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Hôpital-Camfrout, France

Watson L.,University of Liverpool | Tullus K.,NHS Hospital for Children Trust Hospital | Marks S.D.,NHS Hospital for Children Trust Hospital | Holt R.C.L.,Alder Hey Childrens NHS Foundation Trust Hospital | And 2 more authors.
Journal of Clinical Immunology | Year: 2012

Purpose Sphingosine-1-phosphate (S1P) is an active sphin-golipid with chemotactic abilities and has been linked to inflammatory mediators and autoimmune disease. The aim of this study was to assess whether children with juvenileonset systemic lupus erythematosus (JSLE) express increased systemic and/or urinary concentrations of S1P. Methods A subgroup of patients participating in the UK JSLE Cohort Study, were invited to participate. Cross sectional serum and urine samples were prospectively collected along with demographic and standard clinical data. Results were compared to a cohort of disease controls (Henoch Schonlein Purpura; HSP) and healthy controls (HC). Results The median age of JSLE patients (n015) was 13.6 years (7.2-16.9 years). The serum concentrations of S1P in JSLE patients (7.4 uM, IQR 6.3-12.3 uM) were statistically significantly increased when compared to patients with HSP (n010; 5.2 uM, IQR 4.0-7.9 uM; p0 0.016) and HCs (n010; 3.8 uM, IQR 2.1-5.8 uM; p0 0.003). There was a trend towards increased serum S1P concentrations between patients with active lupus nephritis (n08; 8.7 uM, IQR 6.2-15.3 uM) compared to lupus non-nephritis (n07; 6.6 uM, IQR 6.3-10.6 uM; p00.355). No relationship was found between disease activity markers and S1P. Urine S1P concentrations were no different between JSLE patients (56.0 nM, IQR 40.3-96.6 nM) and HCs (58.7 nM, IQR 0-241.9 nM; p00.889). Conclusions We have demonstrated, for the first time, an increased serum concentration of S1P in a cohort of JSLE patients. These findings highlight a role of S1P in the pathophysiology of JSLE that warrants further investigation. © Springer Science+Business Media, LLC 2012.

Collado P.,Hospital Universitario Severo Ochoa | Gamir M.L.,Hospital Universitario Ramon y Cajal | Lopez-Robledillo J.C.,Paediatric Rheumatology | Merino R.,Hospital Universitario La Paz | And 2 more authors.
Clinical and Experimental Rheumatology | Year: 2014

Objective: To determine the prevalence of abnormalities detected by ultrasonography (US) in children with juvenile idiopathic arthritis (JIA) showing clinically inactive disease (ID) on medication and off medication. Methods: Inclusion criteria: 1) JIA patients, 2) clinician-determined ID, 3) JIA drugs withdrawal or stably dosed modified anti- rheumatic drugs (DMARDs) therapy for at least 6 months prior to inclusion, 4) biologics naïve patients. Clinical and US assessments were performed on 44 joints, which were scored for grey-scale (GS) synovitis and Power Doppler (PD) signal. PD signal inside intra-articular synovium or tendon sheath was considered as inflammatory activity. Results: Thirty-four patients were included, of whom 23 patients were labelled as ID on medication and 11 patients without medication. The duration of the current episode of ID at the inclusion time was 9.5 months. Although it was longer for the group off medication there was no significant difference between the two groups (p=0.06). Thirteen patients presented US findings. Number of US-detected synovial abnormalities was higher in patients on medication, but there were no significant differences between both groups in the detection of GS synovitis (p=0.86), GS tenosynovitis (p=0.78) and PD signal (p=0.38). Out of 37 joints presenting US-determined GS-synovitis, 18 joints showed PD signal. Conclusion: Our study provides evidence of synovitis and tenosynovitis on B-mode US in JIA patients with clinical inactivity. In addition, inflammatory activity upheld by power-Doppler has been shown in a few joints from patients on medication. © Clinical and Experimental Rheumatology 2014.

Hayem F.,Paediatric Rheumatology | Hayem G.,Bichat Claude Bernard Hospital
Medical Hypotheses | Year: 2012

Although Still's disease has been first described more than one century ago, it still appears as an orphan entity, which should be separated from the other auto-inflammatory diseases (AID). The main reason to individualize Still's disease among the AID is the absence of any genetic predisposition. Recently, the human mitochondria have been clearly implicated in the systemic inflammation that is observed during the innate immune response. After various types of cellular injuries, including infections, the release of "Damage-Associated Molecular Patterns" (DAMPs) from mitochondria can recruit circulating polymorphonuclear neutrophils (PMNs), monocytes and macrophages, along with the activation of NLRP3 inflammasome. Flares of Still's disease usually mimic systemic bacterial infections, with high levels of PMNs, but no evidence of circulating bacteria. Ubiquitous and usually benign viruses, such as human herpes virus 6 (HHV-6), appear capable of inducing mitochondrial damages. Such a phenomenon could in turn initiate the flares of Still's disease, thereafter persisting as an inappropriate and self-perpetuating reaction to an endogenous bacterial vestige, the mitochondrion itself. © 2012 Elsevier Ltd.

Smith E.,Paediatric Rheumatology | Molyneux E.,University of Malawi | Heikens G.T.,University of Malawi | Foster H.,Northumbria University
Clinical Rheumatology | Year: 2012

The pGALS (paediatric Gait, Arms, Legs, Spine) Musculoskeletal (MSK) screen is validated in English-speaking school-aged children and has been shown to be useful in acute paediatric practice in the UK. The aim of this study is to evaluate the practicality and acceptability of pGALS in children in an acute hospital setting in Malawi. School-aged inpatients and children presenting to the Queen Elizabeth Hospital Blantyre, Malawi, participated. Practicality (time taken, degree of completion) and patient/parent assessed acceptability (time take, discomfort) were assessed using a 'smiley face' visual analogue scale. Fifty-one children (median age 8 years) were assessed; 23 out of 51 (45%) in the emergency department and the remainder were inpatients. Most presentations were infection or trauma related (n = 35, 69%). Practicality of pGALS was good [median time to complete pGALS-4 min (range 1.8-7.4)] and completed in 48 out of 51 children (94%). Acceptability was high; 98% of parents considered the time taken to be acceptable, 84% of children deemed little/no additional discomfort. Abnormalities using pGALS were found in 21 out of 51 (41%), mostly in the lower limbs. The pGALS MSK screen was practical and acceptable in this acute setting. Abnormal findings were common. © 2011 Clinical Rheumatology.

Hofer M.,University of Lausanne | Pillet P.,Paediatric Rheumatology | Cochard M.,University of Lausanne | Berg S.,Gothenburg University | And 12 more authors.
Rheumatology (United Kingdom) | Year: 2014

Objectives: The aims of this study were to describe the clinical features of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) and identify distinct phenotypes in a large cohort of patients from different countries. Methods: We established a web-based multicentre cohort through an international collaboration within the periodic fevers working party of the Pediatric Rheumatology European Society (PReS). The inclusion criterion was a diagnosis of PFAPA given by an experienced paediatric rheumatologist participating in an international working group on periodic fever syndromes. Results: Of the 301 patients included from the 15 centres, 271 had pharyngitis, 236 cervical adenitis, 171 oral aphthosis and 132 with all three clinical features. A total of 228 patients presented with additional symptoms (131 gastrointestinal symptoms, 86 arthralgias and/or myalgias, 36 skin rashes, 8 neurological symptoms). Thirty-one patients had disease onset after 5 years and they reported more additional symptoms. A positive family history for recurrent fever or recurrent tonsillitis was found in 81 patients (26.9%). Genetic testing for monogenic periodic fever syndromes was performed on 111 patients, who reported fewer occurrences of oral aphthosis or additional symptoms. Twenty-four patients reported symptoms (oral aphthosis and malaise) outside the flares. The CRP was >50 mg/l in the majority (131/190) of the patients tested during the fever. Conclusion: We describe the largest cohort of PFAPA patients presented so far. We confirm that PFAPA may present with varied clinical manifestations and we show the limitations of the commonly used diagnostic criteria. Based on detailed analysis of this cohort, a consensus definition of PFAPA with better-defined criteria should be proposed. © The Author 2014. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved.

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