Radiographic markers of hip dysplasia in young adults: predictive effect of factors in early life

Background and objectives Acetabular dysplasia in young adults occurs, despite screening for developmental hip dysplasia (DDH) in the neonatal period. We aimed to examine how early life factors predict radiographic measurements of acetabular dysplasia at 18–19 years of age. Methods From a previous randomized trial (n=12,014; 1988–90) evaluating the role of hip ultrasound in newborn screening of DDH, 4469 participants (2193 males) were invited to a follow-up 18 years later (2007–09), of which 2370 (53% attendance; 932 males) met. We examined associations between early life factors and four radiographic measurements for acetabular dysplasia at skeletal maturity. Hierarchical regressions, with addition of variables observed/ measured consecutively in time, were analyzed using mixed efects models considering hip as the unit in the analyses. The study is approved by the Regional Ethics Committee. Results In total, 2340 participants (921 boys), mean age 18.7 years, (SD 0.6) had hip radiographs performed at followup and were included. Early life factors signifcantly predicting radiographic acetabular dysplasia at age 18–19-years included female gender, breech, low acetabular inclination (alpha) angle and sonographic instability, abduction treatment, as well as the velocity of growth during childhood. A positive family history of DDH was not associated with acetabular dysplasia at skeletal maturity. Conclusion The acetabular inclination (alpha) angle as measured on ultrasound at birth turned out to be a signifcant predictor of dysplasia at 18–19 years of age. The discordant role of a positive family history in early versus adult hip dysplasia is intriguing, warranting further studies on the genetic mechanisms of DDH

Erosion or normal variant? 4-year MRI follow-up of the wrists in healthy children

Background A large proportion of healthy children have wrist changes on MRI, namely carpal depressions, findings that have been described as pathological in children with juvenile idiopathic arthritis. Objective We performed follow-up imaging in a cohort of healthy children to evaluate carpal surface depressions over time, focusing on the presence of overlying cartilage as a potential discriminator between normal variants and true erosions. Materials and methods 74 of the initial cohort of 89 healthy children (83%) had a re-scan of their wrists using the same protocol, including coronal T1 and fat-saturated T2 sequences. A cartilage-selective sequence was added for this study. We registered number and location of bony depressions and presence of overlying cartilage. Results The total number of carpal depressions increased by age group and over time; their location was unchanged in 370 of 487 (76%) carpal sites and 91 of 117 (78%) metacarpal sites. In total, 426 of the 1,087 (39.2%) bony depressions were covered by cartilage, with a decreasing percentage by age (P = 0.001). Conclusion Normal appearances during growth, such as bony depressions, should not be mistaken for pathology. There must be additional findings to support a diagnosis of disease. A cartilage sequence may add to the diagnostic image analysis.publishedVersio

Familial Takayasu arteritis - a pediatric case and a review of the literature

Takayasu arteritis (TA) is a rare chronic inflammatory disease of the aorta and its major branches. It is seen predominantly in females during the second and third decades of life, although it can occur in childhood. The aetiology of TA remains unknown. To date, familial cases of TA have been considered rare; however, a review of the literature suggests that cases are accumulating. We report a case of two sisters affected by severe TA, and review other reported familial cases

Anti-neutrophil cytoplasmatic antibodies and lung disease in cystic fibrosis

AbstractBackground: Bactericidal-permeability-increasing protein (BPI) is a potent anti-microbial protein produced by neutrophil granulocytes. Anti-neutrophil cytoplasmatic antibodies (ANCA) directed against BPI have been detected in up to 91% in patients with cystic fibrosis (CF). We aimed to evaluate the prevalence of BPI-ANCA in our CF patients and to determine whether presence of BPI-ANCA is correlated with organ damage. Methods: Twenty-four patients performed respiratory function testing and pulmonary high-resolution computed tomography (HRCT). HRCT was scored by using a modified Bhalla method. Serum samples were analysed by direct binding enzyme-linked immunosorbent assay for BPI-ANCA. Results: The prevalence of anti-BPI-IgG was 71% and anti-BPI-IgA 33%. Twenty-nine percent of our patients were positive for both BPI-ANCA isotypes. Mean HRCT score was 8.0 ranging from 0 to 22, bronchiectasis presented the most common finding (79%). There was a significant correlation between BPI-ANCA and both HRCT score and FEV1 (p<0.01). High levels of BPI-ANCA were correlated to chronic Pseudomonas aeruginosa lung infection (p<0.01). Conclusions: BPI-ANCA was common in our study group. Highly significant correlations between BPI-ANCA and parameters to evaluate lung disease in CF may be a consequence of the inflammation process, or it may indicate a pathogenic role of BPI-ANCA levels in the development of lung disease. More research is needed and the clinical significance of our findings needs further evaluation

European Society of Paediatric Radiology 2019 strategic research agenda: improving imaging for tomorrow’s children

The European Society of Paediatric Radiology (ESPR) research committee was established to initiate, drive forward and foster excellence in paediatric imaging, paediatric image-guided intervention and radiation protection research, by facilitating more evidence-based standards, protocols and multi-institutional collaborations. The ESPR Strategic Research Agenda outlines our current research approach, highlighting several areas of paediatric imaging where the society can help guide current and future research, and emphasizing those areas where early research (“seed”) funding may need to be allocated by this and other societies as precursors to larger grant applications. The key aims are to evaluate normal variation in order to be able to confidently diagnose disease states, develop robust image-based classification systems to aid diagnosis and treatment monitoring, and help develop evidence-based clinical guidelines using current literature and experience to identify knowledge gaps. For this reason, the development of evidence-based imaging pipelines, broken down step-by-step to include diagnosis, classification and clinical effectiveness, should be the end goal for each disease entity for each affected child. Here, we outline the 2019 ESPR Strategic Research Agenda along three points in the clinical imaging pipeline: clinical referral, disease diagnosis and evolution, and clinical therapeutic evaluation and effectiveness. Through multicentre trials, using existing high-level experience and expertise, and nurturing the next generation of researchers, we will be able to achieve these aims.publishedVersio

Whole-body magnetic resonance imaging in children – how and why? A systematic review

Whole-body magnetic resonance imaging (MRI) is increasingly being used for a number of indications. Our aim was to review and describe indications and scan protocols for diagnostic value of whole-body MRI for multifocal disease in children and adolescents, we conducted a systematic search in Medline, Embase and Cochrane for all published papers until November 2018. Relevant subject headings and free text words were used for the following concepts: 1) whole-body, 2) magnetic resonance imaging and 3) child and/or adolescent. Included were papers in English with a relevant study design that reported on the use and/or findings from whole-body MRI examinations in children and adolescents. This review includes 54 of 1,609 papers identified from literature searches. Chronic nonbacterial osteomyelitis, lymphoma and metastasis were the most frequent indications for performing a whole-body MRI. The typical protocol included a coronal STIR (short tau inversion recovery) sequence with or without a coronal T1-weighted sequence. Numerous studies lacked sufficient data for calculating images resolution and only a few studies reported the acquired voxel volume, making it impossible for others to reproduce the protocol/images. Only a minority of the included papers assessed reliability tests and none of the studies documented whether the use of whole-body MRI affected mortality and/or morbidity. Our systematic review confirms significant variability of technique and the lack of proven validity of MRI findings. The information could potentially be used to boost attempts towards standardization of technique, reporting and guidelines development

Cone beam computed tomography in the assessment of TMJ deformity in children with JIA: repeatability of a novel scoring system

Background The temporomandibular joint (TMJ) is frequently involved in juvenile idiopathic arthritis (JIA). Diagnostic imaging is necessary to correctly diagnose and evaluate TMJ involvement, however, hitherto little has been published on the accuracy of the applied scoring systems and measurements. The present study aims to investigate the precision of 20 imaging features and five measurements based on cone beam computed tomography (CBCT). Methods Imaging and clinical data from 84 participants in the Norwegian study on juvenile idiopathic arthritis, the NorJIA study, were collected. Altogether 20 imaging features and five measurements were evaluated independently by three experienced radiologists for intra- and interobserver agreement. Agreement of categorical variables was assessed by Fleiss’, Cohen’s simple or weighted Kappa as appropriate. Agreement of continuous variables was assessed with 95% limits of agreement as advised by Bland and Altman. Results “Overall impression of TMJ deformity” showed almost perfect intraobserver agreement with a kappa coefficient of 0.81 (95% CI 0.69–0.92), and substantial interobserver agreement (Fleiss’ kappa 0.70 (0.61–0.78)). Moreover, both “flattening” and “irregularities” of the eminence/fossa and condyle performed well, with intra- and interobserver agreements of 0.66–0.82 and 0.55–0.76, respectively. “Reduced condylar volume” and “continuity” of the fossa/eminence had moderate intra- and interobserver Kappa values, whereas continuity of the condyle had Kappa values above 0.55. Measurements of distances and angles had limits of agreement of more than 15% of the sample mean. Conclusions We propose a CBCT-based scoring system of nine precise imaging features suggestive of TMJ deformity in JIA. Their clinical validity must be tested.publishedVersio

Cone beam computed tomography in the assessment of TMJ deformity in children with JIA: repeatability of a novel scoring system

Background The temporomandibular joint (TMJ) is frequently involved in juvenile idiopathic arthritis (JIA). Diag‑ nostic imaging is necessary to correctly diagnose and evaluate TMJ involvement, however, hitherto little has been published on the accuracy of the applied scoring systems and measurements. The present study aims to investigate the precision of 20 imaging features and fve measurements based on cone beam computed tomography (CBCT). Methods Imaging and clinical data from 84 participants in the Norwegian study on juvenile idiopathic arthritis, the NorJIA study, were collected. Altogether 20 imaging features and fve measurements were evaluated indepen‑ dently by three experienced radiologists for intra- and interobserver agreement. Agreement of categorical variables was assessed by Fleiss’, Cohen’s simple or weighted Kappa as appropriate. Agreement of continuous variables was assessed with 95% limits of agreement as advised by Bland and Altman. Results “Overall impression of TMJ deformity” showed almost perfect intraobserver agreement with a kappa coef‑ fcient of 0.81 (95% CI 0.69–0.92), and substantial interobserver agreement (Fleiss’ kappa 0.70 (0.61–0.78)). Moreover, both “fattening” and “irregularities” of the eminence/fossa and condyle performed well, with intra- and interobserver agreements of 0.66–0.82 and 0.55–0.76, respectively. “Reduced condylar volume” and “continuity” of the fossa/emi‑ nence had moderate intra- and interobserver Kappa values, whereas continuity of the condyle had Kappa values above 0.55. Measurements of distances and angles had limits of agreement of more than 15% of the sample mean. Conclusions We propose a CBCT-based scoring system of nine precise imaging features suggestive of TMJ deformity in JIA. Their clinical validity must be tested