Healthcare utilization and unmet needs of patients with antisynthetase syndrome: An international patient survey.

Antisynthease syndrome (ASSD) is a rare, complex and understudied autoimmune disease. Internet-based studies can overcome barriers of traditional on-site research and are therefore very appealing for rare diseases. The aim of this study was to investigate patient-reported symptoms, diagnostic delay, symptoms, medical care, health status, working status, disease knowledge and willingness to participate in research of ASSD patients by conducting an international web-based survey. The multilingual questionnaire was created by an international group of rheumatologists and patients and distributed online. 236 participants from 22 countries completed the survey. 184/236 (78.0%) were female, mean age (SD) was 49.6 years (11.3) and most common antisynthetase antibody was Jo-1 (169/236, 71.6%). 79/236 (33.5%) reported to work full-time. Median diagnostic delay was one year. The most common symptom at disease onset was fatigue 159/236 (67.4%), followed by myalgia 130/236 (55.1%). The complete triad of myositis, arthritis and lung involvement verified by a clinician was present in 42/236 (17.8%) at disease onset and in 88/236 (37.3%) during the disease course. 36/236 (15.3%) reported to have been diagnosed with fibromyalgia and 40/236 (16.3%) with depression. The most reported immunosuppressive treatments were oral corticosteroids 179/236 (75.9%), followed by rituximab 85/236 (36.0%). 73/236 (30.9%) had received physiotherapy treatment. 71/236 (30.1%) reported to know useful online information sources related to ASSD. 223/236 (94.5%) were willing to share health data for research purposes once a year. Our results reiterate that internet-based research is invaluable for cooperating with patients to foster knowledge in rare diseases.

Improvement in dyspareunia after vaginal mesh removal measured by a validated questionnaire.

INTRODUCTION AND HYPOTHESIS: The objective was to examine the effect of the surgical removal of vaginally placed prolapse and incontinence mesh on sexual function. We hypothesize that patients with painful complications of mesh will experience improvement in dyspareunia and sexual function after mesh removal. METHODS: The eligible cohort consisted of 133 women who presented with a new onset of pain attributed to mesh-augmented incontinence or prolapse surgery and who elected to undergo mesh removal between 1 August 2012 and 1 July 2013. Sexual function symptoms were assessed before and after mesh removal surgery using the Pelvic Organ Prolapse and Urinary Incontinence Sexual Function Questionnaire short form (PISQ-12). Multivariate analysis was performed to identify predictors of improvement in dyspareunia. RESULTS: Ninety-four patients undergoing mesh removal completed a pre-operative questionnaire, 63 of whom also completed a post-operative questionnaire. After mesh removal, there was a nearly 50% reduction in the proportion of women reporting always experiencing post-operative pain with intercourse among those experiencing pre-operative pain. There was a statistically significant quantitative improvement in pain with intercourse after mesh removal based on mean change score of PISQ-12 question 5 "How often do you experience pain with intercourse?". In multivariate analysis, only history of vaginal delivery was associated with symptom improvement. CONCLUSION: Removal of transvaginal prolapse mesh is associated with improvement in self-reported dyspareunia based on a standardized question on a validated instrument in a small cohort of women. Although larger studies are needed to confirm the relationship between mesh-augmented surgeries and post-procedural dyspareunia, these data suggest that consideration of mesh removal is a reasonable step for patients with painful intercourse attributed to mesh-augmented prolapse and incontinence surgeries.

Seven cases of probable endotoxin poisoning related to contaminated glutathione infusions.

We report seven cases of probable endotoxin poisoning linked to contaminated compounded glutathione. Five of the cases were using the infusions for treatment of Lyme disease highlighting the risks of using compounded sterile preparations for unapproved indications, especially if the quality of source products cannot be assured.

Ketogenic diet in endocrine disorders: Current perspectives.

Ketogenic diet (KD) is a high-fat, adequate-protein, and low-carbohydrate diet that leads to nutritional ketosis, long known for antiepileptic effects and has been used therapeutically to treat refractory epilepsy. This review attempts to summarize the evidence and clinical application of KD in diabetes, obesity, and other endocrine disorders. KD is usually animal protein based. An empiric vegetarian Indian variant of KD has been provided keeping in mind the Indian food habits. KD has beneficial effects on cardiac ischemic preconditioning, improves oxygenation in patients with respiratory failure, improves glycemic control in diabetics, is associated with significant weight loss, and has a beneficial impact on polycystic ovarian syndrome. Multivitamin supplementations are recommended with KD. Recently, ketones are being proposed as super-metabolic fuel; and KD is currently regarded as apt dietary therapy for "diabesity."

Comparative efficacy of four treatment regimens in Type 2 Leprosy Reactions (Prednisolone alone, Thalidomide alone, Prednisolone plus Thalidomide and Prednisolone plus Clofazimine).

This study has been carried out to assess the comparative efficacy of Prednisolone alone or thalidomide alone for the first attack of T2R, and combination of prednisolone plus thalidomide or prednisolone plus clofazimine for chronic and recurrent T2R. Efficacy of all four regimens was assessed on the basis of clinical recovery of T2R measured by reaction severity scores (RSS) ,visual analogue scale (VAS) and other relevant parameters, requirement of extra dose of steroid for clinical recovery, recurrence of T2R and side effects observed in each regimen. The designof study was an open prospective longitudinal single centre investigation. In the first episode T2R group I (prednisolone alone) the efficacy was 58.8% (10/17) compared to 93.75% (15/16) in Group 2 treated with thalidomide alone. This difference was statistically significant p <0.05 when compared using unpaired ttest. When clinical outcome was compared in recurrent/relapse of T2R Groups 3 and 4, it was observed to be statistically significant p<0.05 when compared using unpaired t test. It was 82.35% (14/17) in Group 3 (prednisolone plus clofazimine) and 10/16 (62.5%) in Group 4 (prednislone plus clofazimine). To conclude, for the management of recurrent/chronic ENL, both the combinations-prednisolone with thalidomide or clofazimine appear to be efficacious. While prednisolone with thalidomide appears to be better than prednisolone and clofazimine, however, by 20 weeks difference narrows down. As of now these findings may be considered indicative and larger experience on more number of cases using robust statistical design is required for marchingtowards making recommendations for clinical application.

Laser induced fluorescence of biochemical for UV LIDAR application.

Laser induced fluorescence spectroscopy in the ultraviolet regime has been used for the detection of biochemical through a fiber coupled CCD detector from a distance of 2 m. The effect of concentration and laser excitation energy on the fluorescence spectra of nicotinamide adenine dinucleotide (NADH) has been investigated. The signature fluorescence peak of NADH was centred about 460 nm. At lower concentration Raman peak centred at 405 nm was also observed. The origin of this peak has been discussed. Detection limit with the proposed set up is found to be 1 ppm.

Naloxone blocks the beneficial effects of aqueous extract of Murraya koenigii (L.) Spreng leaves in models of pain.

AIM: This study investigated the antinociceptive effects of aqueous extract of Murraya koenigii (AEMK) leaves (200, 400 and 800 mg/kg, orally) on animal models of acute and persistent pain and its modulation by naloxone. MATERIALS AND METHODS: Antinociceptive effects were assessed using tail-flick, hot plate and formalin tests in mice. To differentiate between central and peripheral antinociceptive effect of AEMK, naloxone (2 mg/kg) was administered along with the 800 mg/kg dose of extract. Morphine was used as a standard drug. RESULTS: AEMK and morphine significantly increased the tail-flick latency (tfl) and paw licking/jumping latency in tail-flick and hot plate tests, respectively, in comparison to control. Also, in both the tests AEMK and morphine significantly increased the AUC0-120 min. In formalin test, AEMK (400 mg/kg and 800 mg/kg) and morphine significantly reduced licking time in both early and late phases in comparison to control. CONCLUSIONS: Thus, in all three pain models AEMK showed antinociceptive effect, which was blocked by naloxone suggesting the involvement of opioidergic central mechanism.

Evaluation of clinical features scoring system as screening tool for influenza A (H1N1) in epidemic situations.

BACKGROUND: Influenza A (H1N1) hit the headlines in recent times and created mass hysteria and general panic. The high cost and non-availability of diagnostic laboratory tests for swine flu, especially in the developing countries underlines the need of having a cheaper, easily available, yet reasonably accurate screening test. AIMS: This study was carried out to develop a clinical feature-based scoring system (CFSS) for influenza A (H1N1) and to evaluate its suitability as a screening tool when large numbers of influenza-like illness cases are suspect. SETTINGS AND DESIGN: Clinical-record based study, carried out retrospectively in post-pandemic period on subject's case-sheets who had been quarantined at IG International Airport's quarantine center at Delhi. MATERIALS AND METHODS: Clinical scoring of each suspected case was done by studying their case record sheet and compared with the results of RT-PCR. RT-PCR was used to confirm the diagnosis (Gold Standard). STATISTICAL ANALYSIS: We calculated sensitivity, specificity, positive and negative predictive values of the clinical feature-based scoring system (the proposed new screening tool) at different cut-off values. The most discriminant cut-off value was determined by plotting the ROC curve. RESULTS: Of the 638 suspected cases, 127 (20%) were confirmed to have H1N1 by RT-PCR examination. On the basis of ROC, the most discriminant clinical feature score for diagnosing Influenza A was found to be 7, which yielded sensitivity, specificity, positive, and negative predictive values of 86%, 88%, 64%, and 96%, respectively. CONCLUSION: The clinical features scoring system (CFSS) can be used as a valid and cost-effective tool for screening swine flu (influenza A (H1N1)) cases from large number of influenza-like illness suspects.

Use of tenecteplase for left-sided prosthetic valve thrombosis.

INTRODUCTION: Prosthetic valve thrombosis (PVT) following cardiac valve replacement in rheumatic heart disease patients is a common cause for referral to tertiary care centre. Although surgery has been described as the traditional choice of therapy, thrombolytic therapy has reported high success rates in published literature. CASE REPORT: This is a case report of ten patients with left-sided PVT receiving tenecteplase. The mean dose of tenecteplase used was 1.01 mg/kg given as IV bolus injection along with enoxaparin, heparin and acenocoumarol. The diagnosis and response was assessed based on trans-thoracic echocardiography. The reduction in peak transvalvular gradient was in the range of 46% - 81% and in mean transvalvular gradient was in the range of 50% - 84%. There was normalization of valve motion in all patients. There was no incidence of mortality, intracerebral hemorrhage, systemic bleeding or embolism. CONCLUSION: To our knowledge, this is the largest published evidence so far showing efficacy and safety of tenecteplase for PVT.

Quantifying spatio-temporal variation in aquaculture production areas in Satkhira, Bangladesh using geospatial and social survey.

Despite Bangladesh being one of the leading countries in aquaculture food production worldwide, there is a considerable lack of updated scientific information about aquaculture activities in remote sites, making it difficult to manage sustainably. This study explored the use of geospatial and field data to monitor spatio-temporal changes in aquaculture production sites in the Satkhira district from 2017-2019. We used Shuttle Radar Topographic Mission digital elevation model (SRTM DEM) to locate aquaculture ponds based on the terrain elevation and slope. Radar backscatter information from the Sentinel-1 satellite, and different water indices derived from Sentinel-2 were used to assess the spatio-temporal extents of aquaculture areas. An image segmentation algorithm was applied to detect aquaculture ponds based on backscattering intensity, size and shape characteristics. Our results show that the highest number of aquaculture ponds were observed in January, with a size of more than 30,000 ha. Object-based image classification of Sentinel-1 data showed an overall accuracy above 80%. The key factors responsible for the variation in aquaculture were investigated using field surveys. We noticed that despite a significant number of aquaculture ponds in the study area, shrimp production and export are decreasing because of a lack of infrastructure, poor governance, and lack of awareness in the local communities. The result of this study can provide in-depth information about aquaculture areas, which is vital for policymakers and environmental administrators for successful aquaculture management in Satkhira, Bangladesh and other countries with similar issues.

N-Methyl-D-aspartate receptor modulators block hyperalgesia induced by acute low-dose morphine.

1. Following opioid-induced antinociception in mice, hyperalgesic responses may be observed. The present study was designed to evaluate the effect of different N-methyl-d-aspartate (NMDA) receptor modulators (magnesium, dextromethorphan, d-serine) on the development of morphine-induced hyperalgesia in mice. The tail-flick test was used to assess the effects of morphine alone and in combination with the NMDA receptor modulators. 2. Administration of a single low dose (2 mg/kg) of morphine to mice produced antinociception that was followed by hyperalgesia. 3. Administration of magnesium sulphate (5 mg/kg) and d-serine (10 mg/kg) alone produced a transient antinociceptive response, whereas dextromethorphan (10 mg/kg) alone produced a prolonged antinociceptive response that had a relatively delayed onset after 4 h. 4. When coadministered with morphine, the NMDA receptor blockers magnesium (2 mg/kg) and dextromethorphan (2 and 5 mg/kg) and the NMDA receptor agonist d-serine (2, 5 and 10 mg/kg), maintained the duration of the antinociceptive response to morphine and inhibited the development of the hyperalgesic response. Coadministration of dextromethorphan (10 mg/kg) with morphine produced antinociception at 30-120 min and at 4-24 h. 5. The results of the present study suggest that coadministration of low-dose NMDA receptor antagonists, as well as the NMDA receptor agonist d-serine, with morphine can inhibit morphine-induced hyperalgesia.

Risk factors for ischemic heart disease in Delhi. (A prospective study of 7000 cases).

BACKGROUND: A prospective study of 7000 consecutive patients with IHD was carried out for "traditional" risk factors at the National Heart Institute (NHI) of the All India Heart Foundation (AIHF) and compared with 1000 "normal" controls. A specially designed proforma was used. STUDY GROUP: There were 5334 men (76%) and 1666 women (24%). The ages ranged from 24 to 92 years; above 60 (35%), between 51-60 (23%), 41-50 (22%) and below 40 (21.7%). The risk factor profile for the whole group was positive family history 38%, sedentary lifestyle 56%, overweight & obesity 21%, hypertension 64%, smoking 43% diabetes 39%, hypercholesterolemia 21% with no obvious risk factor in 15%. Those with no obvious risk factors could have had some of the "new" ones, which were not addressed. 2 & 3 risk factors were present in the majority (5618 patients 80%) and multiple risk factors in 96%. Hypertension was dominant in all groups. WOMEN: Significant differences were more, sedentary lifestyle, little or no smoking, higher cholesterol and more with no obvious risk factors. PRECOCIOUS IHD: In the 22% below 40 years, there was a significantly higher family history and smoking with significant prevalence of smoking, hypertension and diabetes. EXECUTIVE GROUP: The ages ranged from 24 to 59 with none above 60. There was no obvious risk factor in 55%. Here was a positive family history in 30.8%, sedentary lifestyle in 34%, smoking in 24.7%, hypertension in 16.5%, diabetes 11.8% and high cholesterol only in 5.6%. CONCLUSION: Multiple traditional risk factors were dominant in all the groups, with hypertension heading the list. Control of these, should be the target for all preventive programmes, targeting the general population and high risk groups.

Foramen Magnum Chordoid Meningioma in a 22-Year-old Female.

Chordoid meningioma is a rare variety of meningioma (0.5%-1%) with high rate of recurrence. They are grouped in WHO Grade II tumors. They are mainly distributed in the supratentorial location. There has been a single report of foramen magnum chordoid meningioma in a 3-year, 6-month-old child. Our patient, a 22-year-old female, admitted with progressive spastic quadriparesis with bowel and bladder involvement. She was operated with tumor resection done; postoperatively, patient showed dramatic improvement in the motor power as well as bowel and bladder function. Histopathology was suggestive of chordoid meningioma. She received 54 Gy of radiotherapy. She is doing well with no neurological deficits and no recurrence at 2-year follow-up.

A Randomized Study Comparing Endoscopic Third Ventriculostomy versus Ventriculoperitoneal Shunt in the Management of Hydrocephalus Due to Tuberculous Meningitis.

OBJECTIVES: The objective of this study was to compare safety and efficacy of endoscopic third ventriculostomy (ETV) versus ventriculoperitoneal (VP) shunt in the treatment of hydrocephalus in tuberculous meningitis (TBM) and to assess clinical and radiological profiles of patients with TBM that would be better suited to either VP shunt or ETV. METHODS: This study was a single-center randomized prospective study on 52 patients with TBM hydrocephalus in the pediatric age group (<18 years of age). Patients included in the study were randomized into undergo either VP shunt or ETV. Both groups were followed up for a minimum of 5 months and assessed for success and failure rates as well as procedural complications and neurologic sequelae. RESULTS: Twenty-six patients underwent ETV with a success rate of 65.4% with six of nine failures occurring within the first 16 days after surgery (median time to failure - 3 days). In the VP shunt group, there was a success rate of 61.54% and a median time to failure of 50 days. Modified Vellore grading was found to be a significant factor in determining outcome in both ETV and VP shunt groups with high-grade TBM consistently associated with poor outcome (odds ratio = 4.2). CONCLUSIONS: ETV can be performed effectively in young children including infants, as well as those with communicating hydrocephalus, high cerebrospinal fluid (CSF) cell counts, and protein levels with a lower rate of failure than that of VP shunt. Hence, ETV should be attempted as the first-choice CSF diversion procedure in hydrocephalus secondary to TBM where technical expertise and experience with this procedure is available as it avoids the myriad of lifelong complications associated with shunts.

Abnormal Blood Oxygen Level-Dependent Fluctuations in Focal Cortical Dysplasia and the Perilesional Zone: Initial Findings.

BACKGROUND AND PURPOSE: Focal cortical dysplasia is a common cause of intractable epilepsy for which neurosurgery is an option. Delineations of a focal cortical dysplasia lesion on structural brain images may not necessarily reflect the functional borders of normal tissue. Our objective was to determine whether abnormalities in spontaneous blood oxygen level-dependent fluctuations arise in focal cortical dysplasia lesions and proximal regions. MATERIALS AND METHODS: Fourteen patients with focal cortical dysplasia-related epilepsy and 16 healthy controls underwent structural and resting-state functional MR imaging. Three known blood oxygen level-dependent measures were determined, including the fractional amplitude of low-frequency fluctuations, regional homogeneity, and wavelet entropy. These measures were evaluated in the lesion and perilesional zone and normalized to the contralateral cortex of patients with focal cortical dysplasia and healthy controls. RESULTS: Patients showed significantly decreased fractional amplitude of low-frequency fluctuations and increased wavelet entropy in the focal cortical dysplasia lesion and the perilesional zone (≤2 cm) relative to the contralateral homotopic cortex and the same regions in healthy controls. Regional homogeneity was significantly increased in the focal cortical dysplasia lesion compared with the contralateral homotopic cortex and healthy controls. CONCLUSIONS: Abnormalities in spontaneous blood oxygen level-dependent fluctuations were seen up to 2 cm distant from the radiologically visible boundary. It was demonstrated that functional boundaries go beyond structural boundaries of focal cortical dysplasia lesions. Validation is required to reveal whether this information is valuable for surgical planning and outcome evaluation of focal cortical dysplasia lesions and comparing current results with electrophysiologic analysis.

SPARCS and Pelli-Robson contrast sensitivity testing in normal controls and patients with cataract.

PurposeTo determine the ability of the newly developed internet-based Spaeth/Richman Contrast Sensitivity (SPARCS) test to assess contrast sensitivity centrally and peripherally in cataract subjects and controls, in comparison with the Pelli-Robson (PR) test.MethodsIn this prospective cross-sectional study, cataract subjects and age-matched normal controls were evaluated using the SPARCS and PR tests. Contrast sensitivity testing was performed in each eye twice in a standardized testing environment in randomized order. SPARCS scores were obtained for central, right upper (RUQ), right lower (RLQ), left upper (LUQ), and left lower quadrants (LLQ). PR scores were obtained for central contrast sensitivity. PR and SPARCS scores in cataract subjects were compared with controls. Intraclass correlation coefficients (ICC) and Bland Altman analysis were used to determine test-retest reliability and correlation.ResultsA total of 162 eyes from 84 subjects were analyzed: 43 eyes from 23 cataract subjects, and 119 eyes from 61 controls. The mean scores for SPARCS centrally were 13.4 and 14.5 in the cataract and control groups, respectively (P=0.001). PR mean scores were 1.31 and 1.45 in cataract and control groups, respectively (P<0.001). ICC values for test-retest reliability for cataract subjects were 0.75 for PR and 0.61 for the SPARCS total. There was acceptable agreement between the ability of PR and SPARCS to detect the effect of cataract on central contrast sensitivity.ConclusionsBoth SPARCS and PR demonstrate a significant influence of cataract on contrast sensitivity. SPARCS offers the advantage of determining contrast sensitivity peripherally and centrally, without being influenced by literacy.

Cell adherence--a determinant of virulence in Yersinia enterocolitica.

The adherence of pathogenic bacteria to eukaryotic cells plays a central role in their ability to colonize the mucosal epithelial surfaces. The adherence by Y. enterocolitica to the mucosal surface of the gut is the initiating event leading to penetration of mucosa. Adhesion of 10 probable pathogenic and one non-pathogenic isolate was studied using ileum and colon epithelial cells of mouse for 90 minutes. Adhesion study revealed that isolates of Y. enterocolitica had a good adhesive property while non pathogenic showed negligible adherence. All isolates showed better adherence to colon epithelial cells. The organism continued to be excreted in faeces up to 8-10 days after oral feeding. Adhesion positive isolates were found to be virulent when tested in mice for diarrhoea and death. Adhesion was found to be thermoregulated.

Coexistence of superconductivity and ferromagnetism in Sr0.5Ce0.5FBiS2-xSex (x = 0.5 and 1.0), a non-U material with Tc < TFM.

We have carried out detailed magnetic and transport studies of the new Sr0.5Ce0.5FBiS2-xSex (0.0 ≤ x ≤ 1.0) superconductors derived by doping Se in Sr0.5Ce0.5FBiS2. Se-doping produces several effects: it suppresses semiconducting-like behavior observed in the undoped Sr0.5Ce0.5FBiS2, the ferromagnetic ordering temperature, TFM, decreases considerably from 7.5 K (in Sr0.5Ce0.5FBiS2) to 3.5 K and the superconducting transition temperature, Tc, gets enhanced slightly to 2.9-3.3 K. Thus in these Se-doped materials, TFM is marginally higher than Tc. Magnetization studies provide evidence of bulk superconductivity in Sr0.5Ce0.5FBiS2-xSex at x ≥ 0.5 in contrast to the undoped Sr0.5Ce0.5FBiS2 (x = 0) where magnetization measurements indicate a small superconducting volume fraction. Quite remarkably, as compared with the effective paramagnetic Ce-moment (~2.2 µB), the ferromagnetically ordered Ce-moment in the superconducting state is rather small (~0.1 µB) suggesting itinerant ferromagnetism. To the best of our knowledge, Sr0.5Ce0.5FBiS2-x Sex (x = 0.5 and 1.0) are distinctive Ce-based bulk superconducting itinerant ferromagnetic materials with Tc < TFM. Furthermore, a novel feature of these materials is that they exhibit a dual and quite unusual hysteresis loop corresponding to both the ferromagnetism and the coexisting bulk superconductivity.

Juvenile hyaline fibromatosis in siblings.

Juvenile hyaline fibromatosis (JHF) is a rare, autosomal recessively inherited disorder. We report two siblings with multiple large tumors on the scalp, translucent papules on the nape of the neck, hypertrophic gingiva, and severe flexural contractures of large joints. The histopathology from the skin lesions showed features characteristic of juvenile hyaline fibromatosis. The cases are being reported on account of the extreme rarity of the condition.