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Foot wounds are complications of type II diabetes mellitus (T2DM), which may be prevented with proper foot assessments. Diabetic foot exams are not consistently performed despite evidence-based screening tools available. Objective: To determine if the implementation of an electronic medical record (EMR) alert using Inlow’s 60-Second Diabetic Foot Screen would impact the number of diabetic foot exams performed among T2DM patients at a primary care level. Method: A quantitative quasi-experiment design was used to implement an electronic medical record (EMR) alert using Inlow’s 60-Second Diabetic Foot Screen to impact the frequencies of diabetic foot exams. Sample size was 65, n=35 in the comparative group and n= 30 in the implementation group. Population consisted of male and female patients ages 18 years and older, diagnosed with T2DM within the last two years, no previous diabetic foot exams or received foot care education, did not perform self-reported foot inspections, and were able to read, write, and speak English. A chi square test was run to analyze the frequencies of foot exams between groups. Results: A Pearson’s chi-square showed a statistically significant increase in the foot exams X2 (1, N=65) = 5.286, p= 0.02 using an EMR alert with Inlow’s 60-Second Diabetic Foot screen. Conclusion: EMR alerts increase performance of diabetic foot exams. Recommendations include sustainment of the program, dissemination of results, and further investigation of the impact of EMR alerts. Keywords: Diabetes, Foot screening, EMR alert

Introduction: While females make up more than half of medical school matriculants, they only comprise about one third of emergency medicine (EM) residents. We examined EM residency cohorts with entering years of 2014–2017 to estimate the ratio of males to females among residents and program leadership to determine what correlation existed, if any, between program leadership and residency gender distributions. Methods: We identified 171 accredited EM residency programs in the United States with resident cohorts entering between 2014–2017 with publicly available data that were included in the study. The number of male and female residents and program directors were counted. We then confirmed the counts by contacting the programs directly to confirm accuracy of the data collected from program websites. Results: Within the included 171 programs, the overall male to female EM resident ratio was 1.78:1. Individual program ratios ranged from 0.85–8.0. Only eight programs (5.6%) had a female-predominant ratio. Among program directors, the overall male to female ratio was 2.17:1. TThe gender of the program director did not have a statistically significant correlation with the male to female ratio among its residents (P = .93). Conclusion: Within 171 residency programs across the US with entering cohorts between 2014–2017, the average male to female ratio among residents is nearly 2:1. No significant correlation exists between the gender distribution among a program’s leadership and its residents.

Summary: Background: Worldwide, haemoglobin E β-thalassaemia is the most common genotype of severe β-thalassaemia. The paucity of long-term data for this form of thalassaemia makes evidence-based management challenging. We did a long-term observational study to define factors associated with survival and complications in patients with haemoglobin E thalassaemia. Methods: In this prospective, longitudinal cohort study, we included all patients with haemoglobin E thalassaemia who attended the National Thalassaemia Centre in Kurunegala, Sri Lanka, between Jan 1, 1997, and Dec 31, 2001. Patients were assessed up to three times a year. Approaches to blood transfusions, splenectomy, and chelation therapy shifted during this period. Survival rates between groups were evaluated using Kaplan-Meier survival function estimate curves and Cox proportional hazards models were used to identify risk factors for mortality. Findings: 109 patients (54 [50%] male; 55 [50%] female) were recruited and followed up for a median of 18 years (IQR 14–20). Median age at recruitment was 13 years (range 8–21). 32 (29%) patients died during follow-up. Median survival in all patients was 49 years (95% CI 45–not reached). Median survival was worse among male patients (hazard ratio [HR] 2·51, 95% CI 1·16–5·43), patients with a history of serious infections (adjusted HR 8·49, 2·90–24·84), and those with higher estimated body iron burdens as estimated by serum ferritin concentration (adjusted HR 1·03, 1·01–1·06 per 100 units). Splenectomy, while not associated with statistically significant increases in the risks of death or serious infections, ultimately did not eliminate a requirement for scheduled transfusions in 42 (58%) of 73 patients. Haemoglobin concentration less than or equal to 4·5 g/dL (vs concentration >4·5 g/dL), serum ferritin concentration more than 1300 μg/L (vs concentration ≤1300 μg/L), and liver iron concentration more than 5 mg/g dry weight of liver (vs concentration ≤5 mg/g) were associated with poorer survival. Interpretation: Patients with haemoglobin E thalassaemia often had complications and shortened survival compared with that reported in high-resource countries for thalassaemia major and for thalassaemia intermedia not involving an allele for haemoglobin E. Approaches to management in this disorder remain uncertain and prospective studies should evaluate if altered transfusion regimens, with improved control of body iron, can improve survival. Funding: Wellcome Trust, Medical Research Council, US March of Dimes, Anthony Cerami and Ann Dunne Foundation for World Health, and Hemoglobal.

Abstract Background Though case reports and limited case series of Sickle cell disease in Sri Lanka have been reported previously, no attempt has been made hitherto to undertake a comprehensive genotypic-phenotypic analysis of this “rare” group of patients. Results All accessible Sickle cell disease patients, totaling 60, including, 51 Sickle β-thalassaemia and 9 homozygous sickle patients were enrolled from seven thalassaemia treatment centres between December 2016–March 2019. The majority of patients were of Sinhalese ethnicity (n = 52, 86.67%). Geographically, two prominent clusters were identified and the distribution of Sickle haemoglobin in the island contrasted markedly with the other haemoglobinopathies. 3/ 9 homozygous sickle patients and 3/ 51 Sickle β-thalassaemia patients were receiving regular transfusion. Joint pain was the commonest clinical symptom among all sickle cell disease patients (n = 39, 65.0%). Dactylitis was significantly more common in homozygous sickle patients compared with the Sickle β-thalassaemia groups (p 0.027). Two genetic backgrounds sickle mutation were identified namely, Arab Indian and Benin. Among the regulators of Foetal hemoglobin in Sickle patients of the present study rs1427407 G > T seemed to be the most prominent modifier, with a significant association with Foetal haemoglobin levels (p 0.04). Conclusions Overall, the clinical course of the Asian version of Sickle cell disease in Sri Lanka appears to be milder than that described in India.

Bovine colostrum (BC) has anti-inflammatory, anti-infective, growth and intestinal repair factors that may be beneficial in Crohn’s disease (CD). We assessed whether daily BC for up to 3 months was acceptable to children and young people (CYP) with CD in remission or of mild/moderate severity. CYP were randomised to receive either BC or matching placebo milk daily for 6 weeks (blinded phase); all received BC for the following 6 weeks (open phase). In 23 CYP, median (inter-quartile range) age was 15.2 (13.9–16.1) years and 9 (39.1%) were girls. A similar proportion of CYP in the BC and placebo arms completed the blinded phase (8/12, 75.0% and 9/11, 81.8% respectively). Twelve (70.6%) CYP completed the open phase with 7 (58.3%) tolerating BC for 3 months. Diaries in weeks 2, 6 and 12 revealed that most CYP took BC every day (5/7, 71.4%; 5/8, 62.5% and 6/11, 54.5% respectively). In interviews, opinions were divided as to preference of BC over the placebo milk and some preferred BC over other nutritional supplements. Symptoms, clinical and laboratory variables and quality of life were similar in the two arms. BC may be an acceptable nutritional supplement for daily, longer-term use in CYP with CD.

Abstract β thalassaemia intermedia (βTI) are a heterogeneous group of disorders known to be extremely phenotypically diverse. This group is more complex to manage as no definitive treatment guidelines exist unlike for β thalassaemia major (βTM). There are only a few studies looking at genotype phenotype associations of βTI outside the Mediterranean region. The reasons for the diverse clinical phenotype in βTI are unknown. We categorized fifty Sri Lankan patients diagnosed with βTI as mild, moderate or severe according to published criteria. DNA samples were genotyped for β thalassaemia mutations, α globin genotype and copy number and known genetic modifiers of haemoglobin F production. There were 26/50 (52.0%) in mild group and 12/50 (24.0%) each in moderate and sever categories. 18/26 (69.2%) classified as mild were β heterozygotes and 17/18 (94.4%) had excess α globin genes. 11/12 (91.6%) classified as moderate were β heterozygotes and 8/11 (72.2%) had excess α globin genes. In contrast, 8/12 (66.7%) classified as severe were β homozygotes and 7/8(87.5%) had α globin gene deletions. In Sri Lanka, co-inheritance of either excess α globin genes in β thalassaemia heterozygotes or α globin gene deletions in β thalassaemia homozygotes is a significant factor in modulating disease severity.

The ability to successfully discern and communicate the relevant aspects of a judicial opinion is a fundamental skill that legal professionals must have. Despite its importance, many in the legal arena lack the ability to effectively demonstrate this skill. Needless to say, law students suffer from this same shortcoming. After years of reading inadequate briefs submitted by lawyers and reviewing deficient submissions by law students, I developed an original case synthesis method. This method, titled the “Bell Case Synthesis Method,” teaches one how to select supporting cases and how to adequately explain the relevant aspects of selected cases. This original method has been tested for years and has proven to be quite valuable for memo and brief writing, as well as for the higher level thinking that is needed for success in law school and the practice of law. This article will benefit a broad audience, including lawyers, law students, paralegals, law clerks, inmate counsel and legal educators. In addition, it is timely, given the recent emphasis on producing practice-ready law school graduates.

Introduction: The high frequencies of carriers of severe haemoglobinopathies and of iron deficiency in Southeast Asia require reliable and affordable tests to improve on current screening procedures.Objectives: We evaluate a “one stop” approach using the THALCON dichlorophenolindophenol (DCIP) and one-tube osmotic fragility (OF) tests and measurement of Zinc Protoporphyrin (ZPP) to detect and distinguish HbE and β-thalassaemia traits from iron deficiency. We compare findings with current screening practice in Sri Lanka that relies on the identification of low mean red cell volume and/or mean red cell hemoglobin for this purpose.Methods: Between November 2017 and May 2018, we undertook a cross-sectional survey of secondary school students in Gampaha district, Sri Lanka. The THALCON–DCIP and OF tests were compared to capillary electrophoresis (CE), used as a gold standard to detect haemoglobinopathies. ZPP was measured in whole blood. Plasma ferritin and C-reactive protein (CRP) were measured in students with a raised ZPP concentration.Results: We collected venous blood samples from 1,324/1,332 (99.4%) students. The median age of the students was 17 (IQR 16–18) years, all were Sinhalese and 814/1,324 (61.5%) were female. CE identified 3 students with HbE trait and 26 students with β-thalassaemia trait. The THALCON–DCIP test was positive only in the 3 students with HbE (sensitivity 100%, 95% CI 29.2–100.0; specificity 100%, 95% CI 99.7–100.0). The THALCON–OF test identified all 26 students with β-thalassaemia trait (sensitivity = 100%, 95% CI 86.8–100.0) and 287 students with a normal CE result (specificity = 77.9%; 95% CI 75.5–80.1). It was also positive in 2/3 (66.7%) students with HbE trait. Iron deficiency (ZPP > 70 μmol/mol heme) was present in 118/1,240 (9.5%) students with a normal hemoglobin genotype, all of whom had plasma ferritin

A 58-year-old male with no past medical history presented to the emergency department with sudden onset left lower extremity weakness and central chest pain with radiation to his back. Electrocardiogram revealed an acute inferior and posterior ST-segment elevation myocardial infarction (STEMI). Point-of-care ultrasound (POCUS) demonstrated right ventricular akinesis consistent with infarction, and an intimal defect consistent with an aortic dissection. We determined that cardiothoracic surgery was indicated rather than left-heart catheterization and anticoagulation. Using POCUS we were able to immediately diagnose a dissection of the aortic arch and considerably alter treatment in a patient presenting with STEMI.

ObjectivesOur aim was to describe the numbers and distribution of patients with different types of thalassemia and to assess the standards of care in all thalassemia treatment centers throughout Sri Lanka and the success of the ongoing prevention programme.MethodsThis cross-sectional island-wide survey was conducted by two trained medical graduates, who visited each thalassemia center to collect data from every patient, using a standardized form. Data was collected through review of patient registers and clinical records.ResultsWe collected data on 1774 patients from 23 centers. 1219 patients (68.7%) had homozygous β-thalassemia, 360 patients (20.3%) had hemoglobin E β-thalassemia, and 50 patients (2%) had sickle β-thalassemia. There were unacceptably high serum ferritin levels in almost all centers. The annual number of births of patients with β-thalassaemia varied between 45-55, with little evidence of reduction over 19 years.ConclusionsCentral coordination of the treatment and ultimately prevention of thalassemia is urgently needed in Sri Lanka. Development of expert centers with designated staff with sufficient resources will improve the quality of care and is preferred to managing patients in multiple small units.

BackgroundIron deficiency, the most common micronutrient disorder and cause of anaemia globally, impairs growth, cognition, behaviour and resistance to infection.Methods/resultsAs part of a national survey of inherited haemoglobin variants in 7526 students from 72 secondary schools purposefully selected from the 25 districts of Sri Lanka, we studied 5912 students with a normal haemoglobin genotype. Median age was 16.0 (IQR 15.0-17.0) years and 3189 (53.9%) students were males. Most students were Sinhalese (65.7%), with fewer Tamils (23.1%) and Muslims (11.2%). Anaemia occurred in 470 students and was more common in females (11.1%) than males (5.6%). Haemoglobin, serum ferritin, transferrin receptor and iron were determined in 1196 students with low red cell indices and a structured sample of those with normal red cell indices (n = 513). The findings were weighted to estimate the frequencies of iron deficiency and iron deficiency anaemia classified according to WHO criteria. Iron depletion (serum ferritin 28.1 nmol/l) in 11.6% students. Iron deficiency anaemia (cellular iron deficiency with low haemoglobin) occurred in only 130/2794 (4.6%) females and 28/2789 (1.0%) males. Iron biomarkers were normal in 83/470 (14.6%) students with anaemia. In multiple regression analysis, the odds for iron depletion and cellular iron deficiency were about one-third in males compared with females, and the odds for iron deficiency anaemia were about one fifth in males compared to females. Tamil ethnicity and age ConclusionsLow iron status and anaemia remain common problems in Sri Lankan secondary school students especially females, younger students and the socioeconomically disadvantaged Tamil population. More research is needed to identify factors other than low iron status that contribute to anaemia in adolescents.

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is believed to confer protection against Plasmodium falciparum malaria, but the precise nature of the protective effect has proved difficult to define as G6PD deficiency has multiple allelic variants with different effects in males and females, and it has heterogeneous effects on the clinical outcome of P. falciparum infection. Here we report an analysis of multiple allelic forms of G6PD deficiency in a large multi-centre case-control study of severe malaria, using the WHO classification of G6PD mutations to estimate each individual’s level of enzyme activity from their genotype. Aggregated across all genotypes, we find that increasing levels of G6PD deficiency are associated with decreasing risk of cerebral malaria, but with increased risk of severe malarial anaemia. Models of balancing selection based on these findings indicate that an evolutionary trade-off between different clinical outcomes of P. falciparum infection could have been a major cause of the high levels of G6PD polymorphism seen in human populations.

The heritable haemoglobinopathy alpha(+)-thalassaemia is caused by the reduced synthesis of alpha-globin chains that form part of normal adult haemoglobin (Hb). Individuals homozygous for alpha(+)-thalassaemia have microcytosis and an increased erythrocyte count. Alpha(+)-thalassaemia homozygosity confers considerable protection against severe malaria, including severe malarial anaemia (SMA) (Hb concentration < 50 g/l), but does not influence parasite count. We tested the hypothesis that the erythrocyte indices associated with alpha(+)-thalassaemia homozygosity provide a haematological benefit during acute malaria.Data from children living on the north coast of Papua New Guinea who had participated in a case-control study of the protection afforded by alpha(+)-thalassaemia against severe malaria were reanalysed to assess the genotype-specific reduction in erythrocyte count and Hb levels associated with acute malarial disease. We observed a reduction in median erythrocyte count of approximately 1.5 x 10(12)/l in all children with acute falciparum malaria relative to values in community children (p < 0.001). We developed a simple mathematical model of the linear relationship between Hb concentration and erythrocyte count. This model predicted that children homozygous for alpha(+)-thalassaemia lose less Hb than children of normal genotype for a reduction in erythrocyte count of >1.1 x 10(12)/l as a result of the reduced mean cell Hb in homozygous alpha(+)-thalassaemia. In addition, children homozygous for alpha(+)-thalassaemia require a 10% greater reduction in erythrocyte count than children of normal genotype (p = 0.02) for Hb concentration to fall to 50 g/l, the cutoff for SMA. We estimated that the haematological profile in children homozygous for alpha(+)-thalassaemia reduces the risk of SMA during acute malaria compared to children of normal genotype (relative risk 0.52; 95% confidence interval [CI] 0.24-1.12, p = 0.09).The increased erythrocyte count and microcytosis in children homozygous for alpha(+)-thalassaemia may contribute substantially to their protection against SMA. A lower concentration of Hb per erythrocyte and a larger population of erythrocytes may be a biologically advantageous strategy against the significant reduction in erythrocyte count that occurs during acute infection with the malaria parasite Plasmodium falciparum. This haematological profile may reduce the risk of anaemia by other Plasmodium species, as well as other causes of anaemia. Other host polymorphisms that induce an increased erythrocyte count and microcytosis may confer a similar advantage.