Diagnosis and investigation of suspected haemophagocytic lymphohistiocytosis in adults: 2023 Hyperinflammation and HLH Across Speciality Collaboration (HiHASC) consensus guideline.

Haemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome characterised by persistently activated cytotoxic lymphocytes and macrophages, which, if untreated, leads to multiorgan dysfunction and death. HLH should be considered in any acutely unwell patient not responding to treatment as expected, with prompt assessment to look for what we term the three Fs-fever, falling blood counts, and raised ferritin. Worldwide, awareness of HLH and access to expert management remain inequitable. Terminology is not standardised, classification criteria are validated in specific patient groups only, and some guidelines rely on specialised and somewhat inaccessible tests. The consensus guideline described in this Health Policy was produced by a self-nominated working group from the UK network Hyperinflammation and HLH Across Speciality Collaboration (HiHASC), a multidisciplinary group of clinicians experienced in managing people with HLH. Combining literature review and experience gained from looking after patients with HLH, it provides a practical, structured approach for all health-care teams managing adult (>16 years) patients with possible HLH. The focus is on early recognition and diagnosis of HLH and parallel identification of the underlying cause. To ensure wide applicability, the use of inexpensive, readily available tests is prioritised, but the role of specialist investigations and their interpretation is also addressed.

UK internal medicine training in the time of COVID-19.

COVID-19 has had a significant impact on internal medicine training in the UK. Many unprecedented changes have been made to prioritise the care of affected patients. The medical workforce was re-shaped, training programmes were disrupted, Membership of the Royal College of Physicians (MRCP) examinations were cancelled and their format changed on re-commencement, teaching programmes were suspended and delivery methods amended, out of programme (OOP) opportunities deferred, non-COVID related research halted, trainee progression impacted and trainee mental health and wellbeing suffered. Despite this, the pandemic has undoubtedly created a plethora of unique learning opportunities for trainees that could make them better doctors and healthcare leaders in the future.

Bone health in idiopathic inflammatory myopathies.

OBJECTIVE: To review the extant literature relating to bone health in the idiopathic inflammatory myopathies (IIM) including both adult and juvenile patients. METHODS: A PubMed search® identified relevant studies from 1966 to 2020 in accordance with PRISMA guidelines. Two independent reviewers screened and extracted the abstracts/full manuscripts, and a third author was consulted in the case of disagreement. RESULTS: We identified 37 articles (3 review articles, 2 RCTs, 9 cross-sectional, 16 cohort and 7 case-control studies). The prevalence of osteopenia (n = 7) ranges from 7 to 75% and osteoporosis (n = 7) between 13% to 27%. The prevalence of vertebral fractures ranged from 11 to 75%. Systemic inflammation likely contributes to reduced bone mineral density (BMD) in children with IIM but data is currently lacking in adult patients. Association between with impaired BMD and Vitamin D or calcium intake and physical activity has not been demonstrated in IIM. There is no clear consensus regarding the impact of age, menopause or BMI on bone health. Gender, smoking status, disease activity and inflammatory markers are not obvious independent predictors of low BMD. Several studies have demonstrated that glucocorticoids are associated with an increased risk of low BMD. There are no specific guidelines relating to the management of bone health in adult and juvenile patients with IIM. CONCLUSION: Both adult and juvenile patients with IIM are at high risk of impaired bone health and fracture. The mechanisms behind this are likely multifactorial including systemic inflammation, glucocorticoid treatment, reduced mobility and impaired calcium/vitamin D homeostasis. There are a lack of guidelines and studies relating to the screening, prevention and treatment of impaired bone health in adult and juvenile patients with IIM. Future research is required to understand the complexity of bone health in IIM including to develop much needed disease-specific management recommendations.

ETV for successful treatment of holocord syrinx with hydrocephalus: a case report.

AIM: to present evidence for the use of endoscopic third ventriculostomy (ETV) in the treatment of holocord syrinx. METHODS: ETV has been used in the treatment of obstructive hydrocephalus and syringomyelia secondary to Chiari 1 malformation. However, there have been no reports of ETV being utilised in the management of a holocord. We report a case of an 18 year old male with a symptomatic holocord syrinx who was successfully treated via ETV. RESULTS: neurological improvement was noted both immediately and at follow up following ETV. CONCLUSION: ETV may represent a viable treatment option for holocord syrinx in some population groups.

A systematic review of internet-based information on dermatomyositis and polymyositis.

AIM: Patients with rheumatic diseases are increasingly using internet-based information to inform healthcare utilization and make treatment decisions. Our aim was to assess the readability and quality of internet-based information on dermatomyositis (DM) and polymyositis (PM). METHOD: Key words "Dermatomyositis" and "Polymyositis" were searched on 3 commonly used search engines (Google, Yahoo and Bing). The first 3 pages (~30) of search results were examined from each search engine. Readability of information was assessed using 4 readability formulae (Flesch Reading Ease Score, Flesch-Kincaid Grade Level, the Simplified Measure of Gobbledygook index, the Coleman-Liau index). Quality of information was assessed using the DISCERN tool, Journal of The American Medical Association (JAMA) benchmark criteria and Health on The Net Code (HoN code). We also examined Google Trends® data to determine if there were obvious temporal search patterns. RESULTS: Thirty-two websites were included in the study after duplicates were removed and exclusion criteria were applied. The overall quality was low including DISCERN with a median overall score of 38/80 (interquartile range 12.25), only 4/32 (13%) websites fulfilled all 4 JAMA benchmark criteria, and 9/32 (28%) had HoN code. Readability of information was assessed using 4 readability formulae (Flesch Reading Ease Score, Flesch-Kincaid Grade Level, the Simplified Measure of Gobbledygook index, the Coleman-Liau index. There was no obvious temporal trend in searches on analysis of Google Trends® data. CONCLUSION: The overall quality and readability of internet-based information relating to DM and PM is poor. Patients require appropriate information of high quality and readability throughout the course of their disease in order to make informed decisions on their condition including treatment.

The prevalence and risk factors for acute respiratory infections in children aged 0-59 months in rural Malawi: A cross-sectional study.

BACKGROUND: Acute Respiratory Infections (ARI) are a leading cause of childhood mortality and morbidity. Malawi has high childhood mortality but limited data on the prevalence of disease in the community. METHODS: A cross-sectional study of children aged 0-59 months. Health passports were examined for ARI diagnoses in the preceding 12 months. Children were physically examined for malnutrition or current ARI. RESULTS: 828 children participated. The annual prevalence of ARI was 32.6% (95% CI 29.3-36.0%). Having a sibling with ARI (OR 1.44, P = .01), increasing household density (OR 2.17, P = .02) and acute malnutrition (OR 1.69, P = .01) were predictors of infection in the last year. The point prevalence of ARI was 8.3% (95% CI 6.8-10.4%). Risk factors for current ARI were acute-on-chronic malnutrition (OR 3.06, P = .02), increasing household density (OR1.19, P = .05) and having a sibling with ARI (OR 2.30, P = .02). CONCLUSION: This study provides novel data on the high prevalence of ARI in Malawi. This baseline data can be used in the monitoring and planning of future interventions in this population.