bluuebunny/crossref_metadata_2025_split huggingface dataset:
A30: Assessment of Construct and Discriminative Validity of the 3‐Variable JADAS in Relation of Parent‐Reported Outcomes
Authors: Consolaro Alessandro , Negro Giorgia , Gallo Maria C. , Verazza Sara , Calandra Serena , Robbiano Cristina , Ruperto Nicolino , Martini Alberto , Ravelli Angelo 
Published: March 2014

Background/Purpose:An established approach to the measurement of disease activity in juvenile idiopathic arthritis (JIA) is based on the Juvenile Arthritis Disease Activity Score (JADAS). The feasibility of the JADAS for use in standard clinical practice might be enhanced by implementing of a 3‐variable version, which does not include the acute phase reactant. Recently, the 3‐variable JADAS was found to correlate closely with the original tool. However, the validity of this simplified version of the JADAS in the clinical setting is still insufficiently documented. Aim of the study was to further validate the 3‐variable version of the JADAS, hence named clinical JADAS (cJADAS), by assessing its relationship with parent‐reported outcomes (excluding the parent's rating of overall well‐being, which is included in the JADAS).Methods:The study was conducted in a cohort of 175 newly‐diagnosed consecutive JIA patients, first seen between 2007–2011 and then followed for 2 years. Clinical assessments were made at baseline and then at 6, 12, 18 and 24 months. At each visit, all parent reported outcomes included in the Juvenile Arthritis Multidimensional Assessment Report (JAMAR) were assessed by a parent of each patient. Construct validity of the cJADAS was computed by calculating its Spearman's correlation with pain visual analog scale (VAS) and disease activity VAS. Discriminative validity was evaluated, by means of the non‐parametric analysis of variance (Kruskal‐Wallis test) or Mann‐Whitney U test, by examining the ability of cJADAS to discriminate between: 1) different categories of disease state (remission, persistent activity or flare), rated subjectively by the parents; 2) different categories of disease course (improved, stable or worsened), rated subjectively by the parents; 3) visit in which parents declared themselves as satisfied or not satisfied with the outcome of the illness; 4) different duration of morning stiffness.Results:The cJADAS was strongly correlated with pain VAS (r=0.71) and disease activity VAS (r=0.77). The cJADAS was significantly lower in patients rated as being in remission than in patients rated as being in persistent activity or flare (p < 0.0001), in patients rated as improved or stable than in patients rated as worsened (p < 0001), and in patients whose parents were satisfied with illness outcome than in those who were not (p < 0.0001). The cJADAS was proportionally greater in parallel with the progressive increase in morning stiffness (p < 0001).Conclusion:The cJADAS was strongly associated with all parent‐reported outcomes, which indicates that it reflects well the parent perception of the child's disease status and course.
A35: Differential Benefit of Temporomandibular Joint Steroid Injections by Juvenile Idiopathic Arthritis Subtype
Authors: Groh Brandt , Tashima Alexis 
Published: March 2014

Background/Purpose:20–70% of juvenile idiopathic arthritis (JIA) patients have radiographic involvement of temporomandibular joints (TMJs), often reflective of cartilage loss and erosive bony changes. Intra‐articular steroid (IAS) injections have been shown by magnetic resonance (MR) imaging to reduce TMJ inflammation; however, it is less clear that this therapy provides longer term benefit in terms of preventing or retarding structural changes. Herein, we review the outcomes of our JIA patients treated with IAS for TMJ synovitis.Methods:Records of JIA patients treated with IAS for TMJ synovitis between January of 2003 and March of 2013 were reviewed. Determinations of symptomatic relief and duration of relief were made from both subjective and objective portions of notes generated at clinic visits subsequent to an initial IAS procedure. Determinations of radiographic progression were based on structural changes occurring between 1st and 2nd IAS procedures. The IAS used was triamcinolone hexacetonide in doses of 5–20 mg per joint.Results:63 patients received IAS at least once for TMJ synovitis, almost all for bilateral disease activity by MRI. Within this cohort, 55% of patients had received g1 injection per TMJ (mean of 2.75 for enthesitis‐related arthritis (ERA) patients vs. 1.95 for other combined JIA subtypes, p = 0.03). For 25% of patients, the TMJ was their first synovitic joint (58 % of ERA vs. 7.5% of other JIA subtypes, p < 0.001). ERA patients were older at at the time of TMJ diagnosis than the other JIA subtypes (mean age 14.0 vs. 11.5 years respectively, p = 0.003) and more likely to be symptomatic at the TMJ(s) than the other JIA subtypes (79% vs. 48% respectively, p = 0.018). Symptomatic ERA patients had a shorter mean duration of pain relief than the other symptomatic JIA subtypes (107 vs. 363 days respectively, p = 0.001). Second MR studies indicated progressive structural damage in all (32/32) patients re‐imaged at intervals of 6N36 months following their initial study. More acute TMJ symptoms and heightened concern for progression in the ERA patients resulted in a shorter mean inter‐injection interval of 10.9 vs. 19.9 months in the other JIA subtypes (p = 0.004).Conclusion:Of the JIA patients in clinic who have received IAS for TMJ synovitis, those with ERA benefitted the least in terms of pain relief duration and slowing of structural damage. In light of the higher frequency of IAS and the shorter duration of symptomatic relief in ERA patients, it is possible that TMJ synovitis is more aggressive in this subtype, or that a symptom‐driven increase in the frequency of IAS therapy has contributed to the rate and extent of structural damage. IAS therapy, in our experience, has not prevented the progression of TMJ damage in any JIA variant.
A38: Twelve Years' Experience with Etanercept in the Treatment of Juvenile Idiopathic Arthritis: How It Has Changed Practice—The German Biologics JIA Registry (BiKeR)
Authors: Schmeling Heinrike , Minden Kirsten , Foeldvari Ivan , Haas Johannes‐Peter , Hospach Anton , Horneff Gerd 
Published: March 2014

Background/Purpose:The outcome of children with JIA refractory to disease‐modifying antirheumatic drugs (DMARDs) has improved significantly since Etanercept became available. The objectives were to analyse data from the German Biologics JIA Registry (BiKeR) and to report on practice changes of the last twelve years.Methods:Baseline demographics, pre‐treatment, concomitant treatment, clinical characteristics and disease activity parameters and outcome have been prospectively documented in BiKeR since 2000. Efficacy was determined using the PedACR response criteria, the JADAS‐10 and the proposed criteria for inactive disease on medication.Results:From 2000 to 2012 1678 children with JIA on Etanercept with a total of 11,474 visits have been enrolled in BiKer (3424.2 total patient‐years). The median disease duration at treatment start decreased from 4.5 to 2 years. While initially children have been pre‐treated with numerous antirheumatic agents, including cytotoxic agents, pre–treatment markedly decreased by a mean 6.3 (median 4.3) to 0.99 (median 1.0) DMARDssol;patient. Concomitant treatment initially consisted of corticosteroids in 83%, Methotrexate in 95% and other DMARDs in 45% of children compared to 40%, 74% and 6.7% respectively in 2012. At Registry start, 26% of newly enrolled children belonged to the systemic JIA category compared to only 1% in 2012. In contrast, the rate of children with enthesitis related arthritis increased from 2% to 16%. The median JADAS‐10 in 2000 compared to 2012 was significantly higher at treatment start (24.4+/−6.8 vs. 13.3+/−6.2) and remained higher after 6 months on treatment (7.5+/−7.2 vs. 4.5+/−5.2). The number of patients that have reached a PedACR 70 after 12 months on treatment increased from 41% starting in 2000 to 51% of patients starting in 2012. Inactive disease within one year was documented initially in 20% of children and increased to 43% in 2012.Conclusion:In recent years, children with JIA have been treated earlier, received less pre‐ and concomitant treatment with corticosteroids as well as with DMARDs. Patients have shown a markedly better outcome reflected in the higher PedACR response, lower JADAS‐10 and higher rate of patients with inactive disease after one year on treatment. These data suggest that early disease control and better pre–selection of patients who need biologics are important to improve outcome in children with JIA.
A39: Efficacy and Safety of Methotrexate in Oligoarticular Persistent Juvenile Idiopathic Arthritis
Authors: Schmeling Heinrike , Foeldvari Ivan , Horneff Gerd 
Published: March 2014

Background/Purpose:Based on the ACR 2011 recommendations for the treatment of juvenile idiopathic arthritis (JIA) with persistent oligoarticular course methotrexate (MTX) is indicated for children with high disease activity and features of poor prognosis or if they failed intraarticular glucocorticoid steroid injections. However, no report has been published on MTX outcome in this category. Therefore our aim was to evaluate the efficacy and safety of MTX in children with oligoarticular persistent JIA.Methods:Baseline demographics, clinical characteristics and disease activity parameters have been prospectively documented in the German JIA Register. Efficacy was determined using the PedACR response criteria and the JADAS‐10. Safety assessments were based on adverse events reports from the responsible physician.Results:343 patients with a total of 2039 visits were identified (894.7 total patient‐years). 67% (n = 231) were females. The median age at JIA onset was 4.7 years (quartile (Q) 25/75 2.5/9.2). At treatment initiation the median age was 8.0 years (Q25/75 4.3/12.0) and the disease duration 1.1 years (Q25/75 0.5/3.1). ANA positivity has been found in 63.0%, HLB27 positivity in 11.1%. Prior to MTX therapy 90.1% of children have been treated with nonsteroidal anti‐inflammatory drugs (NSAIDs), 46.1% have received intraarticular glucocorticoid steroid injections. 88.3 of children were treated concomitantly with NSAIDs, 11.5% with corticosteroids and 2.6% with other disease‐modifying antirheumatic drugs.A high proportion of patients showed a significant response to treatment. Compared to baseline disease activity parameters at MTX initiation, at month 6 70.5%/61.0%/41.5%, at month 12 73.9%/63.9%/43.5% and at month 24 83.6%/80.0%/69.7% of patients met the PedACR response criteria of 30%/50%/70%. The median JADAS‐10 at treatment start was 10.0 and decreased markedly to 2.1 at month 6, 1.4 at month 12 and 1.0 at month 24. At month 6 and month 12, 83 patients (33%) and 96 patients (42%) reached preliminary criteria for inactive disease.113 children experienced 216 adverse events (AE) (24.7/100 patient–years). Four were reported as serious (0.4/100 patient–years (pj)). The most common reported AEs were nausea/vomiting (8.5/100 pj), abnormal liver enzymes (4.7/100 pj) and mild to moderate infections (3.6/100 pj). 48 children (14%) suffered from uveitis prior treatment. None of these patients flared with uveitis. Four new uveitis index cases (1.1%) were reported while on treatment.Treatment has been discontinued in 134 children for the following reasons: Inefficacy 3.2%, adverse events 9.8%, remission 24.3%, patients wish 16.5%, others 2.9%. 17 of 84 children (20.2%) who stopped MTX because of remission flared and re‐started MTX.Conclusion:MTX appears to be highly effective in oligoarticular persistent JIA. The treatment is safe and well‐tolerated. Few patients discontinued due to intolerance or inefficacy.
A53: Natural History of Pediatric Class V Membranous Lupus Nephritis—A Serial Biopsy Study
Authors: Canter Debra , Eldin Karen , Hicks M. John , Muscal Eyal , DeGuzman Marietta , Wenderfer Scott E. 
Published: March 2014

Background/Purpose:In 1998, Sorof et al. reported 28% of 60 pediatric SLE patients had class V lesions on their first biopsy and 37% had class V lesions on their most recent biopsy. Although 19% of patients with LN transformed from other classes to class V, there were no patients with repeat biopsies who showed complete resolution of their class V lesions. The prognosis of the patients with class V LN was better than those with non‐class V LN (only 5% vs. 21% developed renal dysfunction or died).Methods:We retrospectively reviewed pathology records of pediatric lupus nephritis patients who underwent renal biopsies at Texas Children's Hospital between 1990 and 2013.Results:A total of 321 biopsies were performed on 174 patients with SLE. The cumulative incidence of class V histopathology was 50% (160/321). Upon initial biopsy, 93/174 (53%) patients had Class II, III or IV LN without class V lesions, and 81/174 (47%) patients had class V lesions, either alone (n = 38), or associated with class II, III or IV LN (n = 43). Repeat biopsies were obtained in 97 cases. The median interval between first and second biopsies was 11 months (IQR 9–19m, range 3–110 m). Of the 97 patients with at least 2 renal biopsies, 36 (37%) had class V lesions on the initial biopsy, and 61 (63%) did not have class V lesions on the initial biopsy. Although transformation between classes was common, the initially non‐class V LN patients frequently remained free of class V lesions at follow up (n = 42, 70%). Of the 18/61 initially non‐class V LN patients who did later develop class V lesions, 6% initially had pure class II LN, 61% pure class III, and 33% pure class IV. Repeat biopsies revealed mixed class V LN in 16/18 patients, with subsequent resolution of class V lesions or transformation to pure class V in 4.Six (17%) of the 36 patients with class V lesions on the initial biopsy had pure class V lesions, and 30 (83%) had mixed class II, III or IV LNThree (50%) of the 6 patients with initially pure class V lesions showed evolution to mixed class III/V LN, and evolution to class IV with sustained absence of class V lesions in 1 (17%). Of the 30 patients with initially mixed class V LN, repeat biopsies revealed persistent mixed class V LN in 22 (73%), transformation to pure class V in 4 (13%) and sustained resolution of class V lesions in 4 (13%).Conclusion:A comparison of the WHO classification of initial and repeat biopsies showed evolution to pure class V LN, or more commonly, development of a mixed class including class V LN. Although the vast majority of patients with class V LN showed persistence of this histopathology, sustained resolution of class V lesions may be possible.
A59: Dexamethazone Therapy for Septic Arthritis in Children: a Follow Up Study
Authors: Fogel Itay , Amir Jacob , Bar‐On Elhanan , Harel Liora 
Published: March 2014

Background/Purpose:In our previous prospective study we have demonstrated that the administration of Dexamethasone as an adjuvant therapy lead to a significant clinical and laboratory improvement in children with septic arthritis. This therapy has become accepted in some of the pediatric wards at Schneider's medical center. The aim of this study was to evaluate the effect of adding Dexamethasone to antibiotic therapy on the clinical course of septic arthritis in children not under controlled study.Methods:A retrospective study included all children hospitalized with the diagnosis of septic arthritis at Schneider Children's Medical Center from 2008–2013 .Part of the patients were treated with antibiotics alone, whereas others were treated with Dexametasone in addition to antibiotics, according to the policy of the admitting ward.Results:116 medical records were included in our study. Of which, 26 (22.4%) patients were treated with Dexamethasone and antibiotics and 90(77.6%) were treated with antibiotics only. There was no significant difference between the groups in age, duration of symptoms prior to hospitalization, body temperature or levels of acute phase reactants. Bacteria were isolated from 23 patients (20%) without any difference between the two groups. Patients treated with Dexamethasone had a significantly shorter duration of fever (mean 1st day without fever 2.3 vs. 3.9) (p = 0.002), shorter duration of parenteral antibiotic treatment (mean of 7.1 vs. 11.4 days) (p < 0.001) and accelerated clinical improvement (mean 1stday without pain or any limitation in physical examination 6.3 vs. 10 days)(p<0.001).Likewise, C‐reactive protein (CRP) levels declined faster below 1mg/dL in the Dexamethasone group (mean of 5.3 vs. 8.4 days) (p = 0.002) and the hospital stay was significantly shorter (mean of 8 vs. 10.7 days) (p = 0.004). No side effects of treatment were recorded in either group, but in the Dexamethasone group a short rebound of symptoms occurred in 3/26 (11.5%) patients after completing the course of steroids.Conclusion:A short course of Dexamethasone given early, in addition to antibiotics, leads to a significant clinical and laboratory improvement, shortens duration of treatment and accelerates recovery in children with septic arthritis.
A60: Optic Nerve and Retinal Features in Uveitis Associated With Juvenile Systemic Granulomatous Disease (Blau Syndrome)
Authors: Carreno Ester , Guly Catherine M , Chilov Michael , Hinchcliffe Annie , Aróstegui Juan Ignacio , Lee Richard W , Dick Adrew D , Ramanan Athimalaipet V 
Published: March 2014

Background/Purpose:Juvenile systemic granulomatous disease (JSGD), also known as Blau syndrome, is a dominantly‐inherited autoinflammatory disorder associated with gain‐of‐function mutations in the NOD2 gene. The aim of this study was to determine whether patients with JSGD and uveitis have a specific ocular phenotype.Methods:Clinical and imaging data were collected retrospectively from patients attending the Regional Combined Paediatric Rheumatology and Ocular Inflammatory Service, Bristol Eye Hospital. General demographic information, laterality of the uveitis, age at onset, anatomical classification and course of the uveitis, clinical phenotype, and specific NOD2 mutation were recorded for each patient. All data were recorded in a database designed in Microsoft® Access®. Statistical analysis was performed using SPSS 20.0 (Cary, NC).Results:Seventeen eyes from 9 patients (5 males; 4 females) were included in the study. Mean age at the disease onset was 15 months; range 1–84 months. Eight patients had bilateral uveitis. Anterior uveitis was present in five eyes, intermediate uveitis in 2 eyes and there were 10 eyes with panuveitis, manifesting as multifocal choroiditis. Appearance of optic disc included indistinct disc margins in 6 eyes, optic nerve head palor in 6 eyes and optic disc vessels sheathing in 4 eyes. Fundal appearance included peripapillary hypo/hyperpigmention in 13 eyes accompanied with characteristic peripapillary nodular excrescences. Among NOD2 mutations, the p.R334W was the most commonly detected (n: 4 cases) and three patients carried novel variants, the p.E338D and p.D390V variants in one patient, and the p.H520Y and p.Q809K variants in two different patients.Conclusion:Chronic bilateral panuveitis and a nodular peripapillary appearance in childhood onset uveitis are characteristic features of JSGD, which support the need for an appropriate genetic NOD2 analysis.
A61: Prevalence of Streptococcal Antibodies in Pediatric Non‐Rheumatic Fever Syndromes in Areas of Low Rheumatic Fever Incidence
Authors: Price Judith , Patwardhan Anjali 
Published: March 2014

Background/Purpose:Streptococcus pyogens infect an estimated 5 million children in United States/year. Various syndromes are attributed to elevated Streptococcal antibody titer (SAT) & are treated with prophylactic penicillin therapy (PPT). We hypothesize the elevated SAT has no diagnostic & PPT has no therapeutic significance in pediatric non‐Rheumatic Fever Syndromes in areas of Low Rheumatic Fever Incidence.Methods:A cross‐sectional retrospective electronic chart review was conducted. Patients who met the criteria had tics, chronic pain syndrome (CPS) or joint symptoms as presenting complaints, had streptococcal antibody titers (SAT) performed & were seen by pediatricians in past 5 years at Women's and Children's Hospital. Patients were classified into 3 subgroups. Group 1: 184 presented with joint pain with or without (w/o) swelling, Group 2: 105 presented with tics & Group 3: 324 presented with CPS. Reference lab values were: Antistreptolysin O (ASO);0–1 yr:0–200 IU/mL, 2–12 yrs: 0–240 IU/mL, 13 yrs & older: 0–330 IU/mL & DNAse‐B Ab; 0–6 yrs: Less than 250 U/mL, 7–17yrs:Less than 310 U/mL, 18 yrs & older: Less than 260 U/mL. Elevation of titers by ≥1.5 times for age was considered abnormal for research purposes.Results:Six hundred and thirteen charts were reviewed. Chi‐square test was used to identify proportion of abnormal ASO & DNAse‐B Ab among the 3 diagnostic groups ranged from 26% (JS) to 35% (TICS), and 25.5% to 26.7% respectively; the difference in the abnormal ASO & DNAse‐B Ab in the 3 groups was not significant (ASO p = 0.25, DNAse‐B Ab p = 0.97). An exact Pearson chi‐square test compared the proportion of abnormal ASO & DNAse‐B Ab on patients w/o PPT. The proportion of abnormal results w/o PPT were 5.88% (8/136) & 10.87% (5/46) for ASO & 5.88% (8/136) & 4.35% (2/46) for DNAse‐B Ab, which was not statistically significant (p = 0.74 for ASO, p = 0.32 for DNAse‐B Ab). McNemar's test revealed that the proportion of abnormal ASO results (178/613 = 29.04%) was significantly higher (P‐value = 0.0002) than of DNAse‐B Ab (158/613 = 25.77%). For both ASO & DNAse‐B Ab, odds of abnormal results in males were about 1.73 times higher than in females (CI = 95%, OD = 1.19, 2.51); the highest probability of abnormal results occurred around 129 months. Mean Ab test costs for CPS was $426, JS was $377 & Tics was $556. While 137 patients received PPT, 47 received no therapy; there was no difference in abnormal SAT in both groups at 6 months.Conclusion:In low Rheumatic Fever Incidence areas, abnormal SAT in Pediatric non‐rheumatic fever syndromes have poor diagnostic & therapeutic value; repeat testing adds cost without treatment value.