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The original Association of Rehabilitation Nurses (ARN) Competency Model for Professional Rehabilitation Nursing published in 2016, and updated in 2020, provides a framework for rehabilitation nursing practice.This companion, but stand-alone, article to a 2022 publication further explicates and informs the updated Competency Model from inside looking out toward an increasing application for evidence-based practice (EBP).An eight-member 2020 ARN Task Force used an iterative process to review the original four domains and related competencies and came to consensus for the updated model.This model provides revised competency role descriptors or behaviors that guide nurses practicing at different proficiency levels in various settings.The Competency Model for Professional Rehabilitation Nursing is a premier resource that can advance professional rehabilitation nursing and guide EBP, including evaluation, quality improvement, and research. The model describes the nurse's role on the intra/interprofessional team and fosters collaboration with other healthcare professionals to enhance the quality of life for those affected by disability and chronic illness.The domains and associated competencies of this model clarify nursing roles at different proficiency levels, and role descriptors reflect current practice, supporting advancement of the specialty practice of rehabilitation nursing well into the 21st century.

This article details the community-engaged research process employed by a researcher–practitioner partnership (RPP) to develop and pilot a common exit survey of teachers from participating school districts at the end of the 2018–2019 school year. This development occurred with input from school district representatives serving on a study team as well as through ongoing conversations with district human resource directors. There were three goals for this process: (1) to develop a common exit survey relevant to local needs with a strong conceptual framework, (2) to increase response rates and establish consistent administration practices in the region, and (3) to inform future data collection and analysis related to the broader RPP study on teacher retention. The resultant instrument articulated nine common categories of reasons for leaving based on analysis and adaptation of regional exit surveys: retirement, personal reasons, teacher preparation, compensation and benefits, career advancement/switch or higher education, community context, district context, school context, and testing and accountability context. Exit survey items are provided with reliability and validity information, and theoretical and practical implications are discussed.

Background: Leg ulcers are a frequent complication in patients with the inherited hemoglobin disorders. In thalassemia, the literature is limited, and factors associated with the development of leg ulcers in hemoglobin E (HbE) beta thalassemia, the most common form of severe beta-thalassemia worldwide, have not previously been reported. Methods: We reviewed all available medical records of patients with HbE beta thalassemia to document the onset of leg ulcers at the 2 largest treatment centers in Sri Lanka. We reviewed the literature to identify studies reporting outcomes of interventions for ulcers in severe thalassemia. Results: Of a total of 255 actively registered patients with HbE thalassemia in the 2 centers, 196 patient charts were evaluable. A leg ulcer with a documented date of onset was recorded in 45 (22%) of 196 evaluable patients, aged (mean ± SEM) 22.2 ± 1.4 years. Most had been irregularly transfused; steady-state hemoglobin was 6.4 ± 0.2 g/dL. Treatment achieving healing in 17 patients included transfusions, antibiotics, oral zinc, wound toileting, and skin grafting. Conclusion: Leg ulcers may be more common in HbE beta thalassemia than in other forms of thalassemia. A systematic approach to treatment will be needed to document the prevalence and factors placing such patients at risk for leg ulcers. Controlled trials to evaluate the optimal treatment of this common complication are indicated.

We present case histories of three patients who had β-thalassemia (β-thal) trait with 'unusual severity' managed as β-thal intermedia (β-TI) where the basis of disease severity could not be explained with routine hematological and genetic investigations. The clinical diagnosis of 'thalassemia intermedia' was justifiable as they had a β-thal mutation and disease severity that did not fit in with either β-thal trait or with β-thal major (β-TM). As mutations of α, β, and γ genes could not explain the unusual severity of the disease, further analysis with next-generation sequencing (NGS) for red cell diseases was carried out, which led to the diagnosis of coexisting membranopathies. This case series highlights the inherent difficulty in the diagnosis of β-TI with certainty in some patients where the genetic basis is not clear-cut.

In the β-thalassemias, oxidative stress, resulting from chronic hemolysis, globin chain imbalance, iron overload and depleted antioxidant defences, likely contributes to cell death, organ damage, anemia, hypoxia and inflammation. We assessed variations in these parameters in β-thalassemia syndromes in Sri Lanka. Between November 2017 and June 2018, we assessed children and adults attending two thalassemia centres in Sri Lanka: 59 patients with HbE β-thalassemia, 50 β-thalassemia major, 40 β-thalassemia intermedia and 13 HbS β-thalassemia. Median age was 26.0 years (IQR 15.3-38.8), 101 (62.3%) were female and 152 (93.8%) of Sinhalese ethnicity. Methemoglobin, plasma hemoglobin, heme and ferritin were measured as sources of oxidants; plasma total antioxidant capacity, haptoglobin, hemopexin and vitamins C and E assessed antioxidant status; plasma thiobarbituric acid reactive substances and 8-hydroxy-2'-deoxyguanosine assessed oxidative damage; hemoglobin, plasma erythropoietin and transferrin receptor assessed anemia and hypoxia and plasma interleukin-6 and C-reactive protein assessed inflammation. Fruit and vegetable intake was determined by dietary recall. Physical fitness was investigated using the 6-min walk test and measurement of handgrip strength. Oxidant sources were frequently increased and antioxidants depleted, with consequent oxidative damage, anemia, hypoxia and inflammation. Biomarkers were generally most abnormal in HbE β-thalassemia and least abnormal in β-thalassemia intermedia but also varied markedly between individuals with the same thalassemia syndrome. Oxidative stress and damage were also more severe in splenectomized patients and/or those receiving iron chelation therapy. Less than 15% of patients ate fresh fruits or raw vegetables frequently, and plasma vitamins C and E were deficient in 132/160 (82.5%) and 140/160 (87.5%) patients respectively. Overall, physical fitness was poor in all syndromes and was likely due to anemic hypoxia. Studies of antioxidant supplements to improve outcomes in patients with thalassemia should consider individual patient variation in oxidative status both between and within the thalassemia syndromes.

Purpose The purpose of this study was to 1) determine the demographic differences between adults who have accepted, or plan to accept, the COVID-19 vaccine and those who will not accept the COVID-19 vaccine and 2) describe the potential influencers in deciding to accept the COVID-19 vaccine among adults living in the United States who have accepted or plan to accept the vaccine. Design A cross-sectional design utilizing an online survey was deployed using social media and a crowdsourcing platform. Setting United States. Subjects One thousand three hundred ninety-five (1395) adults completed the survey. The majority were white, male, between the ages of 25 and 45, and with representation from 50 states and Puerto Rico. Measures A two-branched survey was used to assess demographic information, vaccination intention, and 19 potential influencers of COVID-19 vaccine acceptance. Analysis Analysis included descriptive statistics and Chi-square tests to determine differences between groups. Results A higher proportion of male ( P < .001), married ( P < .001), and college-educated ( P < .001) participants reported acceptance of the vaccine. The factors with the highest mean score of reported level of influence were duty to protect the vulnerable and contribution to move society back to a sense of normalcy. Conclusion Understanding the potential influencers of vaccine acceptance may provide insight into strategies that could increase vaccination uptake.

BACKGROUND\ud 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.\ud \ud METHODS\ud 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.\ud \ud FINDINGS\ud 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.\ud \ud INTERPRETATION\ud 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.\ud \ud FUNDING\ud Wellcome Trust, Medical Research Council, US March of Dimes, Anthony Cerami and Ann Dunne Foundation for World Health, and Hemoglobal.

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 presentation 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.

Summary Background BIG 3-07/TROG 07.01 is an international, multicentre, randomised, controlled, phase 3 trial evaluating tumour bed boost and hypofractionation in patients with non-low-risk ductal carcinoma in situ following breast-conserving surgery and whole breast radiotherapy. Here, we report the effects of diagnosis and treatment on health-related quality of life (HRQOL) at 2 years. Methods The BIG 3-07/TROG 07.01 trial is ongoing at 118 hospitals in 11 countries. Women aged 18 years or older with completely excised non-low-risk ductal carcinoma in situ were randomly assigned, by use of a minimisation algorithm, to tumour bed boost or no tumour bed boost, following conventional whole breast radiotherapy or hypofractionated whole breast radiotherapy using one of three randomisation categories. Category A was a 4-arm randomisation of tumour bed boost versus no boost following conventional whole breast radiotherapy (50 Gy in 25 fractions over 5 weeks) versus hypofractionated whole breast radiotherapy (42·5 Gy in 16 fractions over 3·5 weeks). Category B was a 2-arm randomisation between tumour bed boost versus no boost following conventional whole breast radiotherapy, and category C was a 2-arm randomisation between tumour bed boost versus no boost following hypofractionated whole breast radiotherapy. Stratification factors were age at diagnosis, planned endocrine therapy, and treating centre. The primary endpoint, time to local recurrence, will be reported when participants have completed 5 years of follow-up. The HRQOL statistical analysis plan prespecified eight aspects of HRQOL, assessed by four questionnaires at baseline, end of treatment, and at 6, 12, and 24 months after radiotherapy: fatigue and physical functioning (EORTC QLQ-C30); cosmetic status, breast-specific symptoms, arm and shoulder functional status (Breast Cancer Treatment Outcome Scale); body image and sexuality (Body Image Scale); and perceived risk of invasive breast cancer (Cancer Worry Scale and a study-specific question). For each of these measures, tumour bed boost was compared with no boost, and conventional whole breast radiotherapy compared with hypofractionated whole breast radiotherapy, by use of generalised estimating equation models. Analyses were by intention to treat, with Hochberg adjustment for multiple testing. This trial is registered with ClinicalTrials.gov , NCT00470236 . Findings Between June 1, 2007, and Aug 14, 2013, 1208 women were enrolled and randomly assigned to receive no tumour bed boost (n=605) or tumour bed boost (n=603). 396 of 1208 women were assigned to category A: conventional whole breast radiotherapy with tumour bed boost (n=100) or no boost (n=98), or to hypofractionated whole breast radiotherapy with tumour bed boost (n=98) or no boost (n=100). 447 were assigned to category B: conventional whole breast radiotherapy with tumour bed boost (n=223) or no boost (n=224). 365 were assigned to category C: hypofractionated whole breast radiotherapy with tumour bed boost (n=182) or no boost (n=183). All patients were followed up at 2 years for the HRQOL analysis. 1098 (91%) of 1208 patients received their allocated treatment, and most completed their scheduled HRQOL assessments (1147 [95%] of 1208 at baseline; 988 [87%] of 1141 at 2 years). Cosmetic status was worse with tumour bed boost than with no boost across all timepoints (difference 0·10 [95% CI 0·05–0·15], global p=0·00014, Hochberg-adjusted p=0·0016); at the end of treatment, the estimated difference between tumour bed boost and no boost was 0·13 (95% CI 0·06–0·20; p=0·00021), persisting at 24 months (0·13 [0·06–0·20]; p=0·00021). Arm and shoulder function was also adversely affected by tumour bed boost across all timepoints (0·08 [95% CI 0·03–0·13], global p=0·0033, Hochberg adjusted p=0·045); the difference between tumour bed boost and no boost at the end of treatment was 0·08 (0·01 to 0·15, p=0·021), and did not persist at 24 months (0·04 [–0·03 to 0·11], p=0·29). None of the other six prespecified aspects of HRQOL differed significantly after adjustment for multiple testing. Conventional whole breast radiotherapy was associated with worse body image than hypofractionated whole breast radiotherapy at the end of treatment (difference –1·10 [95% CI –1·79 to –0·42], p=0·0016). No significant differences were reported in the other PROs between conventional whole breast radiotherapy compared with hypofractionated whole breast radiotherapy. Interpretation Tumour bed boost was associated with persistent adverse effects on cosmetic status and arm and shoulder functional status, which might inform shared decision making while local recurrence analysis is pending. Funding National Health and Medical Research Council, Susan G Komen for the Cure, Breast Cancer Now, OncoSuisse, Dutch Cancer Society.

BackgroundUtilization of intracranial pressure monitors (ICPMs) has not been consistently shown to improve mortality in patients with severe traumatic brain injury (TBI). A single-center analysis concluded that venous thromboembolism (VTE) chemoprophylaxis (CP) posed no significant bleeding risk in patients following ICPM implementation; however, there is still debate about the optimal use and timing of CP in patients with ICPMs for fear of worsening intracranial hemorrhage. We hypothesized that ICPM use is associated with increased time to VTE CP and thus increased VTE in patients with severe TBI.MethodsA retrospective analysis of the Trauma Quality Improvement Program (2010-2016) was performed to compare severe TBI patients with and without ICPMs. A multivariable logistic regression analysis was completed.ResultsFrom 35,673 patients with severe TBI, 12,487 (35%) had an ICPM. Those with ICPMs had a higher rate of VTE CP (64.3% vs. 49.4%, p

The provision of formative assessment and formative feedback is challenging for an educator working with large cohorts of students. To avoid being overwhelmed with the volume of work, appropriate technologies need to be selected to support learners and scale the module assessment and delivery to accommodate the size of the class. This paper presents the results of implemented online formative assessment delivery for a large postgraduate cohort of both full- and part-time students. We experimented with early and frequent student access to online formative assessment followed by immediate automated feedback. We review the impact of this assessment regime on student experience and achievement of learning outcomes. End-of-term student feedback indicates that frequent online quizzes, formative feedback and repetitive practicing were very well received by the students. We observe sustained levels of student engagement through data collected during student interaction with online module content and assessment. Analysis of the data has determined that the quizzes which had the highest number of attempts showed strong correlation with the highest student scores at the end-of-term exam. Such an experiment reflects how students learn via online content delivery and make use of available learning technologies for self-directing and practicing in computing subject. Frequent access to online formative assessment tool followed by immediate automated feedback helps to create effective learning environment and accommodate learners in large full-time and part-time classes.

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.

Purpose Pediatric genitalia injury represents 0.6% of all pediatric trauma. It is crucial for providers to understand whether pediatric patients are at risk for violent mechanisms, such as rape, assault, or other abuse. Therefore, we sought to perform a large database analysis of pediatric and adolescent female genitalia trauma, comparing mechanisms of injury (i.e., sexual abuse) and need for operative intervention between adolescent and pediatric cohorts. Methods The National Trauma Data Bank was queried (years 2007–2015) for female patients ≤ 16 years old with external genitalia (vaginal or vulvar) trauma. Two groups were compared: pediatrics (

It is highly desirable to provide our computer science and software engineering students with hands-onexposure to currently industry techniques and technologies. One area of extreme growth and skills shortageis cloud computing which led to a curriculum redesign and a new module to teach these skills to final yearstudents. Cloud computing products are available from a number of vendors, but their academicprogrammes often leave much to be desired, with limited credits and features. In the resource-constraineduniversity sector simply buying vendor credits for students would be expensive but also wasteful as thereis no capital investment. In an attempt to provide hands-on experience with the latest technologies an inhouse “cloud in a cupboard” was designed and created at Queen’s University Belfast. This took advantageof the wide range of free open-source software systems which underpin modern cloud vendors and, for verylittle cost thanks to re-purposed equipment, was able to support a large final-year module and severalundergraduate dissertation projects successfully. Student feedback was very positive, and plans continue togrow this system further to support the current functionality and add more in the coming years.

Following significant success in the use of so-called “backchannel” tools in building student engagement within the School of Electronics, Electrical Engineering and Computer Science at Queen’s University Belfast a situation arose in 2018 where our choice of provider closed down. We attempted to use other avjavascript:void(0);ailable off-the-shelf tools but for various reasons these were not widely adopted. At the end of 2018 we decided to implement our own student-facing communication, question and, chat tool for students and lecturing staff to use. Called QChan the system went live, while still under development, to classes aslarge as 400 in size in January 2019. Developing a system to encourage use, be easily accessible and yet secure and robust enough to withstand experimenting computer science students was something of a challenge. In 2020, QChan is widely used in our school and has been a driver to better student engagement. We continue to develop QChan and also analyse it and the other platforms’ content to better understand how to consistently engage with students

Iron deficiency complicates the use of red cell indices to screen for carriers of haemoglobin variants in many populations. In a cross sectional survey of 7526 secondary school students from 25 districts of Sri Lanka, 1963 (26.0%) students had low red cell indices. Iron deficiency, identified by low serum ferritin, was the major identifiable cause occurring in 550/1806 (30.5%) students. Low red cell indices occurred in iron-replete students with alpha-thalassaemia including those with single alpha-globin gene deletions. Anaemia and low red cell indices were also common in beta-thalassaemia trait. An unexpected finding was that low red cell indices occurred in 713 iron-replete students with a normal haemoglobin genotype. It is common practice to prescribe iron supplements to individuals with low red cell indices. Since low red cell indices were a feature of all forms of α thalassaemia and also of iron deficiency, in areas where both conditions are common, such as Sri Lanka, it is imperative to differentiate between the two, to allow targeted administration of iron supplements and avoid the possible deleterious effects of increased iron availability in iron replete individuals with low red cell indices due to other causes such as α thalassaemia.

Worsening quality indicators of health care shake public trust. Although safety and quality of care in hospitals can be improved, healthcare quality remains conceptually and operationally vague. Therefore, the aim of this analysis is to clarify the concept of healthcare quality. Walker and Avant’s method of concept analysis, the most commonly used in nursing literature, provided the framework. We searched general and medical dictionaries, public domain websites, and 5 academic literature databases. Search terms included health care and quality, as well as healthcare and quality. Peer-reviewed articles and government publications published in English from 2004 to 2016 were included. Exclusion criteria were related concepts, discussions about the need for quality care, gray literature, and conference proceedings. Similar attributes were grouped into themes during analysis. Forty-two relevant articles were analyzed after excluding duplicates and those that did not meet eligibility. Following thematic analysis, 4 defining attributes were identified: (1) effective, (2) safe, (3) culture of excellence, and (4) desired outcomes. Based on these attributes, the definition of healthcare quality is the assessment and provision of effective and safe care, reflected in a culture of excellence, resulting in the attainment of optimal or desired health. This analysis proposes a conceptualization of healthcare quality that defines its implied foundational components and has potential to improve the provision of quality care. Theoretical and practice implications presented promote a fuller, more consistent understanding of the components that are necessary to improve the provision of healthcare and steady public trust.

Introduction This patterns of practice study was conducted on behalf of the RANZCR Breast Interest Group in order to document current radiotherapy practices for breast cancer in Australia and New Zealand. The survey identifies variations and highlights potential contentious aspects of radiotherapy management of breast cancer. Methods A fifty-eight question survey was disseminated via the Survey Monkey digital platform to 388 Radiation Oncologists in Australia and New Zealand. Results In total, 156 responses were received and collated. Areas of notable consensus among respondents included hypofractionation (77.3% of respondents would ‘always’ or ‘sometimes’ consider hypofractionation in the management of ductal carcinoma in-situ and 99.3% in early invasive breast cancer); margin status in early breast cancer (73.8% believe a clear inked margin is sufficient and does not require further surgery) and use of bolus in post-mastectomy radiotherapy (PMRT) (91.1% of participants use bolus in PMRT). Areas with a wider degree of variability amongst respondents included regional nodal irradiation and components of radiotherapy planning and delivery (examples include the technique used for delivery of boost and frequency of bolus application for PMRT). Conclusion The results of these patterns of practice survey informs radiation oncologists in Australia and New Zealand of the current clinical practices being implemented by their peers. The survey identifies areas of consensus and contention, the latter of which may lead to a development of research trials and/or educational activities to address these areas of uncertainty.

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.

Objectives To report the clinical and virologic epidemiology of a recent epidemic of hepatitis C in thalassaemia patients in Sri Lanka. Background Transfusion‐dependent thalassaemia patients remain at risk for hepatitis C virus (HCV). Here, we report a cluster of recent HCV infections in Sri Lankan thalassaemia patients and examine the phylogenetic relationship of viral sequences. Methods We conducted two prospective cross‐sectional surveys of 513 patients in four Sri Lankan thalassaemia centres in 2014/2015 and re‐surveyed one centre in 2016. We screened for anti‐HCV antibodies using the CTK Biotech enzyme‐linked immunosorbent assay (ELISA) kits and confirmed active infection by reverse transcription‐polymerase chain reaction (RT‐PCR) for HCV‐RNA. HCV genomes were sequenced by unbiased target enrichment. Results Anti‐HCV antibodies were found in 116/513 (22.6%) of patients initially tested. Active hepatitis C infection was found in 26 patients with no cases of active hepatitis B infection. Of 26 patients with HCV, two were infected with genotype 1(a), and the rest had 3(a). In a single centre (Ragama), 122 patients (120 new cases and two previously tested, but negative) were retested for anti‐HCV antibodies. 32/122 (26.2%) patients were seropositive. Twenty‐three (23/122; 18.8%) of these new cases were confirmed by HCV PCR (all genotype 3[a]). Conclusion There is a significant cluster of recent HCV cases in multiply transfused thalassaemia patients in several centres in Sri Lanka. Most of the viruses shared a close phylogenetic relationship. The results are consistent with recent continuing transfusion‐transmitted HCV infection. Routine surveillance for HCV of chronically transfused patients is required irrespective of screening of blood products.

More than half a million children experience non-accidental trauma (NAT) annually. Historically, NAT has been associated with an increased hospital length of stay (LOS). We hypothesized that in pediatric trauma patients, NAT is associated with longer hospital LOS, independent of injury severity, compared to accidental trauma (AT). The Pediatric Trauma Quality Improvement Program (2014–2016) was queried for patients aged 1–16 years. Patients were stratified into two groups: AT and NAT. The median LOS for the entire cohort was identified and used in a multivariable logistic regression analysis. From 93,089 pediatric trauma patients, 417 (

In hereditary hemochromatosis, iron overload is associated with homozygosity for the p.C282Y mutation. A second mutation, p.H63D, occurs at significant frequencies in Europe, North Africa, the Middle East and Asia. Early studies in Sri Lanka indicated that the variant had arisen independently, suggesting that it had been the subject of selective pressure. However, its role in iron absorption is unclear. In a survey of 7526 Sri Lankan secondary school students, we determined hemoglobin genotype and measured red cell indices, serum ferritin, transferrin receptor, iron zinc protoporphyrin and hepcidin. These variables were compared according to the presence or absence of the p.H63D variant in a subset of 1313 students for whom DNA samples were available. Students were classified as having low red cell indices if they had an MCV

Previous studies have had conflicting results when comparing risk of mortality in patients with gunshot wounds (GSWs) treated at Level-I and II trauma centers. However, the populations studied were restricted geographically. We hypothesized that patients presenting after a GSW to the torso at Level-I centers would have a shorter time to surgical intervention (exploratory laparotomy or thoracotomy) and a lower risk of mortality, compared to Level-IIs in a national database. The Trauma Quality Improvement Program (2010–2016) was queried for patients presenting to Level-I or II trauma center after a GSW to the torso. A multivariable logistic regression analysis was performed. From 17,965 patients with GSWs, 13,812 (76.8%) were treated at Level-Is and 4153 (23.2%) at Level-IIs. There was no difference in the injury severity score (ISS) (p = 0.55). The Level-I cohort had a higher rate of laparotomy (38.9% vs. 36.5%, p

We conducted a genome-wide association study of host resistance to severePlasmodium falciparummalaria in over 17,000 individuals from 11 malaria-endemic countries, undertaking a wide ranging analysis which identifies five replicable associations with genome-wide levels of evidence. Our findings include a newly implicated variant on chromosome 6 associated with risk of cerebral malaria, and the discovery of an erythroid-specific transcription start site underlying the association inATP2B4. Previously reported HLA associations cannot be replicated in this dataset. We estimate substantial heritability of severe malaria (h2~ 23%), of which around 10% is explained by the currently identified associations. Our dataset will provide a major building block for future research on the genetic determinants of disease in these diverse human populations.

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.

Background Squamous cell carcinoma of the scalp is a common clinical problem in an aging population. Despite its high incidence, little has been documented regarding treatment or outcomes. Methods We retrospectively analysed 235 cases treated with curative intent at Peter MaCallum Cancer Centre between 1998 and 2010. The cohort was analysed for its characteristics, management, survival and prognostic factors. Results The patients were primarily male (88%) with a median age of 79 years (range 53–98 years). There was a high proportion of immunosuppressed patients (29%) and stage T2 (48%) tumours. Management included surgery (45%), radiotherapy (28%) and surgery and adjuvant radiotherapy (26%). Median follow up from treatment was 4.5 years. Estimated 5-year overall survival (OS), disease-specific survival and progression-free survival (PFS) were 59, 94 and 51%, respectively. The 5-year cumulative incidence of local and regional relapse was 11 and 7%, respectively. There were four patients who developed distant metastases and died of their disease. Statistically significant prognostic factors identified for poor outcomes for OS and PFS were T2 stage (hazard ratio [1.7 and 2.1) and immunosuppression (HR 3.3 and 3.4). Conclusions We conclude the presence of immunosuppression and T2 stage is prognostic for survival. Further research to establish treatment principles is warranted.

The difficulties in establishing and delivering reliable clinical hematology and laboratory services in resource-limited settings are well recognized. However, much can be achieved by better use of existing resources through a concerted quality improvement approach. The recommendations of this article are based in part upon work in the thalassemias, inherited disorders of hemoglobin that are widely prevalent in Asia, which may serve as a model that is applicable to other common, chronic disorders in resource-poor settings. Available resources are highlighted and recommendations made regarding approaches to improving services. Over the last few years, a number of low and middle-income countries, obtaining support from appropriate governmental sources, have identified and overcome difficulties and significantly improved clinical services for patients with thalassemia.

Background Visual impairment in developing countries has both social and economic impact on individuals and communities. Understanding the subjective visual functioning of populations will allow for local policymakers to identify the need for optometric or ophthalmic services in their communities. Methods The authors surveyed 644 adult patients in Mwanza, Tanzania at three clinics (Buzuruga, Mwananchi and Kisesa) using a modified Visual Functioning Questionnaire 25. Responses were categorized into General health, General vision, Ocular pain, Near activities, Distance activities, Social function, Mental health, Role difficulties, Color vision, Peripheral vision and Dependency. Results Patients at Buzuruga reported the lowest scores on most subscales. Of 100 employed patients, 37% claimed to have at least some difficulty in performing job duties due to their eyesight. At Kisesa, 146 (246/221) patients (66.1%) had never had an eye exam, compared with 134/227 (59.0%) at Buzuruga and 69/173 (39.9%) at Mwananchi (p Conclusions Due to regional differences in visual functioning in Mwanza, a national effort for vision health cannot be entirely successful without addressing the individualized needs of local communities. Reducing the cost of vision care appointments may expand vision health care utilization in Mwanza.