Clinical and laboratory characteristics in juvenile-onset systemic lupus erythematosus across age groups.

BACKGROUND: Systemic lupus erythematous (SLE) is a systemic autoimmune/inflammatory condition. Approximately 15-20% of patients develop symptoms before their 18th birthday and are diagnosed with juvenile-onset SLE (JSLE). Gender distribution, clinical presentation, disease courses and outcomes vary significantly between JSLE patients and individuals with adult-onset SLE. This study aimed to identify age-specific clinical and/or serological patterns in JSLE patients enrolled to the UK JSLE Cohort Study. METHODS: Patient records were accessed and grouped based on age at disease-onset: pre-pubertal (≤7 years), peri-pubertal (8-13 years) and adolescent (14-18 years). The presence of American College of Rheumatology (ACR) classification criteria, laboratory results, disease activity [British Isles Lupus Assessment Group (BILAG) and Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K) scores] and damage [Systemic Lupus International Collaborating Clinics (SLICC) damage index] were evaluated at diagnosis and last follow up. RESULTS: A total of 418 JSLE patients were included in this study: 43 (10.3%) with pre-pubertal disease onset; 240 (57.4%) with peri-pubertal onset and 135 (32.3%) were diagnosed during adolescence. At diagnosis, adolescent JSLE patients presented with a higher number of ACR criteria when compared with pre-pubertal and peri-pubertal patients [pBILAG2004 scores: 9(4-20] vs. 7(3-13] vs. 7(3-14], respectively, p = 0.015] with increased activity in the following BILAG domains: mucocutaneous (p = 0.025), musculoskeletal (p = 0.029), renal (p = 0.027) and cardiorespiratory (p = 0.001). Furthermore, adolescent JSLE patients were more frequently ANA-positive (p = 0.034) and exhibited higher anti-dsDNA titres (p = 0.001). Pre-pubertal individuals less frequently presented with leukopenia (p = 0.002), thrombocytopenia (p = 0.004) or low complement (p = 0.002) when compared with other age groups. No differences were identified in disease activity (pBILAG2004 score), damage (SLICC damage index) and the number of ACR criteria fulfilled at last follow up. CONCLUSIONS: Disease presentations and laboratory findings vary significantly between age groups within a national cohort of JSLE patients. Patients diagnosed during adolescence exhibit greater disease activity and "classic" autoantibody, immune cell and complement patterns when compared with younger patients. This supports the hypothesis that pathomechanisms may vary between patient age groups.

Mass and the Dangers of Syncope.

Introduction Syncope is defined as a transient, self-limited loss of consciousness with an inability to maintain postural tone that is followed by spontaneous recovery. We revisit situational syncope focusing on one situation, Mass. Methods We interrogated our electronic syncope database for key terms associated with situational syncope. From the most commonly encountered situation, Mass, we interrogated the results of tilt testing performed to identify evidence of orthostatic hypotension. Results There were 110 cases of situational syncope identified with 56.3% (n=62) taking place at mass. All had tilt table testing performed and 15.4% (n=17) had evidence of orthostatic hypotension. Conclusion The multiple sudden changes in position during mass from sitting to kneeling to standing can precipitate an episode of orthostatic hypotension. Consideration should be given as to whether it is safe for older mass goers to be subjected to such significant orthostatic stress.

Outcomes following mycophenolate mofetil versus cyclophosphamide induction treatment for proliferative juvenile-onset lupus nephritis.

BACKGROUND: Juvenile-onset systemic lupus erythematosus (JSLE) is more severe than adult-onset disease, including more lupus nephritis (LN). Despite differences in phenotype/pathogenesis, treatment is based upon adult trials. This study aimed to compare treatment response, damage accrual, time to inactive LN and subsequent flare, in JSLE LN patients treated with mycophenolate mofetil (MMF) versus intravenous cyclophosphamide (IVCYC). METHODS: UK JSLE Cohort Study participants, ≤16 years at diagnosis, with ≥4 American College of Rheumatology criteria for SLE, with class III or IV LN, were eligible. Mann-Whitney U tests, Fisher's exact test and Chi-squared tests were utilized for statistical analysis. RESULTS: Of the patients, 34/51 (67%) received MMF, and 17/51 (33%) received IVCYC. No significant differences were identified at 4-8 and 10-14 months post-renal biopsy and last follow-up, in terms of renal British Isles Lupus Assessment Grade scores, urine albumin/creatinine ratio, serum creatinine, ESR, anti-dsDNA antibody, C3 levels and patient/physician global scores. Standardized Damage Index scores did not differ between groups at 13 months or at last follow-up. Inactive LN was attained 262 (141-390) days after MMF treatment, and 151 (117-305) days following IVCYC ( p = 0.17). Time to renal flare was 451 (157-1266) days for MMF, and 343 (198-635) days for IVCYC ( p = 0.47). CONCLUSION: This is the largest study to date investigating induction treatments for proliferative LN in children, demonstrating comparability of MMF and IVCYC.

Evaluation of the ACR and SLICC classification criteria in juvenile-onset systemic lupus erythematosus: a longitudinal analysis.

Objectives The Systemic Lupus International Collaborating Clinics (SLICC) group proposed revised classification criteria for systemic lupus erythematosus (SLICC-2012 criteria). This study aimed to compare these criteria with the well-established American College of Rheumatology classification criteria (ACR-1997 criteria) in a national cohort of juvenile-onset systemic lupus erythematosus (JSLE) patients and evaluate how patients' classification criteria evolved over time. Methods Data from patients in the UK JSLE Cohort Study with a senior clinician diagnosis of probable evolving, or definite JSLE, were analyzed. Patients were assessed using both classification criteria within 1 year of diagnosis and at latest follow up (following a minimum 12-month follow-up period). Results A total of 226 patients were included. The SLICC-2012 was more sensitive than ACR-1997 at diagnosis (92.9% versus 84.1% p < 0.001) and after follow up (100% versus 92.0% p < 0.001). Most patients meeting the SLICC-2012 criteria and not the ACR-1997 met more than one additional criterion on the SLICC-2012. Conclusions The SLICC-2012 was better able to classify patients with JSLE than the ACR-1997 and did so at an earlier stage in their disease course. SLICC-2012 should be considered for classification of JSLE patients in observational studies and clinical trial eligibility.

The effect of carotid stenting on endarterectomy practice--A single institution experience.

OBJECTIVES: The number of operations performed per surgeon is thought to determine the quality of carotid endarterectomy (CEA) surgery. The advent of carotid artery stenting (CAS) threatens to reduce the volume of CEA. This paper assesses CEA and the effects of the introduction of CAS service on outcomes. DESIGN: Retrospective cohort study. METHODS: Clinical data and results of CEA were reviewed retrospectively for the treatment of carotid stenosis, between January 1988 and December 2010. CEA patients were grouped into those treated before and after the introduction of CAS to our hospital in 2001. RESULTS: 757 patients underwent a CEA between 1988 and 2010. The perioperative stroke rate prior to the introduction of CAS was 4.9%, and 3.3% after stent introduction in 2001. In this latter period, 85.5% had symptomatic stenosis which suggests that the patients were not low risk. The major adverse event rate (inclusive of death and myocardial infarction) post introduction of CAS from 2001 to 2010 was 4.1%. There was no correlation between post-operative stroke/MAE and procedure volume, despite the trend of decreasing CEA numbers over time. CONCLUSION: The introduction of carotid artery stenting has led to a decrease in carotid endarterectomy volume. However, outcomes in our high risk patient population are acceptable. Therefore, CEA remains the procedure of choice for carotid artery revascularization.

Endovascular versus open repair of ruptured abdominal aortic aneurysm.

INTRODUCTION: Endovascular aneurysm repair (EVAR) is a comparatively less invasive technique than open repair (OR). Debate remains with regard to the benefit of EVAR for patients with ruptured abdominal aortic aneurysm (RAAA). We sought to evaluate and report outcomes of EVAR for RAAA in an Irish tertiary vascular referral centre. METHODS: Patients undergoing emergency surgery for ruptured or symptomatic AAA were identified from theatre logbooks and HIPE database. Retrospective chart review was undertaken. Data were exported to IBM SPSS version 21 for statistical analysis with p < 0.05 considered significant. RESULTS: A total of 41 patients underwent surgery for RAAA. The mean age was 74 years old with a range from 55 to 89 years. The majority (n = 25, 61%) were baseline American Society of Anaesthesiology (ASA) grade 3-4. Of these 56% underwent EVAR with the remaining 44% repaired open. Mortality rate in those undergoing emergency EVAR was 34.8%, compared with 38.9% in those undergoing open surgery. This difference was not statistically significant. The mean overall length of stay was 13 days. With regard to prognostic indicators of patient outcome, increasing patient age was noted to be significantly associated with increased mortality (p = 0.013), as was increased ASA score at time of surgery (p = 0.029). CONCLUSIONS: Mortality rates in those undergoing EVAR for RAAA are comparable with those undergoing open repair. Increasing age and ASA score are significant predictors of mortality in patients with RAAA undergoing intervention.

What next after thermal ablation for varicose veins: non-thermal ablation?

BACKGROUND: Endothermal treatment of the great saphenous vein has become the first line of treatment for superficial venous reflux. Newer treatments, especially non-thermal ablation have potential benefits both for patient acceptability and decreased risk of nerve injury. APPROACH: We describe the current non-thermal options available including advantages and disadvantages. Ultrasound guided foam sclerotherapy avoids the risk of nerve injury, however it is not as effective as endothermal ablation. Mechanochemical endovenous ablation combines mechanical endothelial damage using a rotating wire, with the infusion of a liquid sclerosant (the Clarivein™ system). Reports suggest that this system is safe and effective, eliminating the need for tumescent anaesthesia with no reported case of nerve injury. Finally the VenaSeal™ Sapheon Closure System comprises the endovenous delivery of cyanoacrylate tissue adhesive to the vein causing fibrosis. Peri-operative discomfort seems to be minimal but the complication of thrombophlebitis has been reported in up to 15% of patients. CONCLUSIONS: Non-thermal options promise comparable treatment efficacy without the added morbidity associated with high thermal energies. The potential of treating venous reflux without the risk of nerve damage may change how surgeons approach venous disease.

Immunocompromised children and young people are at no increased risk of severe COVID-19.

OBJECTIVES: We aimed to prospectively describe the incidence and clinical spectrum of SARS-CoV-2 infection in immunocompromised paediatric patients in the UK. METHODS: From March 2020 to 2021 weekly questionnaires were sent to immunocompromised paediatric patients or their parents. Information, including symptom presentation and SARS-CoV-2 PCR test results, was collected from 1527 participants from 46 hospitals. Cross-sectional serology was investigated in February and March 2021. RESULTS: Until the end of September 2020, no cases were reported. From September 28th 2020 to March 2021 a total of 38 PCR-detected SARS-CoV-2 infections were reported. Of these, four children were admitted to hospital but none had acute severe COVID-19. Increasing age in association with immunodeficiency increased reporting of SARS-CoV-2 infection. Worsening of fever, cough, and sore throat were associated with participants reporting SARS-CoV-2 infection. Serology data included 452 unvaccinated participants. In those reporting prior positive SARS-CoV-2 PCR, there were detectable antibodies in 9 of 18 (50%). In those with no prior report of infection, antibodies were detected in 32 of 434 (7•4%). CONCLUSIONS: This study shows SARS-CoV-2 infections have occurred in immunocompromised children and young people with no increased risk of severe disease. No children died.

Western Australian adolescent emotional wellbeing during the COVID-19 pandemic in 2020.

BACKGROUND: The impacts of the COVID-19 pandemic have been vast and are not limited to physical health. Many adolescents have experienced disruptions to daily life, including changes in their school routine and family's financial or emotional security, potentially impacting their emotional wellbeing. In low COVID-19 prevalence settings, the impact of isolation has been mitigated for most young people through continued face-to-face schooling, yet there may still be significant impacts on their wellbeing that could be attributed to the pandemic. METHODS: We report on data from 32,849 surveys from Year 7-12 students in 40 schools over two 2020 survey cycles (June/July: 19,240; October: 13,609), drawn from a study of 79 primary and secondary schools across Western Australia, Australia. The Child Health Utility Index (CHU9D) was used to measure difficulties and distress in responding secondary school students only. Using comparable Australian data collected six years prior to the pandemic, the CHU9D was calibrated against the Kessler-10 to establish a reliable threshold for CHU9D-rated distress. RESULTS: Compared to 14% of responding 12-18-year-olds in 2013/2014, in both 2020 survey cycles almost 40% of secondary students returned a CHU9D score above a threshold indicative of elevated difficulties and distress. Student distress increased significantly between June and October 2020. Female students, those in older Grades, those with few friendships or perceived poor quality friendships, and those with poor connectedness to school were more likely to score above the threshold. CONCLUSIONS: In a large dataset collected during the first year of the COVID-19 pandemic, the proportion of secondary school students with scores indicative of difficulties and distress was substantially higher than a 2013/2014 benchmark, and distress increased as the pandemic progressed, despite the low local prevalence of COVID-19. This may indicate a general decline in social and emotional wellbeing exacerbated by the events of the pandemic. TRIAL REGISTRATION: ANZCTRN (ACTRN12620000922976). Retrospectively registered 17/08/2020. https://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=380429&isReview=true .

Making the case for cardiovascular screening in Irish males: detection of abdominal aortic aneurysms, and assessment of cardiovascular risk factors.

INTRODUCTION: AAA screening programmes have proven to be beneficial and cost effective worldwide for males greater than 65 years of age, with 4.9% males of 65-75 years of age having an un-diagnosed AAA at screening, resulting in a 42% reduction in the risk of rupture in an English population. This study assessed the incidence of AAA and risk factors for atherosclerosis in Irish males of 55-75 years. METHODS: From April 2006 to December 2007, males between the ages of 55 and 75 years, living within the catchment area of Blanchardstown Hospital were invited for AAA screening using duplex ultrasound and cardiovascular risk factor screening. RESULTS: 1.9% (17/904) of the study population had previously un-diagnosed aneurysms detected, with sizes ranging from 3.0 cm to 5.8 cm (0.6% in 55-65 years old (yo) and 4.2% in 65-75 yo, p<0.01). 33% (302/904) of patients had hyperlipidaemia, while 16% of those with a previous diagnosis of hyperlipidaemia, were inadequately controlled on the test date. 31% of patients had a single elevated blood pressure reading, meriting further investigation for possible hypertension. 3% (28/904) of all patients had a raised glucose levels which had not previously been identified and of those who had a previous history of DM, 46% had abnormal glucose levels. 16% of patients (93/573) were morbidly obese (BMI>30) and 64% (292/573) were overweight. CONCLUSION: The incidence of AAAs in 65-75-year-old men is similar to international figures. This study confirms that screening for hyperlipidaemia, hypercholesterolaemia, obesity and hypertension may be worthwhile in all males over 55 years, while AAA screening should be reserved for 65-75-year-old Irish males.

Concurrent colorectal malignancy and abdominal aortic aneurysm: a multicentre experience and review of the literature.

OBJECTIVES: There is lack of consensus regarding concurrent vs. staged approaches, and the prioritisation of staged procedures in cases presenting with colorectal carcinoma (CRC) and abdominal aortic aneurysm (AAA) synchronously. We aim to present our experience, review the literature on this therapeutic dilemma and examine the role of endovascular aortic repair (EVAR). DESIGN, MATERIALS AND METHODS: An observational study of the experience of two centres and a systematic review of the published literature. RESULTS: Twenty-four patients were identified from the prospective databases of two tertiary referral centres between 2001 and 2006. Intervention for both malignancy and aneurysm was performed in 13 patients. In 10 patients, cancer resection was performed initially and was followed by open aneurysm repair (n=3) or EVAR (n=7). Two patients (AAA diameters: 7.0 and 8.0cm) underwent EVAR prior to colonic resection. One patient was selected for synchronous surgery. There were no interval AAA ruptures, graft infection or postoperative mortalities. Literature review identified 269 such cases; of these 101 were treated by combined surgery. In staged surgery, there were nine interval aneurysmal ruptures and one aortic graft infection. CONCLUSIONS: In our experience, staged management can be undertaken, without interval aneurysmal rupture. EVAR has an evolving role in preventing delay in CRC management, in high-risk patients, and during combined intervention.

Virtual reality simulation in endovascular surgical training.

BACKGROUND: Shortened trainingtimes duetothe European Working Time Directive (EWTD) and increased public scrutiny of surgical competency have led to a move away from the traditional apprenticeship model of training. Virtual reality (VR) simulation is a fascinating innovation allowing surgeons to develop without the need to practice on real patients and it may be a solution to achieve competency within a shortened training period. METHOD: A Medline search was performed to identify studies and commentaries on the use of VR simulators in endovascular training. FINDINGS: Three studies on carotid stenting and four on peripheral vascular angioplasty demonstrate that simulator training is a valid, feasible and acceptable training tool. One randomised study reports that these skills learned on simulators are transferable to the operating room. CONCLUSION: VR simulators have a role in competency based, structured training of vascular interventionalists and should improve patient safety.

Synchronous appendiceal tumours.

Primary appendiceal neoplasms are a rare clinical and pathological entity. We report a case of synchronous appendiceal tumours of different histological types which presented as a symptomatic palpable and radiologically apparent mass in the right iliac fossa. This case demonstrates the importance of pre-operative diagnosis of these neoplasms, as it may alter the surgical approach and obviate the need for additional surgery. Furthermore, some of the controversies associated with the management of an appendix mass in the elderly population are discussed.

Perforation of a retrocaecal appendix presenting clinically as a right lumbar abscess.

Presentations of acute appendicitis represent an ongoing challenge to surgeons. This case of retrocaecal perforated appendicitis presented clinically as a right lumbar abscess, with the concomitant new diagnosis of type 2 diabetes mellitus in a 66-year-old male. This case emphasises the importance of an abdominal CT scan in any patient presenting with unusual sepsis near the abdomen.

Faecal Campylobacter shedding among dogs in animal shelters across Texas.

Epidemiologic studies on faecal Campylobacter shedding among dogs in the United States have been limited, despite evidence that the incidence of human campylobacteriosis has increased over the last decade. Our objectives were to estimate the prevalence of faecal Campylobacter shedding among shelter dogs in Texas, to estimate the specific prevalence of Campylobacter jejuni and Campylobacter coli shedding, and to identify risk factors for Campylobacter-positive status. Using a cross-sectional study design, we collected faecal samples from dogs in six animal shelters across Texas between May and December, 2014. Quantitative PCR protocols were used to detect Campylobacter in samples and to specifically identify C. jejuni and C. coli. The prevalence of faecal Campylobacter shedding among sampled dogs was 75.7% (140/185). Prevalence varied significantly by shelter (p = .03), ranging from 57% to 93%. There was a marginal association (p = .06) between abnormal faecal consistency and positive Campylobacter status, after controlling for shelter as a random effect. However, approximately 70% of Campylobacter-positive dogs had grossly normal faeces. Campylobacter prevalence did not vary significantly by age group or sex. The prevalence of C. jejuni-positive samples was 5.4% (10/185), but C. coli was not detected in any samples. Dogs are a potential source of zoonotic Campylobacter transmission.

A new look at an under-diagnosed poorly treated condition: restless legs syndrome.

Restless legs syndrome (RLS) is a common condition that is difficult to diagnose. However, once correctly diagnosed, its management is straightforward and evidence-based. The purpose of this article is to familiarise clinicians with both the symptoms and diagnostic criteria for RLS and to elucidate secondary and often curable causes of this prevalent condition. There is also a concise review of its pathophysiology and current management strategies. The authors hope this article will raise awareness of this condition among clinicians and in doing so improve the quality of life for the patients affected by it.

Adult intussusception--need for en-bloc resection.

BACKGROUND: Adult intussusception is an uncommon surgical presentation AIMS: We report a case of adult intussusception, review the literature and discuss the optimal management. METHODS: We describe a woman who presented with severe abdominal pain and a large supra-umbilical mass. Ileocolic intussception was confirmed on CT, and a laparotomy and en-bloc resection were carried out. Postoperatively she made an uneventful recovery. CONCLUSION: Adult intussusception is a rare clinical presentation. En-bloc resection should be the surgical treatment of choice in the majority of cases due to the high percentage of malignant lead points.

Evaluation of Faecal Salmonella Shedding Among Dogs at Seven Animal Shelters across Texas.

Estimates of prevalence of faecal Salmonella shedding among dogs in the United States have varied widely. Surveillance among shelter dogs has been limited, although dogs in animal shelters may be at elevated risk of Salmonella infection because of their previous exposure history as well as factors inherent to shelter environments. Our objectives were to estimate the prevalence of Salmonella shedding among shelter dogs across Texas, to identify risk factors for shedding and to characterize the isolates. Using a repeated cross-sectional study design, we collected faecal samples from dogs on two or three visits to each of seven Texas animal shelters between May 2013 and December 2014. Standard bacteriologic culture methods were used to isolate Salmonella from samples, and isolates were characterized via serotyping and anti-microbial susceptibility testing. The prevalence of faecal Salmonella shedding among sampled dogs was 4.9% (27/554), and within-shelter prevalence ranged from 1.9% to 8.3%. There was a marginal association (P = 0.09) between watery faecal samples and positive Salmonella status, as estimated by a logistic regression model that controlled for shelter as a random effect. However, over 60% of Salmonella-positive dogs had grossly normal faeces. Salmonella prevalence did not vary significantly by age group or sex. The most common serovars were Newport (22%) and Javiana (15%), both of which were widespread among shelters. Resistance to anti-microbial agents was uncommon. The prevalence of faecal Salmonella shedding among shelter dogs in Texas appears to be comparable to that seen among pet dogs in general.