Use of the Auto-inflammatory Disease Activity Index to monitor disease activity in patients with colchicine-resistant Familial Mediterranean Fever, Mevalonate Kinase Deficiency, and TRAPS treated with canakinumab

To evaluate the feasibility of the autoinflammatory disease activity index (AIDAI) as a tool to assess disease activity in patients with hereditary recurrent fever syndromes (HRFs) treated with canakinumab

Clinical efficacy and satisfaction of a digital wheeze detector in a multicentre randomised controlled trial: the WheezeScan study.

INTRODUCTION: Wheezing is common in preschool children and its clinical assessment often challenging for caretakers. This study aims to evaluate the impact of a novel digital wheeze detector (WheezeScan™) on disease control in a home care setting. METHODS: A multicentre randomised open-label controlled trial was conducted in Berlin, Istanbul and London. Participants aged 4-84 months with a doctor's diagnosis of recurrent wheezing in the past 12 months were included. While the control group followed usual care, the intervention group received the WheezeScan™ for at-home use for 120 days. Parents completed questionnaires regarding their child's respiratory symptoms, disease-related and parental quality of life, and caretaker self-efficacy at baseline (T0), 90 days (T1) and 4 months (T2). RESULTS: A total of 167 children, with a mean±sd age of 3.2±1.6 years, were enrolled in the study (intervention group n=87; control group n=80). There was no statistically significant difference in wheeze control assessed by TRACK (mean difference 3.8, 95% CI -2.3-9.9; p=0.2) at T1 between treatment groups (primary outcome). Children's and parental quality of life and parental self-efficacy were comparable between both groups at T1. The evaluation of device usability and perception showed that parents found it useful. CONCLUSION: In the current study population, the wheeze detector did not show significant impact on the home management of preschool wheezing. Hence, further research is needed to better understand how the perception and usage behaviour may influence the clinical impact of a digital support

Relationship between serum bilirubin levels and metabolic syndrome in patients with schizophrenia spectrum disorders

Objective: We investigated the relationship between serum bilirubin levels and metabolic syndrome (MetS), and the longitudinal effects of baseline serum bilirubin concentrations on MetS in patients with schizophrenia spectrum disorders undergoing atypical antipsychotics. Methods: The sample of this study consisted of 131 patients with schizophrenia spectrum disorders. Waist circumference, blood pressure, and levels of triglycerides, high-density lipoprotein cholesterol, fasting glucose, and insulin were evaluated at baseline and at month six. Serum bilirubin levels were measured at baseline. Serum bilirubin levels of the patients with and without MetS criteria were compared. We also compared patients with high and low bilirubin levels (upper and lower 50th percentiles of serum bilirubin levels) in terms of MetS criteria, MetS frequency, and course of MetS. Results: Serum direct bilirubin levels were more consistently related to MetS and MetS-related variables. The waist circumference and triglyceride criteria for MetS were significantly related to low serum direct bilirubin at baseline; waist circumference and fasting glucose criteria, and insulin resistance were associated with low serum direct bilirubin at follow-up. MetS diagnosis and the presence of the waist circumference criterion were more frequent at the baseline and the follow-up in low bilirubin group. At the end of the follow-up period, the rate of reverse MetS was significantly higher in the high bilirubin group. Conclusion: Our results have suggested that serum direct bilirubin levels showed a more reliable and stable relationship with abdominal obesity for MetS components.in patients with schizophrenia spectrum disorders using antipsychotics. Further studies are required. Copyright © 2017, Korean College of Neuropsychopharmacology

Reliability of dynamic causal modelling of resting‐state magnetoencephalography

This study assesses the reliability of resting‐state dynamic causal modelling (DCM) of magnetoencephalography (MEG) under conductance‐based canonical microcircuit models, in terms of both posterior parameter estimates and model evidence. We use resting‐state MEG data from two sessions, acquired 2 weeks apart, from a cohort with high between‐subject variance arising from Alzheimer's disease. Our focus is not on the effect of disease, but on the reliability of the methods (as within‐subject between‐session agreement), which is crucial for future studies of disease progression and drug intervention. To assess the reliability of first‐level DCMs, we compare model evidence associated with the covariance among subject‐specific free energies (i.e., the ‘quality’ of the models) with versus without interclass correlations. We then used parametric empirical Bayes (PEB) to investigate the differences between the inferred DCM parameter probability distributions at the between subject level. Specifically, we examined the evidence for or against parameter differences (i) within‐subject, within‐session, and between‐epochs; (ii) within‐subject between‐session; and (iii) within‐site between‐subjects, accommodating the conditional dependency among parameter estimates. We show that for data acquired close in time, and under similar circumstances, more than 95% of inferred DCM parameters are unlikely to differ, speaking to mutual predictability over sessions. Using PEB, we show a reciprocal relationship between a conventional definition of ‘reliability’ and the conditional dependency among inferred model parameters. Our analyses confirm the reliability and reproducibility of the conductance‐based DCMs for resting‐state neurophysiological data. In this respect, the implicit generative modelling is suitable for interventional and longitudinal studies of neurological and psychiatric disorders

Recurrent post-partum coronary artery dissection

Coronary artery dissection is a rare but well-described cause for myocardial infarction during the post-partum period. Dissection of multiple coronary arteries is even less frequent. Here we present a case of recurrent post-partum coronary artery dissections. This unusual presentation poses unique problems for management. A 35 year-old female, gravida 3 para 2, presented with myocardial infarction 9 weeks and 3 days post-partum. Cardiac catheterization demonstrated left anterior descending (LAD) dissection but an otherwise normal coronary anatomy. The lesion was treated with four everolimus eluting stents. Initially the patient made an unremarkable recovery until ventricular fibrillation arrest occurred on the following day. Unsynchronized cardioversion restored a normal sinus rhythm and repeat catheterization revealed new right coronary artery (RCA) dissection. A wire was passed distally, but it was unclear whether this was through the true or false lumen and no stents could be placed. However, improvement of distal RCA perfusion was noted on angiogram. Despite failure of interventional therapy the patient was therefore treated conservatively. Early operation after myocardial infarction has a significantly elevated risk of mortality and the initial dissection had occurred within 24 hours. This strategy proved successful as follow-up transthoracic echocardiography after four months demonstrated a preserved left ventricular ejection fraction of 55-60% without regional wall motion abnormalities. The patient remained asymptomatic from a cardiac point of view

Quality standards for managing children and adolescents with bronchiectasis: an international consensus

The global burden of bronchiectasis in children and adolescents is being recognised increasingly. However, marked inequity exists between, and within, settings and countries for resources and standards of care afforded to children and adolescents with bronchiectasis compared with those with other chronic lung diseases. The European Respiratory Society (ERS) clinical practice guideline for the management of bronchiectasis in children and adolescents was published recently. Here we present an international consensus of quality standards of care for children and adolescents with bronchiectasis based upon this guideline. The panel used a standardised approach that included a Delphi process with 201 respondents from the parents and patients’ survey, and 299 physicians (across 54 countries) who care for children and adolescents with bronchiectasis. The seven quality standards of care statements developed by the panel address the current absence of quality standards for clinical care related to paediatric bronchiectasis. These internationally derived, clinician-, parent-and patient-informed, consensus-based quality standards statements can be used by parents and patients to access and advocate for quality care for their children and themselves, respectively. They can also be used by healthcare professionals to advocate for their patients, and by health services as a monitoring tool, to help optimise health outcomes.</p

Risk of infections in bronchiectasis during disease-modifying treatment and biologics for rheumatic diseases

<p>Abstract</p> <p>Background</p> <p>Bronchiectasis is frequently associated (up to 30%) with chronic inflammatory rheumatic diseases and leads to lower respiratory tract infections. Data are lacking on the risk of lower respiratory tract infections in patients treated with biologic agents.</p> <p>Methods</p> <p>Monocenter, retrospective systematic study of all patients with a chronic inflammatory rheumatic disease and concomitant bronchiectasis, seen between 2000 and 2009. Univariate and multivariate analyses were performed to evidence predictive factors of the number of infectious respiratory events.</p> <p>Results</p> <p>47 patients were included (mean age 64.1 ± 9.1 years, 33 (70.2%) women), with a mean follow-up per patient of 4.3 ± 3.1 years. Rheumatoid arthritis was the main rheumatic disease (90.1%). The mean number of infectious events was 0.8 ± 1.0 event per patient-year. The factors predicting infections were the type of treatment (biologic vs. non biologic disease-modifying treatments), with an odds ratio of 8.7 (95% confidence interval: 1.7-43.4) and sputum colonization by any bacteria (odds ratio 7.4, 2.0-26.8). In multivariate analysis, both factors were independently predictive of infections.</p> <p>Conclusion</p> <p>Lower respiratory tract infectious events are frequent among patients receiving biologics for chronic inflammatory rheumatic disease associated with bronchiectasis. Biologic treatment and pre-existing sputum colonization are independent risk factors of infection occurrence.</p