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Precast prestressed concrete girders are widely used in U.S. bridge construction. With advancements in high-performance concrete and new girder designs, these girders are now capable of achieving significantly longer spans. Such spans often require deeper girders and an increased number of prestressing strands. The resulting bursting forces at the girder ends at time of prestress release may cause end zone cracking, especially horizontal web cracks, which can compromise the durability of the girders and potentially lead to rejection by bridge owners. Current practices focus on mitigating these cracks by providing adequate end zone reinforcement, but completely eliminating them remains a challenge, as girders are typically prestressed along their length only. This paper proposes an innovative approach to combat end zone cracking through the application of vertical prestressing at the girder ends using shape memory alloys (SMAs). This method involves heating prestrained SMAs at the beam ends to induce recovery stress, thus generating vertical prestress that enhances splitting resistance and reduces web cracking. The research employed a variety of NiTi SMA reinforcement, including wires, strands, and cables, demonstrating the feasibility of this method through both small-scale and full-scale beam tests. The beam tests demonstrated the shape memory effects of SMAs and the impact of induced prestressing. The findings suggest that properly designed vertical prestress can effectively counteract bursting forces and mitigate concrete cracking.

Purpose – The main aim of this study is to explore the appropriate factors in measuring COVID-19-induced supply chain disruptions and the impact of these disruptions on the economic vulnerability of small-scale farmers in Sri Lanka. Findings – The findings revealed that most of the farmers have continued to cultivate even during the pandemic despite several challenges which affected their economic status. Therefore, it is concluded that COVID-19-induced transportation and demand disruptions exacerbated the economic vulnerability of small-scale farmers over the disruptions in supply and production. Practical implications – The findings of this study are crucial for formulating novel policies to improve the sustainability of the Sri Lankan agricultural sector and alleviate the poverty level of Agri-communities in the countryside. As farming is a vital sector in the economy, increased attention ought to be given on facilitating farmers with government-encouraged loans or allowances for their financial stability. Further, the respective government authorities should develop programs for importing and distributing adequate quantities of fertilizers among all the farmers at controlled prices so that they can continue their operations without any interruption. Moreover, the government could engage in collaboratively work with private organizations to streamline the Agri-input supply process. There should be a government initiative for critical consideration of the issues of farming families and their continued motivation to engage in agriculture. Thus, farmers' livelihoods and agricultural prosperity could be upgraded through alternative Agri-inputs and marketing strategies, providing financial assistance, encouraging innovative technology, etc. Originality/value – Despite the significance and vulnerability of the vegetable and fruit sector in Sri Lanka, there is a limitation in the empirical studies conducted on the supply chain disruptions caused by COVID-19 measures and their implications on the farmers' livelihood. Furthermore, previous empirical research has not employed adequate quantitative tools to analyze the situation or appropriate variables in evaluating COVID-19-induced disruptions. Hence, the current study explored the appropriate factors for measuring COVID-19-induced supply chain disruption using exploratory factor analysis. Then, the impact of those factors on the economic vulnerability of the small scale farmers was revealed through the ordinal logistics regression analysis.

Annona fruit, leaves, seeds, roots, and bark have been conventionally used in many countries for medical treatments as they are considered ideal sources of pharmacologically active compounds, but Annona remains an underutilized fruit in many countries. The fruit of these plants is delicately flavored and is used in industrial products such as ready-to-serve beverages, wine, jellies, jam, and fruit-butter preserve, while the seeds generally go to waste. Annona seed oil contains numerous health-benefiting factors such as vitamins, minerals, bioactive compounds, fatty acids, antioxidants, and phenolic compounds, which are responsible for various biological activities, including antibacterial, antioxidant, and antitumor activities. Cancer is a worldwide major health problem that remains unresolved. Even though the current treatments can manage to reduce tumor growth, there is an urgent need to investigate more efficient but less expensive novel techniques to overcome some of the restrictions in treating tumors. Annona might offer an indispensable choice besides chemotherapy and radiotherapy, especially for terminally ill patients, as the Annona genus contains secondary metabolites in nearly every component of Annona plants. Research has shown that many Annona species contain promising components that could potentially exhibit anticancer activity, but the information available is scarce and inconsistent. Annona muricata (Soursop, “Katuanoda”), Annona squamosa (Sweetsop, “Seenianoda”), and Annona reticulata (Custard apple, “Welianoda”) are three commonly cultivated edible Annona species in Sri Lanka. The main objective of the review was to present an updated comprehensive literature analysis of the putative chemopreventive functions against cancer cell lines/the anticancer effect on cancers, phytochemical properties, and antioxidant properties possessed by the seed oils of three selected common Annona species. Although there are some in vitro and in vivo experimental investigations supporting the benefits of Annona seed oils, clinical investigations are still needed to explore concealed areas, determine the effects on the human body, determine the safest concentration, and determine health-contributing benefits before they are submitted to clinical trials.

Leptospirosis is a re-emerging zoonotic disease. This article reports the complete genome sequences of three novel strains of Genus Leptospira: two from the species Leptospira weilii (FMAS_RT1, FMAS_PD2) and one from Leptospira kirschneri (FMAS_PN5). These isolates were recovered from the blood samples of acute febrile patients in different geographical and climatic zones of Sri Lanka. High-quality genomic DNA was extracted from the three isolates in mid-log phase cultures. Whole genome sequencing was conducted using the PacBio Single Molecule Real-Time (SMRT) platform to identify the species, genome features, and novelty of the strains. The annotation was conducted using RAST (Rapid Annotation Using Subsystem Technology version 2.0) and the NCBI Prokaryotic Genome Annotation Pipeline. The genome sequences of three isolates have been deposited in the Mendeley data repository and the National Center for Biotechnology Information (NCBI) repository. This data will be useful for future researchers when conducting comparative genomic analysis, revealing the exact mechanism of pathogenesis of leptospirosis and developing molecular diagnostic tools for early detection.

Stock price data often exhibit nonlinear patterns and dynamics in nature. The parameter selection in generalized autoregressive conditional heteroskedasticity (GARCH) and autoregressive integrated moving average (ARIMA) models is challenging due to stock price volatility. Most studies examined the manual method for parameter selection in GARCH and ARIMA models. These procedures are time-consuming and based on trial and error. To overcome this, we considered a GWO method for finding the optimal parameters in GARCH and ARIMA models. The motivation behind considering the grey wolf optimizer (GWO) is one of the popular methods for parameter optimization. The novel GWO-based parameters selection approach for GARCH and ARIMA models aims to improve stock price prediction accuracy by optimizing the parameters of ARIMA and GARCH models. The hierarchical structure of GWO comprises four distinct categories: alpha (α), beta (β), delta (δ) and omega (ω). The predatory conduct of wolves primarily encompasses the act of pursuing and closing in on the prey, tracing the movements of the prey, and ultimately launching an attack on the prey. In the proposed context, attacking prey is a selection of the best parameters for GARCH and ARIMA models. The GWO algorithm iteratively updates the positions of wolves to provide potential solutions in the search space in GARCH and ARIMA models. The proposed model is evaluated using root mean squared error (RMSE), mean squared error (MSE), and mean absolute error (MAE). The GWO-based parameter selection for GARCH and ARIMA improves the performance of the model by 5% to 8% compared to existing traditional GARCH and ARIMA models.

ABSTRACT Pathogenic Leptospira, the causative agents of leptospirosis, comprise >200 serotypes (called serovars). Most have a restricted reservoir-host range, and some, e.g., serovar Copenhageni, are cosmopolitan and of public health importance owing to their propensity to produce severe, fatal disease in humans. Available serotyping approaches—such as multilocus sequence typing, core genome sequence typing, pulsed-field gel electrophoresis, and the cross-agglutination absorption test—are tedious and expensive, and require isolation of the organisms in culture media—a protracted and incredibly inefficient process—precluding their use in prospective studies or outbreak investigations. The unavailability of culture-independent assays capable of distinguishing Leptospira serotypes remains a crucial gap in the field. Here, we have developed a simple yet specific real-time qPCR assay—targeting a Leptospira-unique gene encoding a putative polysaccharide flippase—that provides intraspecies, serotype-defining (i.e., epidemiologically useful) information, and improves upon the sensitivity of preferred lipL32-based qPCR-based diagnostic tests. The assay, dubbed RAgI (“rage one”), is rapid and affordable, and reliably and specifically detects group I pathogenic Leptospira in culture, serum, and urine, with no detectable off-target amplification—even of the genetically related but low virulence group II pathogenic (formerly “intermediate”) or nonpathogenic Leptospira. It retained 100% diagnostic specificity when tested against difficult sample types, including field-collected dog urine samples and environmental samples containing varied and complex microbial species-consortia. This assay holds considerable promise in the clinical setting, and for routine epidemiological and environmental surveillance studies. IMPORTANCE Leptospirosis is caused by a diverse group of pathogenic spirochetes comprising over 200 different serotypes. Some are widely reported and of public health importance owing to their propensity to produce severe, fatal disease in humans. Apart from their tedium and expense, current serotyping approaches require isolation of the organisms in culture media—a protracted and incredibly inefficient process—rendering them useless clinically and limiting their utilization in prospective studies or outbreak investigations. The unavailability of culture-independent assays capable of distinguishing Leptospira serotypes remains a crucial gap in the field. The 11108 qPCR-assay overcomes this barrier to progress via direct taxonomic and serotype classification of Leptospira from urine and serum samples, and hence, is the first qPCR-based prognostic test for human leptospirosis.

This dataset includes data from febrile patients recruited for a large hospital-based study in Sri Lanka from 2016 to 2019. The variables include primary socio-demographic data, exposure data, clinical data, biochemical and investigation data. Some of these data are available as serial data from admission to discharge daily. Microscopic agglutination test, quantitative PCR of whole blood, urine and serum and culture isolation was performed to diagnose the patients with leptospirosis.

Land use of the sub-Himalayan region is not that intensive like the intensively land-managed region of Punjab, India. Land resources of the sub-Himalayas must be managed effectively for sustainable development by preparing carbon inventories and data banks. Such macro-level studies have not been conducted yet in the present study area, and thus were conducted to suggest sustainable land use management options. To achieve the present study’s desired goal, 33 tree-based land uses were identified from forested and agricultural landscapes of the sub-humid tropical region of West Bengal, India. Stratified random nested quadrat sampling was adopted for the study. The SOC, biomass, and carbon accumulation significantly differed. Mixed forests had the highest soil primary nutrients and carbon stock. Positive correlations were observed between SOC, total standing biomass, litter production, and ecosystem carbon. The sequence of land uses based on carbon stock was mixed-species forest > sole tree species stands in a forest landscape > tea plantations > homegardens. This baseline information can be used for developing prediction models for future interventions towards sustainable land management. The study, however, could not estimate the carbon fluxes in and out of the systems due to the absence of detailed land use land-cover databases.

Venom peptides have been evolving complex therapeutic interventions that potently and selectively modulate a range of targets such as ion channels, receptors, and signaling pathways of physiological processes making it potential therapeutic. Several venom peptides were deduced in vivo for clinical development targeting pain management, diabetes, cardiovascular diseases, antimicrobial activity. Several contributions have been detailed for a clear perspective for a better understanding of venomous animals, their venom, and their pharmacological effects. Here we unravel and summarize the recent advances in wide venom peptides across varieties of species for their therapeutics prospects.

BackgroundLeptospirosis has globally significant human mortality and morbidity, yet estimating the clinical and public health burden of leptospirosis is challenging because timely diagnosis remains limited. The goal of the present study was to evaluate leptospirosis undercounting by current standard methods in both clinical and epidemiological study settings.Methodology/principal findingsA prospective hospital-based study was conducted in multiple hospitals in Sri Lanka from 2016 to 2019. Culture, whole blood, and urine samples were collected from clinically suspected leptospirosis cases and patients with undifferentiated fever. Analysis of biological samples from 1,734 subjects confirmed 591 (34.1%) cases as leptospirosis and 297 (17.1%) were classified as "probable" leptospirosis cases. Whole blood quantitative PCR (qPCR) did identify the most cases (322/540(60%)) but missed 40%. Cases missed by each method include; urine qPCR, 70% (153/220); acute sample microscopic agglutination test (MAT), 80% (409/510); paired serum sample MAT, 58% (98/170); and surveillance clinical case definition, 53% (265/496). qPCR of negative culture samples after six months of observation was of diagnostic value retrospectively with but missed 58% of positives (109/353).ConclusionLeptospirosis disease burden estimates should consider the limitations of standard diagnostic tests. qPCR of multiple sample types should be used as a leading standard test for diagnosing acute leptospirosis.

In this study, the objective was to develop hybrid composites combining the semi-crystalline engineered polysaccharide α-1,3-glucan (Nuvolve™) with typical long glass fiber in a polypropylene matrix to optimize specific indicators of performance while also considering environmental attributes. Morphological analyses were conducted in conjunction with the evaluation of mechanical performance of these hybrid composites to gain further understanding of filler-matrix interaction. Optimum loadings of the polysaccharide / glass fiber system were identified as promising alternative to the current glass fiber / polypropylene incumbent material utilized in many commercial application (e.g. by Ford Motor Company for body interior and under-the-hood applications). Formulations with 10/15 (e.g. 10 wt.% polysaccharide and 15 wt.% glass fiber) or 10/20 showed an overall increase of >100% with respect to modulus, strength and impact properties while also demonstrating a density reduction of up to 13%. Interestingly, the life cycle analysis showed that addition of only 10 wt.% polysaccharide was able to save 4,720 liters of fuel for every ton of polysaccharide used in vehicles. Therefore, hybrid reinforced thermoplastic composites offer a balance of engineering and environmental performance to exceed materials in use. These new composites are commercially viable while also advancing the environmental stewardship and eco-efficiency within the automotive industry.

Elaeocarpus ganitrus Roxb. commonly identified as ‘rudraksha’, belonging to Elaeocarpaceae possess a great religious, spiritual and materialistic significance which is placed under the threatened category in the tropical wet evergreen forests of North East India. Because of its over-exploitation, poor germination, low viability rate and hard seed coat, an attempt has been done for mass production of planting materials through cuttings and air layering’s. Cuttings trials were conducted during three different periods, mid - March, mid – July and mid – November by using Indol butyric acid and Napthalene acetic acid of 1000, 2000, 4000, 6000 ppm where air layering’s were done during mid - May by treating IBA and NAA solution of 500, 1000 and 2000 ppm in combination with para-hydroxybenzoic acid of 2000 ppm as synergist. Application of IBA and NAA of different concentration did not encourage the growth in the cuttings in all three periods. Air layering’s treated with NAA 1000 ppm+2000ppm para- hydroxy benzoic acid and IBA 500 ppm +2000ppm para- hydroxy benzoic acid resulted the highest and same value of callusing and rooting percent (70 and 66.67%), respectively while minimum callusing and rooting percentage (56.67 and 51.67%) was recorded in control. The present study focused on the mass production of planting materials in shorter duration with a reasonable cost for enhancing production as well as socio-economic conditions.

Coronal mass ejections (CMEs) are subject to changes in their direction of propagation, tilt, and other properties. This is because CMEs interact with the ambient solar wind and other large-scale magnetic field structures. In this work, we report on the observations of the 2012 October 5 stealth CME using coronagraphic and heliospheric images. We find clear evidence of a continuous rotation of the CME, i.e., an increase in the tilt angle, estimated using the graduated cylindrical shell (GCS) reconstruction at different heliocentric distances, up to 58 R _⊙ . We find a further increase in the tilt at L1 estimated from the toroidal and cylindrical flux rope fitting on the in situ observations of interplanetary magnetic field (IMF) and solar wind parameters. This study highlights the importance of observations of Heliospheric Imager (HI), on board the Solar Terrestrial Relations Observatory. In particular, the GCS reconstruction of CMEs in the HI field of view promises to bridge the gap between the near-Sun and in situ observations at the L1. The changes in the CME tilt have significant implications for the space weather impact of stealth CMEs.

The rate of suicide is far more prevalent than reported in India and over the last few decades. The main objective of this study is to establish the correlation of selected socio-economic determinants for suicide and its link to the caste issue. This is a cross-sectional study based on police records in Mysore City, Karnataka between the years 2017 to 2021. In the study period, 923 suicide cases were registered, of which 57% were from urban areas and 43% were from rural areas. Mental illness, loan and health issues are the major causes of suicide. The 21-30 age groups had the highest rate (32.5%) of suicide suspecting quarter-life crisis in contrast to the national crime record bureau report. Male victims have outnumbered women. The study found socioeconomic factors, like low schooling (24%), unskilled job (43%), poor income (74%), are significantly linked to higher suicide rates. As a first time it is noted that the highest rate of suicide among excluded castes (79.5%). Well-off castes have shown a low rate of suicide (12%). The study concludes that suicide is connected with the disadvantaged caste factor and poor socio-economic deterrents, including low income, low schooling, and occupation. It is advocated that poverty-related mental disorder needs urgent policy interventions today.

The microscopic agglutination test (MAT) is the standard serological reference test for the diagnosis of leptospirosis, despite being a technically demanding and laborious procedure. The use of a locally optimised MAT panel is considered essential for proper performance and interpretation of results. This paper describes the procedure of selecting such an optimised panel for Sri Lanka, a country hyper-endemic for leptospirosis. MAT was performed using 24 strains on 1132 serum samples collected from patients presenting with acute undifferentiated fever. Of 24 strains, 15 were selected as the optimised panel, while only 11% of serum samples showed positivity. A geographical variation in predominantly reactive serovars was observed, whereas reactivity was low with the saprophytic strain Patoc. Testing with paired sera yielded a higher sensitivity but provided only a retrospective diagnosis. Serological tests based on ELISA with complementary molecular diagnosis using PCR are a feasible and robust alternative approach to diagnose leptospirosis in countries having a higher burden of the disease.

In this work, fiber strands of Wine Palm (Caryota urens) and Roselle (Hibiscus sabdariffa) plants were extracted and characterized. The extracted fiber strands were given water treatment for making comparisons with its dried counterpart. Techniques such as X-ray diffraction (XRD), Fourier transform infrared spectroscopy (FTIR) and scanning electron microscopy (SEM)-energy dispersive X-ray spectroscopy (EDX) were used to determine the micro structure, morphological properties and chemical compositions. Mechanical properties were studied using universal testing machine (UTM). XRD results indicate an increase in crystallinity of the fiber strands with water treatment and FTIR Spectroscopy reveals the variation of functional groups in these fibers. SEM analysis indicates smoothening of the surface on water treatment. The fineness of these fiber strands was found to be 372.18 denier and 13.63 denier respectively for CU and HS fibers. UTM results confirm that tensile strength increases on water treatment for both the fibers. These fibers have competitive advantages when evaluated with other natural fibers.

Abstract Background Cystic fibrosis has been largely under-diagnosed and thus, limited data is available on the incidence of cystic fibrosis in Sri Lanka. Our aim is to describe the phenotypic and genotypic spectrum of children with cystic fibrosis in Sri Lanka. Case presentation This report describes 10 unrelated cystic fibrosis cases with phenotypic features of cystic fibrosis and abnormal or intermediate sweat tests. The most common phenotypic features in this sample of symptomatic patients were persistent or recurrent lower respiratory tract infections, failure to thrive and Pseudo-Bartter syndrome. Altogether 7 cystic fibrosis causing mutations were identified in 10 patients. Except delta F508 which is the commonest mutation worldwide all the other mutations detected in Sri Lankan patients are rare mutations. 1161delC and V456A detected in our patients are South Asian mutations. The other mutations such as [C.1282C > G; C.2738A > G], C.53 + 1G > C, 2184insA and a deletion encompassing exons 4 to 11 have been reported previously from European patients with cystic fibrosis. Conclusion These cases highlight the importance of considering the diagnosis of cystic fibrosis in children and young adults presenting with persistent respiratory tract infections associated with severe malnutrition and Pseudo-Bartter syndrome, especially in low income countries where newborn screening for cystic fibrosis is not available. The spectrum of CFTR mutations in Sri Lanka is heterogeneous and possibly linked to genetic flow from Indian subcontinent and Europe. The common mutations should be identified by sequencing the entire CFTR gene in adequate number of cystic fibrosis patients in order to design a mutation panel for common regional mutations.

[This corrects the article DOI: 10.1371/journal.pntd.0009272.].

Leptospirosis is a ubiquitous zoonotic disease and a major clinical challenge owing to the multitude of clinical presentations and manifestations that are possibly attributable to the diversity of Leptospira, the understanding of which is key to study the epidemiology of this emerging global disease threat. Sri Lanka is a hotspot for leptospirosis with high levels of endemicity as well as annual epidemics. We carried out a prospective study of Leptospira diversity in Sri Lanka, covering the full range of climatic zones, geography, and clinical severity. Samples were collected for leptospiral culture from 1,192 patients from 15 of 25 districts in Sri Lanka over two and half years. Twenty-five isolates belonging to four pathogenic Leptospira species were identified: L. interrogans, L. borgpetersenii, L. weilii, and L. kirschneri. At least six serogroups were identified among the isolates: Autumnalis (6), Pyrogenes (4), Icterohaemorrhagiae (2), Celledoni (1), Grippotyphosa (2) and Bataviae (1). Seven isolates did not agglutinate using available antisera panels, suggesting new serogroups. Isolates were sequenced using an Illumina platform. These data add 25 new core genome sequence types and were clustered in 15 clonal groups, including 12 new clonal groups. L. borgpetersenii was found only in the dry zone and L. weilii only in the wet zone. Acute kidney injury and cardiovascular involvement were seen only with L. interrogans infections. Thrombocytopenia and liver impairment were seen in both L. interrogans and L. borgpetersenii infections. The inadequate sensitivity of culture isolation to identify infecting Leptospira species underscores the need for culture-independent typing methods for Leptospira.

C3 glomerulopathy (C3GP) is a group of diseases caused by a deregulated complement system, which encompasses both dense deposit disease and C3 glomerulonephritis. Renal manifestations of C3GP are primarily of proliferative glomerulonephritis, and only a few case reports of crescentic glomerulonephritis (CGN) in association with C3GP are available. Here is a case of an adult South-Asian female, who was diagnosed as seropositive acute Mycoplasma pneumoniae infection, with associated systemic manifestations, including immune-type extravascular haemolysis and nephrotic range proteinuria. Subsequent renal biopsy revealed CGN with disrupted Bowman’s capsules and necrotizing lesions. Immunofluorescence showed coarse granular mesangial C3 deposits with negative IgM, IgG, IgA, and C1q. The immunomorphological phenotype raised two possibilities including C3GP and infection-related glomerulonephritis (IRGN). Persistent proteinuria with no evidence of resolution even after 6 months of follow-up favoured C3GP over IRGN. The patient proceeded to end-stage renal failure requiring renal replacement despite aggressive immunosuppression. This case illustrates the rare association of CGN with C3GP induced by Mycoplasma pneumoniae infection, highlighting the importance of correct diagnosis as well as timely identification of triggering factors in CGN on patient outcome.

Phosphatidate phosphatase-1 (lipin-1) is encoded by LPIN1 gene. Lipin-1 deficiency has been reported as the second most common cause of early-onset rhabdomyolysis after primary fatty acid oxidation disorders. We report a case of a 32-year-old Sri Lankan female with a history of more than 10 episodes of rhabdomyolysis and exercise intolerance since childhood. These episodes were triggered by infections and exercise. A temporal relationship between the acute episodes and use of drugs such as theophylline, mefenamic acid, co-trimoxazole, and combined oral contraceptive pills was also noted. There was marked elevation of serum creatine kinase and transaminases during acute episodes. Family history revealed parental consanguinity and an affected sibling who died of an acute episode associated with muscle weakness, dark coloured urine, and cyanosis, at the age of 2 years. The histochemical findings of the patient under discussion were consistent with a metabolic myopathy affecting membrane integrity. A homozygous, likely pathogenic variant c.1684G>T encoding p.(Glu562∗) was identified by clinical exome sequencing. Even though the studies to date give no convincing evidence of a possible causal or contributory relationship between the drugs under discussion and lipin-1 related rhabdomyolysis, this case highlights the importance of pharmacovigilance and reporting adverse drug reactions in patients with lipin-1 deficiency.

Abstract Background Renal hypouricemia is a rare heterogeneous inherited disorder characterized by impaired tubular uric acid transport, reabsorption insufficiency and /or acceleration of secretion. The affected individuals are predisposed to nephrolithiasis and recurrent episodes of exercise-induced acute kidney injury. Type 1 is caused by dysfunctional variants in the SLC22A12 gene (URAT1), while type 2 is caused by defects in the SLC2A9 gene (GLUT9). To date, more than 150 patients with the loss-of-function mutations for the SLC22A12 gene have been found (compound heterozygotes and/or homozygotes), most of whom are Japanese and Koreans. Case presentation Herein, we report a nine year old Sri Lankan boy with renal hypouricemia (serum uric acid 97 μmol/L, fractional excretion of uric acid 33%).The sequencing analysis of SLC22A12 revealed a potentially deleterious missense variant c.1400C > T (p.T467 M, rs200104135) in heterozygous state. This variant has been previously identified in homozygous and/or compound heterozygous state with other causative SLC22A12 variant c.1245_1253del (p.L415_G417del) in Roma population. Conclusions This is the first identification of a family with mild renal hypouricemia1 associated to the p.T467 M variant. Detailed investigations of urate blood and urine concentrations in patients with unexplained hypouricemia are needed and renal hypouricemia should also be considered in patients other than those from Japan and/or Korea. Our finding confirms an uneven geographical and ethnic distribution of Romany prevalent SLC22A12 variant that need to be considered in Asian patients (population data Genome Aggregation Database: allele frequency in South Asia 0.007055, in East Asia 0.001330).

Background: Patient satisfaction is one of the well-known yardsticks to measure the strength of services being provided in hospitals. Objective: To assess the level of satisfaction on health care services provided by the out-patient department (OPD) and associated socio-demographic factors among patients attending a base hospital in Sri Lanka. Methods: A cross-sectional study was conducted among 422 out-patients at Base Hospital (BH) Panadura, selected using a systematic sampling method during January 2017. Critically ill patients and first timers to the OPD were excluded. Data were collected using a self-administered questionnaire on the personal characteristics of patients and their satisfaction with OPD services under five domains: physical facilities, registration, consultation, pharmacy and accessibility of services. Satisfaction was assessed based on a pre-determined scoring system. Patients were dichotomized into two groups: ‘satisfied’ and ‘less-satisfied’ with the OPD services, and were compared by selected socio-demographic factors using chi-square test. Results: Response rate was 85% (n=359). The majority consisted of females (n=261, 72.7%). Patient satisfaction varied according to the service domain: physical facilities (n=214, 59.6%), registration (n=138, 38.4%), consultation (n=175, 48.7%), pharmacy services (n=198, 55.1%) and accessibility of service (n=118, 32.9%). Further, only smaller proportions of patients were satisfied with the adequacy (n=119, 33.1%) and comfort (n=160, 44.6%) of seating facilities; adequacy (n=130, 36.2%) and cleanliness (n=109, 30.4%) of toilets; waiting time at the dispensary (n=65, 18.1%) and for consultation (n=120, 33.4%), and examination done by doctor (n=131, 36.5%). Females were significantly less-satisfied with the overall OPD services compared to males (25% versus 18.4%; p=0.04). No such relationship was seen in relation to age (p=0.28) and level of education (p=0.31). Conclusions: Most patients were satisfied with the overall OPD services. Yet, areas such as registration services, waiting time especially for dispensary and consultation, and sanitary and seating facilities need further improvement. Strengthening the infrastructure and introducing an appointment system are recommended.

Abstract Background Dubin–Johnson syndrome and intrahepatic cholestasis of pregnancy are rare chronic liver disorders. Dubin–Johnson syndrome may manifest as conjugated hyperbilirubinemia, darkly pigmented liver, presence of abnormal pigment in the parenchyma of hepatocytes and abnormal distribution of the coproporphyrin isomers I and III in the urine. Intrahepatic cholestatic jaundice of pregnancy presents as pruritus, abnormal liver biochemistry and increased serum bile acids. Case presentation A Sri Lankan girl presented with recurrent episodes of jaundice. She had conjugated hyperbilirubinaemia with diffuse, coarse brown pigments in the hepatocytes. Urine coproporphyrin examination suggested Dubin–Johnson syndrome. Genetic studies confirmed missense homozygous variant p.Trp709Arg in the ATP-binding cassette sub-family C member 2 gene ABCC2 that encodes the Multidrug resistance-associated protein 2 that causes Dubin–Johnson syndrome. The gene study of the mother revealed the same missense variant in ABCC2/MRP2 but with a heterozygous status, and in addition a homozygous missense variant p.Val444Ala in the ATP-binding cassette, sub-family B member 11 gene ABCB11 that encodes the bile salt export pump. Conclusion Dubin–Johnson syndrome should be considered when the common causes for conjugated hyperbilirubinaemia have been excluded, and patient has an increased percentage of direct bilirubin relative to total bilirubin concentration. Its early diagnosis prevents repeated hospital admissions and investigations. Knowledge of a well known homozygous variant in ABCB11 gene could help in the management of pregnancy.

Abstract Background Uric acid is the metabolic end product of purine metabolism in humans. Altered serum and urine uric acid level (both above and below the reference ranges) is an indispensable marker in detecting rare inborn errors of metabolism. We describe different case scenarios of 4 Sri Lankan patients related to abnormal uric acid levels in blood and urine. Case 1 A one-and-half-year-old boy was investigated for haematuria and a calculus in the bladder. Xanthine crystals were seen in microscopic examination of urine sediment. Low uric acid concentrations in serum and low urinary fractional excretion of uric acid associated with high urinary excretion of xanthine and hypoxanthine were compatible with xanthine oxidase deficiency. Case 2 An 8-month-old boy presented with intractable seizures, feeding difficulties, screaming episodes, microcephaly, facial dysmorphism and severe neuro developmental delay. Low uric acid level in serum, low fractional excretion of uric acid and radiological findings were consistent with possible molybdenum cofactor deficiency. Diagnosis was confirmed by elevated levels of xanthine, hypoxanthine and sulfocysteine levels in urine. Case 3 A 3-year-10-month-old boy presented with global developmental delay, failure to thrive, dystonia and self-destructive behaviour. High uric acid levels in serum, increased fractional excretion of uric acid and absent hypoxanthine–guanine phosphoribosyltransferase enzyme level confirmed the diagnosis of Lesch–Nyhan syndrome. Case 4 A 9-year-old boy was investigated for lower abdominal pain, gross haematuria and right renal calculus. Low uric acid level in serum and increased fractional excretion of uric acid pointed towards hereditary renal hypouricaemia which was confirmed by genetic studies. Conclusion Abnormal uric acid level in blood and urine is a valuable tool in screening for clinical conditions related to derangement of the nucleic acid metabolic pathway.

Aim: Spontaneous coronary artery dissection (SCAD) is a rare cause of angina, myocardial infarction (MI), and sudden cardiac death (SCD) and may frequently manifest as acute coronary syndrome (ACS). The diagnosis of SCAD relies on angiographic visualization of a radiolucent intimal flap. Therapeutic options include medical therapy, percutaneous coronary interventions, and bypass surgery. The aim of this study is to analyze the clinical profile, inhospital outcomes, management, and follow-up of patients with angiographic SCAD. Methods: About 19,676 diagnostic coronary angiograms (CAGs) were reviewed retrospectively during a 2-year period; 64 patients had SCAD and were included in the study. Complete medical histories before and during the event as well as treatment regimens were obtained from patients' hospital files. Results: A total of 64 cases of SCAD were considered for the study within an age range of 25–70 years. Fifty-eight patients presented with ACS, two patients presented with unstable angina, one patient presented with rheumatic mitral stenosis in atrial fibrillation, one patient presented with non-ST-elevation myocardial infarction, one patient with dilated cardiomyopathy with left ventricular dysfunction, and one patient with effort. Out of 64 patients, four patients died and the average hospital stay is 3–5 days. Conclusion: SCAD occurs in 0.32% of patients undergoing CAG for evaluation of coronary artery disease. Majority of SCAD occurs in men. The left coronary artery is most commonly affected. The inhospital outcomes are good. Most of the patients with SCAD have good prognosis following optimal medical therapy.

Our essay examines the recursions, rationalities, limits, and promise of the law drawing on three recent cases of women who encountered law enforcement authorities and the courts in Sri Lanka. It provides a strong account of how dominant gender norms are mobilized to determine who is afforded the sanctuary of the law and who is not. By foregrounding the troubled encounters of the women with the law the essay also demonstrates the ways in which the law, culture, and the state combine, pull apart, and recombine in a manner that draws attention to their own internal relations; and how procedures established to ensure legal objectivity and judicial impartiality often fold back on themselves, reflecting the pliancy of the law. The essay also foregrounds the conditions of possibility, including feminist legal methodologies, that enable women to (re)turn to the law despite its transgressions. In doing so it argues for seeing the law as multilayered and recursive, reflecting the thick and uneven conditions under which women access justice in Sri Lanka. In highlighting how these women challenge and bargain with the law, the essay also acknowledges their tenacity and endurance in what, ultimately, is an effort at demanding an improved and substantive justice.