bluuebunny/crossref_metadata_2025 huggingface dataset:
Activity budget, diet, and habitat use in the critically endangered Ka'apor capuchin monkey (Cebus kaapori) in Pará State, Brazil: A preliminary comparison to other capuchin monkeys
Authors: de Oliveira S. G. , Lynch Alfaro Jessica W. , Veiga Liza M.
Published: March 2014

The Ka'apor capuchin,Cebus kaapori, is perhaps the most endangered primate of the Brazilian Amazon. Endemic to a region with extreme intensification of habitat‐degrading activities, it survives in remnant populations in a completely fragmented landscape. Before now, the only data available were isolated observations of feeding, locality records, and information on population densities and group size obtained during census. Here we present the first data on the activity budget, diet, and daily path length of the species, and compare our preliminary results with those for other capuchin monkeys. A group of nine Ka'apor capuchins was monitored over a period of four months during the dry season in the Goianésia do Pará municipality, Pará, Brazil. We used instantaneous scan sampling (n = 4,647 scans) to construct an activity budget for the monkeys, and we identified the plants in their diet to species level (n = 41 plant taxa), allowing us to compare dietary overlap with other gracile capuchin species, as well as with the robust capuchin (Sapajusspp.), a potential competitor present throughout the range of the Ka'apor capuchin. Like other species of gracile capuchins,C. kaaporiwas highly frugivorous, with the vast majority of the feeding records of arils and fruit pulp (74%), supplemented by arthropods (13%) and seeds (10%), although diet composition was highly variable across months. The group used a total area of 62.4 ha during the study period, and average daily path length was 2,173 m (±400 m), with the entire home range utilized in every month of the study. We found significant overlap in the diet of the Ka'apor capuchin andSapajus, highlighting the urgency to increase knowledge of the ecological needs ofC. kaaporiand understand synergistic effects of sympatry with competitive species, increasing forest fragmentation, and widespread human impact onC. kaaporisustainability. Am. J. Primatol. 76:919–931, 2014. © 2014 Wiley Periodicals, Inc.
Chemical vapor deposition of poly(3‐alkylthiophene) nanoparticles on fabric: Chemical and electrochemical characterization
Authors: Jahan Biglari Maryam , Mokhtari Javad , Nouri Mahdi , Sarabi Ali Asghar
Published: March 2014

Chemical vapor deposition of poly(3‐methylthiophene) and poly (3‐hexylthiophene) as conductive polymers on the surface of polyester fabrics was successfully obtained. Fourier transform infrared spectroscopy confirmed the formation of polymers on surface of fabrics (the fingerprint of polythiophenes, υ 600–1500 cm−1). The uniformity of deposition and nanoparticles (average size of 60 nm) were proved with scanning electron microscopy. Electrochemical impedance spectroscopy showed that P3HT‐coated samples offer higher conductivity in compared to P3MT‐coated samples. The impedance modulus of P3HT‐coated samples was lowered nine times to that of row materials and reached to c8000 Ω. The samples have also shown electrochromic properties under electrical current, changing its color from yellowish green at 0 V to dark green at +12 V for poly (3‐hexylthiophene) samples and from brown at 0 V to red at +12 V for poly(3‐methylthiophene)‐coated fabrics (V = 0 V, λ = 450 nm; V = 12 V, λ = 650 nm). © 2014 Wiley Periodicals, Inc. J. Appl. Polym. Sci. 2014, 131, 40673.
A18: Risk Factors for Symptomatic Avascular Necrosis in Childhood‐Onset Systemic Lupus Erythematous
Authors: Yang Yelin , Kumar Sathish , Silverman Earl D. , Levy Deborah M.
Published: March 2014

Background/Purpose:

Childhood‐onset systemic lupus erythematous (cSLE) has a more severe clinical course with higher disease activity and involvement of major organs compared to adult‐onset SLE. Avascular necrosis (AVN) is a significant morbidity causing pain and disability, sometimes requiring joint replacement surgery. Our objective was to examine the frequency and risk factors for symptomatic AVN in cSLE.

Methods:

A single center matched case‐control design was used. 617 patients with cSLE followed at SickKids Lupus Clinic between July 1982 and June 2013 were included in the study. The AVN cohort consisted of 37 patients identified with clinical findings of symptomatic AVN and diagnosis confirmed by one or more imaging modalities. Three controls were matched to each AVN patient by date and age at diagnosis (± 1 year). Baseline clinical, laboratory and treatment characteristics were compared between the patients with AVN and the controls by univariate analyses and if statistically significant, were included in a multivariable logistic regression model.

Results:

A total of 37/617 (6%) patients developed symptomatic AVN during follow‐up at SickKids. The majority was female (30/37, 81%) and of Asian descent (20/37, 54%). The mean age at diagnosis was 16.1 years (±2.1), with median time to AVN of 1.52 years. Only 2/37 (5%) of patients developed AVN prior to the onset of puberty. The hip was the most commonly involved joint (26/37, 70%) with bilateral involvement in 49% (18/37) of patients. Compared to the matched non‐AVN cohort, patients with AVN had higher incidence of CNS disease (p = 0.003), nephritis (p < 0.001), acute or chronic renal failure (p = 0.029), but less frequent photosensitivity (p = 0.006). Patients with AVN also required greater cumulative prednisone from cSLE diagnosis to AVN (364 ± 53 vs. 232 ± 36 mg/kg, p < 0.001), higher prednisone dose at time of AVN diagnosis (±3 months, 0.30 ± 0.25 vs. 0.19 ± 0.24 mg/kg, p = 0.012), maximal daily prednisone dose (1.25 ± 0.36 vs. 0.71 ± 0.53 mg/kg, p < 0.001) and more frequent use of pulse methylprednisolone therapy (39% vs. 10%, p < 0.001). The median prednisone dose at time of AVN was 0.27 vs. 0.09 mg/kg. Multivariable regression analysis confirmed nephritis, CNS disease, maximal daily prednisone dose and use of pulse methylprednisolone as significant predictors of symptomatic AVN development. Overall disease activity from SLE diagnosis to AVN diagnosis as measured by adjusted mean SLEDAI was not significantly different (6310 ± 976 vs. 4994 ± 440, p = 0.165).

Conclusion:

cSLE patients with severe organ involvement including nephritis and CNS disease, higher maximal daily dose of prednisone, and more frequent use of pulse methylprednisolone are more likely to develop symptomatic AVN.
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.
A64: Patient‐Reported Side Effects with Weekly Injections of Methotrexate in Tertiary Care Rheumatology Clinic
Authors: Bechard Melanie , Lemieux Julie , Roth Johannes , Watanabe Duffy Karen , Duffy Ciaran M. , Aglipay Mary , Barrowman Nick , Jurencak Roman
Published: March 2014

Background/Purpose:

Methotrexate is frequently used to treat a variety of paediatric rheumatologic conditions. We explored patient‐reported frequency of side effects and coping strategies for weekly injections of subcutaneous methotrexate.

Methods:

A prospective observational cohort study was conducted from June 2013 through August 2013. All patients with clinic appointments in rheumatology during this period were screened for eligibility. Patients aged 4–17 years who were currently receiving weekly subcutaneous methotrexate injections for at least 4 weeks were invited to participate in the study. A focused interview with the patient and family members, conducted by one interviewer, investigated their experiences with subcutaneous methotrexate. The interview consisted of 18 questions and required approximately 10 minutes to complete.

Results:

42 patients met inclusion criteria and 41 consented to participate in the study. The mean age was 11.2 years (SD = 3.9 yrs) and 68% of participants were female. Most of these patients were diagnosed with juvenile idiopathic arthritis (73%). Mean duration of therapy with subcutaneous injections of methotrexate was 2.5 years (SD = 2.1 yrs) and the dosing range was 0.3\N1 mg/kg/week (maximum 25 mg/week). Almost all patients (95%) reported excellent adherence to the treatment regimen, missing less than one dose per month. The two most commonly reported side effects were nausea and vomiting (56% and 34% of patients, respectively). Less frequently reported side effects included fatigue (29%), anorexia (24%), headache (15%), and recurrent oral ulcers (10%). Of patients who experienced nausea, 74% used dimenhydrinate while 48% used ondansetron to manage their symptoms. Similarly, many patients who experienced vomiting tried to alleviate their symptoms with dimenhydrinate (64%) or ondansetron (50%). While dimenhydrinate effectively treated nausea in only 42% of patients, most patients reported good efficacy of ondansetron (83%). A similar effect was observed for treatment of vomiting (efficacy of dimenhydrinate 12% versus ondansetron 75%). None of the patients tried ginger to alleviate nausea/vomiting. Except for oral ulcers, clinical side effects resolved within 24 hours in 88% of patients. Of patients who had used oral methotrexate in the past, only 26% felt that the oral form was better tolerated.

Conclusion:

More than half of the patients reported at least one side effect that could be attributed to methotrexate injections; the most frequent ones were nausea and vomiting. Importantly, most side effects resolved within 24hours and patients reported excellent compliance. In general, patients did not have a preference for oral methotrexate.
A71: Localized Myositis as a Manifestation of Systemic‐Onset Juvenile Idiopathic Arthritis: A Case Series From a Tertiary‐Care Hospital
Authors: Kitcharoensakkul Maleewan , White Andrew J. , French Anthony R. , Cooper Megan A. , Baszis Kevin W.
Published: March 2014

Background/Purpose:

Localized myositis is rarely reported in patients with systemic‐onset JIA (sJIA), despite common symptoms of myalgia. We describe sJIA patients with the manifestation of localized myositis.

Methods:

We reviewed the charts of 6 pediatric patients with sJIA who developed localized myositis during their disease course.

Results:

The age at presentation with myositis ranged from 5 to 19 years. 4 were males and 2 were females. Races included Caucasian (4), Caucasian/Mayan (1), and African‐American (1). Three patients had localized myositis with initial presentation of sJIA, and 3 patients developed localized myositis during disease flares. For the disease flare group, the time from diagnosis of sJIA until development of myositis ranged from 3 to 6 years, and 2 of 3 patients were on immunosuppressive agents: methotrexate (MTX) (1), anakinra and MTX (1). All 6 patients complained of localized swelling and pain in a localized muscle group. Accompanying symptoms included arthralgia, arthritis, fever and rash. Three patients had localized erythema of the skin overlying myositis areas on exam. Sites of myositis included unilateral biceps (3), bilateral biceps (1), quadriceps (1), and flexor pollicis longus and flexor digitorum superficialis and profundus (1). All patients had at least one muscle enzyme obtained, and all were normal. Five patients had imaging studies demonstrating evidence of myositis: 2 by ultrasound, 2 by ultrasound and MRI, and 1 by MRI. All 6 patients had laboratory evidence of systemic inflammation, including elevated ESR (41–172 mm/hr) and CRP (54–269 mg/L). Four patients had thrombocytosis (462,000–834,000/μL). Two patients transferred care to another hospital, thus we had no information on follow‐up. The remaining 4 patients responded well to prednisone, with resolution of their symptoms within 4 weeks. One patient underwent incision and drainage for a fluid collection and myositis of biceps muscle. He was initially treated with antibiotics and started on anakinra after negative cultures. Duration of follow‐up since onset of myositis was 6–14 months, and no patients had recurrent myositis. The current treatment of the 4 patients includes infliximab (1), MTX (1), canakinumab and leflunomide (1), and canakinumab and MTX (1).

Conclusion:

Localized myositis is a rare extraarticular manifestation of sJIA and can present either as an initial manifestation or during a disease flare. Patients usually present with unilateral or bilateral localized swelling, overlying warmth and erythema, and pain of one muscle group, most commonly the biceps in our series of patients. Our subjects' muscle enzymes were normal, likely secondary to the focal nature of muscle inflammation. Imaging studies are helpful in establishing the diagnosis. To our knowledge, this study represents the largest series to date of sJIA patients with localized myositis.
A79: Pulmonary Hemorrhage in Pediatric Systemic Lupus Erythematous: Clinical Course and Outcomes
Authors: Singla Saimun , Muscal Eyal , Canter Debra , DeGuzman Marietta , Vece Timothy
Published: March 2014

Background/Purpose:

Pulmonary hemorrhage (PH) is a rare and devastating manifestation of SLE, occurring in 1–5% of adult cohorts with mortality rates ranging from 70%‐90%. The majority of PH reported in SLE refers to the adult population, and there is a paucity of data in pediatrics. Therefore, we describe our experience with PH in pediatric SLE, outlining disease presentation, treatment, and outcomes.

Methods:

We reviewed records of pediatric SLE patients who presented with pulmonary involvement diagnosed between January 2000 and August 2010. Inclusion criteria included > 4 SLE classification criteria and PH based on pulmonary evaluation by BAL, lung biopsy and/or characteristic imaging with worsening anemia. We describe our pediatric cohort, presenting symptoms, diagnostic modalities, various interventions and overall outcomes.

Results:

Seven of 401 (1.7%) SLE patients met inclusion criteria for PH. Patients were predominantly male (71.4%) and Hispanic (57.1%). Median age of PH presentation was 13.5 years (range 2.0–15.2). Episodes were primarily within the first 3 months of SLE diagnosis (71.4%) and were a presenting manifestation for SLE in 28.6% of our patients. All patients presented with a new cough, dyspnea, hypoxemia, anemia and abnormal CXR. Hemoptysis was present in 28.6%. Four out of 6 children had chest CT findings with diffuse ground glass opacities (66.6%) and 3 with nodules (50%). Additional non‐pulmonary features included fever (71.4%), renal disease (57.1%), cytopenias (71.4%), cardiac disease (50.0%), and positive antiphospholipid antibodies (85.7%). Eighty‐five percent required supplemental oxygen and 57.1% required mechanical ventilation. Hemosiderin‐laden macrophages without infection were observed in 3 BAL's. Out of the two lung biopsies performed, both revealed diffuse hemorrhage and one demonstrated immunoglobulin and complement deposition. All were treated with pulse doses of methylprednisolone infusions. Other therapeutic interventions included cyclophosphamide (42.8%), plasma exchange (42.8%), rituximab (85.7%) and IVIG (14.2%). Survival rate was 87.5%. All patients were maintained on corticosteroids and 66.6% received monthly cyclophosphamide infusions. Subsequent outpatient therapies included IVIG (33.3%), azathioprine (16.6%) or rituximab (16.6%). No patients had recurrences of PH. No survivors required supplemental oxygen nor developed interstitial lung disease (median follow‐up 2.6 years, range 1.5–5.0).

Conclusion:

In our review of pSLE patients with PH, the prevalence of this complication approximated that of adult SLE cohorts. However, in contrast to the adult population, PH presented earlier in the course of SLE in children. Additionally, outcomes were favorable with initiation of aggressive, multi‐modal immunosuppressive therapy with no recurrences. The mortality rate was much lower than described in adult cohorts.
A90: Pediatric Henoch‐NSchöAdonlein Purpura: Can Length of Hospital Stay Be Predicted?
Authors: Cohen Neta , Mimouni Francis , Freidel Nadav , Amarilyo Gil
Published: March 2014

Background/Purpose:

Henoch Schonlein purpura (HSP) is a common, usually benign, arthritis in childhood. However, at times, it follows a protracted course involving prolonged length of hospital stay (LOS).

Objective:

To determine clinical and laboratory characteristics that allow prediction of prolonged LOS in children with HSP.

Methods:

We retrospectively collected all consecutive cases of HSP that were admitted to the Dana‐Duek Children's hospital between Jan. 1, 2000 and Sep. 30, 2013. All charts were thoroughly reviewed for demographic characteristics (e.g. age, gender), LOS (in days by date), presenting and later symptoms (abdominal pain, vomiting, GI bleeding, palpable purpura, arthralgia or periarthritis, fever) and laboratory data on admission and discharge (CBC, CRP, ESR, serum creatinine, urinalysis). In univariate analysis we studied the variables that were statistically related to LOS. Variables that significantly predicted LOS in univariate analysis or that were clinically relevant (i.e. Proteinuria (defined as positive protein in urinalysis in 2 consecutive tests), systemic fever (defined as rectal temperature ≥38.0°C), gastrointestinal (GI) bleeding, arthralgia, CRP concentration, age (years) and abdominal pain as sole presenting symptom on admission) were analyzed in stepwise backward multiple regression analysis. Variables that remained significant in the final analysis were correlated with LOS, were each given specific weight (according to their contribution to the final R square) and were used to assemble an HSP severity score. Discriminate analysis was used to determine the score best discriminating patients with prolonged LOS (4 or more days) from the others.

Results:

Eighty nine children (aged 9 months–17 years) were retrieved for analysis. In univariate analysis, variables that were associated with prolonged LOS were: abdominal pain as initial sole presentation (P < 0.001), CRP (P = 0.001) and systemic fever (P = 0.018). Four variables remained significant in the final stepwise multiple regression analysis, and were used as parameters in assembling the HSP severity score. The HSP severity score consisted of sole abdominal pain at presentation (0 or 3 points), Fever (0 or 2 points), CRP ≥45 (0 or 1 point) and Age ≥6 (0 or 0.5 points). A score of ≥3 predicted a prolonged LOS (4 or more days) with a sensitivity of 76%, a specificity of 79% and a positive predictive value of 93%.

Conclusion:

Four selected variables available on the day of admission allow predicting the severity (in terms of LOS) of HSP. This score will be validated in an ongoing prospective study.
A100: Predictors of Involvement in Leisure Activities Among Children and Youth With Juvenile Idiopathic Arthritis
Authors: Cavallo Sabrina , Majnemer Annette , Duffy Ciarán M. , Feldman Debbie Ehrmann
Published: March 2014

Background/Purpose:

Children with juvenile idiopathic arthritis may be less involved in leisure activities compared to their healthy peers in part due to disease related factors such as pain, joint stiffness and tenderness. The main purpose of our study was to describe involvement in leisure activities, as well as to identify potential socio‐demographic, disease‐related, personal and environmental determinants.

Methods:

Ninety three children and youth aged 8 to 18 years diagnosed with juvenile idiopathic arthritis (JIA) and their families participated in this cross‐sectional study. Children and youth were administered the Children's Assessment of Participation and Enjoyment (CAPE), which measures involvement in leisure activities (recreation, physical, social, skill‐based, self‐improvement). The disease characteristics were abstracted from the child's medical file (JIA sub‐type, active joint count, age of diagnosis), pain perception, functional status, motivation, self‐perception and perceived social support were obtained through self‐report. Parents completed questionnaires on socio‐demographic data, as well as on family function and environmental barriers. Multiple linear regression was used for data analysis.

Results:

The leisure activities completed by most participants (>90%) were playing computer/video games, watching television, hanging out with friends and doing homework. Least popular activities were (h10%) martial arts, gymnastics and horseback riding. The level of enjoyment was highest for social activities (mean: 4.2+0.5). Involvement in active physical activities was negatively associated with the presence of active disease (β = −0.385, p = 0.028). However a higher level of perceived athletic competence was associated with greater intensity in active physical activities (β = 0.261, p = 0.037). Higher environmental barrier scores were negatively associated with involvement in social (β = −0.222, p = 0.023) leisure activities.

Conclusion:

Disease activity may dissuade children and youth from participating in more active pursuits, which places them at greater risk for adopting sedentary lifestyles. There is evidence that contextual factors (personal and environmental) influence leisure participation. The identification of determinants of leisure activities in children and youth with arthritis may allow healthcare professionals to assess children's health needs with more precision and promote a healthier lifestyle.
A101: The Impact of Sleep and Cytokine Levels on Pain Perception in JIA
Authors: De Ranieri Deirdre I. , Wagner‐Weiner Linda , Tesher Melissa , Bandla Hari , Onel Karen
Published: March 2014

Background/Purpose:

Children with JIA experience more pain and disordered sleep than healthy children. JIA and poor sleep are associated with particular cytokine abnormalities, and the correlation between the two and how they relate to a patient's pain perception have not been studied. Elevation in TNF‐a, IL‐6, IL‐17, and IFN‐g have been found in the serum of children with JIA during active disease. Levels of proinflammatory cytokines are also increased during sleep loss. Indeed, blocking TNF‐a has been shown in adults with RA to improve sleep the night after the first dose. We hypothesize that dysfunctional sleep enhances pain in our patients and may be associated with abnormal cytokine profiles. In this pilot study, we hope to identify a subgroup of children with JIA that could benefit from intervention in improving sleep patterns.

Methods:

We plan to enroll 60 patients with JIA. Sleep is assessed by the sleep self‐report (SSR), the children's sleep habits questionnaire (CSHQ), and actigraphy. Good concordance in measurements of sleep duration has been established between PSG and actigraphy. Patients wear an actigraphy watch and keep a daily sleep and pain diary for 14 days. Disease activity is assessed using ESR, sum of active joints, patient/parent and physician global assessment of disease activity. Serum cytokine testing will be performed by flow cytometry.

Results:

31 patients are enrolled. We identified 7 children with disordered sleep using CSHQ/ SSR. These patients report having pain on most days, and they trend toward having elevated disease activity (increased physician and patient global assessments as well as number of active joints, ESR was non‐contributory). To date, 9 of the 31 patients underwent actigraphy. Preliminary data does not indicate an association between disease activity and objective sleep dysfunction. There is not yet enough data to comment on the association of pain and disordered sleep in the absence of disease activity. Serum from 22 patients has been collected for cytokine studies.

Conclusion:

There is a trend toward an association of perceived poor sleep (as reported subjectively by SSR/CSHQ) with increased pain and disease activity in our patient population. Increased disease activity does not appear to be objectively related to disordered sleep using actigraphy, however. So far, actigraphy has been conducted on patients without subjective complaints of sleep dysfunction. We plan to collect more objective data (via actigraphy) on patients with subjective sleep complaints (with and without active disease) to better characterize the relationship of pain and sleep. When this has been accomplished, we plan to investigate cytokine profiles in these groups.
A111‐a: Setting Up of Japanese Online Registry of Pediatric Rheumatic Disease
Authors: Yamaguchi Ken‐ichi , Nerome Yasuhito , Imagawa Tomoyuki
Published: March 2014

Background/Purpose:

Performing quality clinical and translational research in pediatric rheumatic diseases (eg. Juvenile Idiopathic Arthritis (JIA), Child onset Systemic Lupus Erythematosus (cSLE) and Juvenile Dermatomyositis (JDM)) had been difficult due the rarity of these diseases.

Methods:

Professor Takei, the former chairman of Pediatric Rheumatology Association of Japan (PRAJ), had ordered his working group to set up a new registry system in 2012. We, working group, have set up Japanese online registry for pediatric rheumatic disease in 2014 and have named it as Pediatric Rheumatology International Collaboration Unit Registry (PRICURE).

Results:

Under this registry, PRAJ will collect basic information (e.g. age of onset, sex, symptoms, major complication, main treatment) about these diseases (JIA, SLE, JDM, Sjogren syndrome, Mixed Connective Tissue Disease, Systemic Sclerosis, Behcet's disease, Vasculitis, Autoinflammatory syndromes, Fibromyalgia).

Conclusion:

We had set up new registry of pediatric rheumatic diseases. We will show initial data from this registry here.