (interquartile range, 4-49) years were included. A total of 53 shunt operations were performed: 18 in the pediatric population and 35 in adults. The overall complication rate was 22.6%. Infection was the most common (9.4%) complication, with coagulase-negative staphylococci being the common organism causing the infection. The shunt failure rate (defined as removal or revision) was 20.8%. Univariate and multivariate analyses did not identify factors associated with complications or shunt failure. Conclusion Ventriculoperitoneal shunt surgery, a common and technically non-demanding neurosurgical procedure, is associated with significant complications. We highlighted that shunt infection remains a concern and advocate practices to negate this risk.

Clinical profile of anti-NXP-2 antibody-positive inflammatory myositis and outcome in an Indian population.

INTRODUCTION: Myositis-specific antibodies (MSA) play an important role in the clinical presentation and prognosis of patients with idiopathic inflammatory myositis (IIM). Anti-NXP-2 is one of the newly described MSA. OBJECTIVE: We aimed to describe various clinical presentations associated with anti-NXP2 antibodies and assess response to treatment. METHODS: In this retrospective study, the electronic medical records of all patients who tested positive for anti-NXP2 during June 2019 to April 2022 were screened. Details of demography, clinical presentation, and treatment data were recorded. The anti-NXP2 was tested using the Euro line test kit. Any patient who had an intensity of ≥1+ was considered testing positive. The diagnosis of IIM was reviewed after applying the 2017 European League of Rheumatology (EULAR)/American College of Rheumatology (ACR) criteria of myositis. RESULTS: Among the 660 suspected patients, 470 (71.2%) patients were positive for IIM, and 28 (5.95%) patients were positive for anti-NXP2. From anti-NXP2-antibody positive, 21/470 (4.46%) patients fulfilled criteria for IIM. Among 12 adult (57.14%) patients with IIM, 7 (58.33%) presented as polymyositis (PM) and 5 (41.6%) as dermatomyositis (DM) with median age at presentation of 45 (IQR: 25-58) years. Calcinosis and subcutaneous oedema were observed in 4 (19%) and 2 (9.52%), respectively; myalgia in 6 (28.6%); and distal muscle weakness in 5 (23.8%) patients. Malignancy at the time of diagnosis was observed in two adults with IIM (16.7%), one with DM (intraductal breast cancer), and another with PM (anaplastic large cell lymphoma). Remaining, 9 had juvenile dermatomyositis (JDM) with a median age of 4 (IQR: 3-8) years. Seven (77.8%) patients with JDM had skin rash specific for DM (heliotrope rash and Gottron's papule). None of the patients had cardiac and lung involvement, while GI symptoms, especially dysphagia, were present in 5 (23.8%) patients. During a median follow-up of 19 months (IQR: 12-26 months), 19/19 patients reported improvement and were in remission with treatment. CONCLUSION: The current study shows that adult DM patients with anti-NXP-2 autoantibodies have a unique clinical phenotype. Its presentation differs between adult and JDM, even in different parts of the world. Muscle weakness is mild and responds to treatment. Dysphagia needs more time and aggressive IS for improvement as compared to other muscle involvement. Key Points • Anti-NXP-2 antibody presentation varied from adult to child, as in different parts of the world. • In Indian adult patients, non-specific skin manifestations were more common, whereas in JDM, specific skin features were common. • There was less likely involvement of the lung and heart. But more risk of GI involvement requiring aggressive management. • Adult with anti-NXP-2 antibody should be screened for malignancy at the time of presentation.

Can There be a Biometric Parameter for Voice?-An Investigation Into the Vocal Cry of Twins.

The voice of an individual is the result of interaction between the laryngeal and supra-laryngeal structures of a person. Since the laryngeal and supra-laryngeal structures differ from one person to another, the voice produced by each person becomes unique and recognizably different from another person. If this uniqueness in voice can be found in the acoustic parameters of the voice, it can contribute to the fields of forensic speaker identification and voice biometrics. The best methodology for such a study would be a comparison of the voices of twins who have a number of similar characteristics. OBJECTIVES OF THE STUDY: To find out whether it is possible to perceptually differentiate the cry of identical twins from each other. To compare the acoustic parameters of cry among the twin pairs to find out the differentiating parameter. MATERIALS AND METHODS: This was an observational longitudinal study. Nineteen pairs of twins less than 1 week old who had completed 34 months of gestation period with no major congenital abnormalities were included in the study. The PRAAT software was used for the analysis of the voice samples. RESULTS: The present study on neonates could not identify one single differentiating acoustic parameter among the twins. We infer from the study that rather than an individual parameter, it could be a complex interaction of different parameters that could give individual identity to a person's voice. CONCLUSION: The high similarity noticed in the acoustic parameters among the twin pairs, suggests that rather than individual parameters, it could be a complex interaction of different parameters that could give individual identity to a person's voice. The study can offer information to automatic speaker recognition, Forensic speaker identification, and voice biometry.