Chronic nonbacterial osteomyelitis in children: a retrospective multicenter study.

BACKGROUND: To determine the clinical presentation, current treatment and outcome of children with nonbacterial inflammatory bone disease. METHODS: Retrospective multicenter study of patients entered into the Swiss Pediatric Rheumatology Working Group registry with a diagnosis of chronic nonbacterial osteomyelitis (CNO) and synovitis acne pustulosis hyperostosis osteitis (SAPHO) syndrome. The charts were reviewed for informations about disease presentation, treatment, course and outcome. RESULTS: Forty-one children (31 girls and 10 boys) from 6 pediatric hospitals in Switzerland diagnosed between 1995 and 2010 were included in the study. The diagnosis was multifocal CNO (n = 33), unifocal CNO (n = 4) and SAPHO syndrome (n = 4). Mean age at onset of CNO was 9.5 years (range 1.4-15.6) and mean follow-up time was 52 months (range 6-156 months). Most patients (n = 27) had a chronic persistent disease course (>6 months), 8 patients had a course with one or more relapses and 6 patients had only one episode of CNO. Forty nine percent had received at least one course of antibiotics. In 57% treatment with nonsteroidal anti-inflammatory drugs (NSAID) was sufficient to control the disease. Twelve out of 16 children with NSAID failure subsequently received corticosteroids, methotrexate, TNF α inhibitors, bisphosphonates or a combination of these drugs. CONCLUSIONS: In a multicenter cohort of 41 children 22% started with unifocal lesion with a significant diagnostic delay. A higher proportion presented with chronic persistent disease than with a recurrent form. An osteomyelitis in the pelvic region is significantly associated with other features of juvenile spondylarthritis.

Phagocyte-specific S100 proteins and high-sensitivity C reactive protein as biomarkers for a risk-adapted treatment to maintain remission in juvenile idiopathic arthritis: a comparative study.

OBJECTIVES: Juvenile idiopathic arthritis (JIA) is a chronic inflammatory joint disease affecting children. Even if remission is successfully induced, about half of the patients experience a relapse after stopping anti-inflammatory therapy. The present study investigated whether patients with JIA at risk of relapse can be identified by biomarkers even if clinical signs of disease activity are absent. METHODS: Patients fulfilling the criteria of inactive disease on medication were included at the time when all medication was withdrawn. The phagocyte activation markers S100A12 and myeloid-related proteins 8/14 (MRP8/14) were compared as well as the acute phase reactant high-sensitivity C reactive protein (hsCRP) as predictive biomarkers for the risk of a flare within a time frame of 6 months. RESULTS: 35 of 188 enrolled patients experienced a flare within 6 months. Clinical or standard laboratory parameters could not differentiate between patients at risk of relapse and those not at risk. S100A12 and MRP8/14 levels were significantly higher in patients who subsequently developed flares than in patients with stable remission. The best single biomarker for the prediction of flare was S100A12 (HR 2.81). The predictive performance may be improved if a combination with hsCRP is used. CONCLUSIONS: Subclinical disease activity may result in unstable remission (ie, a status of clinical but not immunological remission). Biomarkers such as S100A12 and MRP8/14 inform about the activation status of innate immunity at the molecular level and thereby identify patients with unstable remission and an increased risk of relapse.

Size, shape and age-related changes of the mandibular condyle during childhood.

OBJECTIVE: To determine age-related differences in the size and shape of the mandibular condyle in children to establish anatomical reference values. METHODS: A total of 420 mandibular condyles in 210 children (mean age, 7 years) were retrospectively analysed by using computed tomography (CT) imaging. The greatest left-right (LRD) and anterior-posterior (APD) diameters and the anteversion angles (AA) were measured by two readers. An APD/LRD ratio was calculated. The shape of the condyles was graded into three types on sagittal images. Correlations of parameters with the children's age were assessed by using Pearson's correlation analyses. RESULTS: The LRD (mean, 14.1 ± 2.4 mm), APD (mean, 7.3 ± 1.0 mm) and LRD/APD ratio (mean, 1.9 ± 0.3) increased (r (LRD) = 0.70, p < 0.01; r (APD) = 0.56, p < 0.01; r (rat) = 0.28, p < 0.01) while the AA (mean, 27 ± 7°) decreased significantly (r (antang) = -0.26, p < 0.001) with age. The condylar shape as determined on sagittal images correlated significantly with age (r = 0.69, p < 0.05). Boys had significantly higher anteversion angles (p < 0.01), greater LRDs (p < 0.05) and greater mean ratios (p < 0.05). CONCLUSION: The mandibular condyle is subject to significant age-related changes in size and shape during childhood. As the size of the condyles increases with age, the anteversion angles decrease and the shape of the condyle turns from round to oval.

Radiographic and MRI features of deferiprone-related arthropathy of the knees in patients with beta-thalassemia.

OBJECTIVE: This study was undertaken to describe the radiographic and MRI appearances of arthropathy of the knees in 14 patients with beta-thalassemia major undergoing chelation therapy with deferiprone (L1). MATERIALS AND METHODS: All available radiographs and MRI studies of the knees in 14 beta-thalassemia major patients (mean age, 16.3 years; age range, 7-33 years) undergoing chelation therapy with L1 were retrospectively assessed for changes in the synovium, cartilage, and bone. Imaging findings and signs of knee arthropathy were correlated with chelation therapy and average serum ferritin concentration. RESULTS: Nine (64%) of the 14 patients developed arthralgia of the knees during treatment with L1. Abnormal imaging findings were present in all symptomatic and two asymptomatic patients (12/14, 86%) and included joint effusion, subchondral bone irregularity, and patellar beaks. Additional MRI findings were thickening and enhancement of the synovium; hypointense bands in the synovium; irregularly thickened epiphyseal and articular cartilage overlying subchondral bone defects; and, on T2-weighted sequences, hyperintense articular cartilage lesions. The degree of knee symptoms at the time of imaging did not reflect the severity of cartilage and subchondral bone changes. CONCLUSION: Radiologic changes can be seen in L1-related arthropathy and should be recognized. MRI of the knees should be considered in symptomatic children and young adults with thalassemia undergoing L1 chelation therapy for iron overload.

[Chronic arthroses in children: practical approach and epidemiological data]. / Arthrites chroniques de l'enfant: approche pratique et données épidémiologiques.

Osteo-articular symptoms are frequent in pediatrics, but chronic arthritis is rare in childhood. Arthritis may be difficult to recognize in children and there is a large differential diagnosis including infectious and neoplastic diseases. Even if juvenile arthritis has often a favourable course, significant functional damage may occur. The diagnosis and the follow-up of chronic arthritis should be performed in collaboration with a specialized consultation in pediatric rheumatology, in order to allow access to multidisciplinary medical care and help to increase the clinical and epidemiological knowledge in these rare diseases. A study is starting this fall aimed at collecting epidemiological datas on childhood arthritis in the french part of Switzerland.