Methods Out of 35 patients with JIA, enrolled after parental consent, 31 (23 males and 8 females) completed the study. Mean age of the patients was 9.8 years (Range 6.114.5 years). The patients were enrolled when they demonstrated active disease at presentation or during relapse, as ‘active disease group’ and followed up regularly at 6-8 weekly intervals, till they improved with treatment. Disease activity of JIA was assessed by ACR PEDI criteria. Sleep was studied using SDSC which is a validated screening tool (for the age interval studied), that categorizes sleep disturbances into six different categories: Disorders of initiating and maintaining sleep (DIMS), Sleep disordered breathing (SDB), Disorders of arousal (DA), Sleep wake transition disorders (SWTD), Disorders of excessive somnolence (DOES) & Sleep hyperhydrosis (SHY). The SDSC questionnaire was filled by the parent / patient in the waiting room at each follow up visit. When the patients showed maximum improvement in JIA, they were included in the ‘post treatment’ group. For quantifying sleep disorders, we compared the scores of the subjects with scores derived from a sample of normal population in a similar age band from our city. Differences between the active and post treatment groups were analyzed by non parametric Wilcoxson test for paired data and non parametric Mann-Whitney U test for unpaired data
Methods 369 parents completed a multidimensional questionnaire (the JAMAR), including Juvenile Arthritis Functionality Scale (JAFS) (score 0–30), Pediatric Rheumatology Quality of Life (PRQL) questionnaire (score 0–30) and traditional JIA outcome measures, and stated whether they considered their children's status satisfactory or not. PASS thresholds were estimated based on parent opinion and targeting the 75th percentile of cumulative distribution. Stepwise logistic regression was used to assess contributors to PASS. PASS was validated by analyzing proportions of patients who were judged by parent or physician in remission, flare or continued activity.
To determine in a prospective analysis whether baseline demographic, clinical, and laboratory variables predict the outcome of intraarticular corticosteroid (IAC) injection of the knees in children with juvenile idiopathic arthritis (JIA).We studied consecutive patients who met the criteria for the diagnosis of JIA and received their initial injection of triamcinolone hexacetonide in one or both knees. Predictor variables included sex, age, age at onset of JIA, onset subtype, disease duration, drug therapy at the time of IAC injection, physician and parent global assessment of disease status, Childhood Health Assessment Questionnaire disability index, erythrocyte sedimentation rate (ESR), C-reactive protein, involvement of other joints besides knees, amount of fluid aspirated, and dose of IAC injected. The primary outcome measure was persistence of complete clinical response at 6 months, i.e., no evidence of synovitis clinically.Ninety-four patients were available for analysis. At 6 months after the IAC injection, 65 (69%) patients showed a sustained complete clinical response, whereas 29 (31%) had had a recurrence of joint inflammation. Univariate statistical analyses showed that patients who had a sustained clinical response had a significantly higher ESR than those who did not (p = 0.023). The ESR was the only variable that remained in the best-fit model from multivariate logistic regression analysis (OR 2.61, p = 0.049).Our findings indicate that patients with JIA who have a higher ESR are more likely to benefit from IAC injection of the knees.
The prevalence of asthma and obesity, two often associated conditions, is influenced not only by age and gender but also by lifestyle factors. This study aimed to determine whether, in a Mediterranean northern Italian region, Liguria, an increased prevalence of obesity could be detected in asthmatic children and adolescents and to evaluate the possible relationship between body mass index (BMI) and the characteristics and/or severity of asthma. BMI was determined in 554 asthmatic subjects (2.2–16.1 years) and 625 age-matched controls; BMI was expressed as a continuous variable in standard deviation score (SDS) units, determined as difference between the individual observed value and the reference mean for age and sex, divided by the corresponding standard deviation (BMI-SDS). Overweight/obesity was set at BMI-SDS of 2 or more. BMI-SDS was significantly higher in controls than in asthmatics (p = 0.04); however, the proportion of overweight/obesity subjects (BMI-SDS ≥ 2) was similar in controls and in asthmatic patients (p = 0.08). Evaluation of the asthmatic group revealed that BMI-SDS was independent of gender (p = 0.57), atopic sensitization (p = 0.69), and comorbidity with other allergic symptoms (p = 0.60). By contrast, BMI-SDS was lower in preschool-age children than in school-age children and adolescents (p < 0.0001), in subjects with a high rate of acute respiratory tract infections (p = 0.04), and in those not treated with inhaled corticosteroids (IGCs) (p = 0.02). Although an increase in the prevalence of overweight/obesity was not detected in asthmatic children and adolescents, the results reported here suggest a preventive surveillance of calorie intake and a promotion of physical activity in children requiring long-term treatment with inhaled glucocorticosteroids.
Over the past two decades there has been a remarkable advance in the management of juvenile idiopathic arthritis (JIA), which has led to considerable improvement in prognosis. In 2018, the introduction of the treat-to-target (T2T) strategy in JIA has been advocated to further ameliorate disease outcome. To provide a benchmark for comparing future outcomes in the "T2T era", this study investigates the percentage of JIA patients who achieved clinical inactive disease (CID) in the decade that preceded the publication of the T2T recommendations in JIA. The clinical charts of all JIA patients followed at the study center between 2007 and 2017 who were first seen within 6 months after disease onset and had a minimum of 6-month follow-up information available were reviewed retrospectively. The attainment of CID, defined by 2004 Wallace criteria, was assessed cross-sectionally at 6, 12, 24, and 60 months after first observation. A total of 394 patients were included. Patients were classified into four "functional phenotypes": systemic arthritis (7.1%), oligoarthritis (48.2%), polyarthritis (40.4%), and other arthritis (4.3%). The overall frequency of CID was 25.1% at 6 months, 34.5% at 12 months, 44.6% at 24 months, and 49.1% at 60 months. The systemic and oligoarticular subgroups had the highest rates of CID at 6 months (32.1% and 29.5%, respectively) and at 12 months (40% and 41.1%, respectively). At the 60-month evaluation, which was available for 226 out of 394 patients (57.4%), the frequency of CID among patients still followed at study center was 42.9%, 51.7%, 46.7%, and 45.5% for the systemic, oligoarticular, polyarticular, and other arthritis phenotypes, respectively. A sizeable proportion of patients treated in the decade preceding the beginning of the "T2T era" and on continued follow-up did not achieve or maintain the state of CID over the long term. Future studies will determine whether the application of the T2T strategy increases the ability to achieve sustained disease quiescence in patients who respond suboptimally to the conventional therapeutic regimens.
To the Editor,Asthma control is currently the cornerstone strategy in the management of asthmatics (1). Actually, uncontrolled and severe asthma significantly affect patients and society (2). Uncon...
Abstract Objective To develop adapted versions of the Sharp/van der Heijde radiographic scoring system for use in juvenile idiopathic arthritis (JIA), and to investigate their validity in JIA patients with polyarticular disease. Methods The study group comprised 177 patients with polyarticular JIA. Radiographs of the wrist/hand of each patient were obtained at baseline (first observation) and then at 1, 3, 5, 7/8, and 10 years and were assessed independently by 2 pediatric rheumatologists according to different adaptations of the Sharp/van der Heijde method. To facilitate score assignment, the radiograph for each patient was compared with a bone age–related standard. Validation procedures included analysis of reliability, construct validity, and score progression over time. Results Interobserver and intraobserver agreement on longitudinal score values and score changes was good for all of the adapted scoring versions (intraclass correlation coefficient >0.85). Score changes over time were moderately to strongly correlated with the clinical indicators of long‐term joint damage and with the amount of long‐term radiographic damage as measured with the carpo:metacarpal ratio, thereby demonstrating good construct validity. A steady increase in scores over time was observed, with joint space narrowing being the most common form of damage throughout the disease course. The inclusion of 5 new areas appeared to increase the overall construct validity of erosion scores. Conclusion Our results show that the adapted versions of the Sharp/van der Heijde score are reliable and valid for the assessment of radiographic progression in patients with JIA.
BackgroundOver the years the need for surgical treatment, timing of intervention, and the type of surgical approach have been discussed, but the treatment of congenital lung malformations remains controversial. The aim of this study was to compare the thoracotomy approach with the thoracoscopic technique by evaluating different surgical outcomes (duration of surgery, postoperative hospital stay, and complications).MethodsAll patients operated from January 2011 to March 2015 for suspected congenital cystic lung were included in the study. Patients treated for congenital lobar emphysema and tracheobronchial neoplasms were excluded from the study.ResultsIn the analyzed period, 31 asymptomatic patients were treated: 18 lung resections were performed with thoracotomy (Group A) and 13 with the thoracoscopic approach (Group B). No significant differences were observed between the age and weight at surgery, length of the procedures, complications, and the need for postoperative intensive care between the two groups. The postoperative hospital stay in Group A was twice that for Group 2 (p = 0.0009).ConclusionComparing thoracoscopic surgery with the traditional open approach, we confirmed the superiority of minimally invasive treatment in terms of postoperative hospital stay. Common technical recommendations can help pediatric centers to develop the thoracoscopic approach for the treatment of congenital pulmonary malformations.
To evaluate the performance of the Birmingham Vasculitis Activity Score (BVAS) v3 and the Disease Extent Index (DEI) for the assessment of disease activity in 4 primary childhood (c-) systemic vasculitides.Patients fulfilling the EULAR/PRINTO/PRES (Ankara) c-vasculitis classification criteria for Henoch-Schönlein purpura (HSP), childhood (c) polyarteritis nodosa (c-PAN), c-Wegener's granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) with disease duration at the time of diagnosis ≤3 months were extracted from the PRINTO database. The performance of the BVAS and DEI were examined by assessing convergent validity, the pattern of disease involvement, and responsiveness. We also evaluated alternative unweighted scoring methods for both tools.The analysis set included 796 patients with 669 HSP, 80 c-PAN, 25 c-WG and 22 c-TA. The median age at diagnosis was 6.9 years (6.6-12) and median delay in making the diagnosis from the onset of signs/symptoms was 0.01 (0.003-0.027) years. A strong correlation was found between the BVAS and DEI (rs=0.78) while correlation with the physician global assessment was moderate (rs=0.48) with BVAS and poor with DEI (rs=0.25). Both the BVAS and DEI sub-scores and total scores were able to descrive the disease involvement in the 4 childhood vasculitides. Responsiveness was large (>1.5) for both tools. The performance characteristics of the BVAS and DEI with the unweighted methods were comparable.This study demonstrates that both the BVAS and DEI are valid tools for the assessment of the level of disease activity in a large cohort of childhood acute and chronic vasculitides.
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