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Specific Learning Disabilities (SLD) are often associated with emotional-behavioral problems. Many studies highlighted a greater psychopathological risk in SLD, describing both internalizing and externalizing problems. The aims of this study were to investigate the emotional-behavioral phenotype through the Child Behavior Checklist (CBCL), and evaluate the mediating role of background and cognitive characteristics on the relationship between CBCL profile and learning impairment in children and adolescents with SLD. One hundred and twenty-one SLD subjects (7–18 years) were recruited. Cognitive and academic skills were assessed, and parents completed the questionnaire CBCL 6–18. The results showed that about half of the subjects manifested emotional-behavioral problems with a prevalence of internalizing symptoms, such as anxiety and depression, over externalizing ones. Older children showed greater internalizing problems than younger ones. Males have greater externalizing problems compared to females. A mediation model analysis revealed that learning impairment is directly predicted by age and familiarity for neurodevelopmental disorders and indirectly via the mediation of the WISC-IV/WAIS-IV Working Memory Index (WMI) by the CBCL Rule-Breaking Behavior scale. This study stresses the need to combine the learning and neuropsychological assessment with a psychopathological evaluation of children and adolescents with SLD and provides new interpretative insights on the complex interaction between cognitive, learning, and emotional-behavioral phenotypes.

ObjectiveTo develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups.MethodsCandidate variables were assembled from published criteria and expert opinion using consensus methodology. Data were collected from 47 rheumatology, dermatology, neurology and paediatric clinics worldwide. Several statistical methods were used to derive the classification criteria.ResultsBased on data from 976 IIM patients (74% adults; 26% children) and 624 non-IIM patients with mimicking conditions (82% adults; 18% children), new criteria were derived. Each item is assigned a weighted score. The total score corresponds to a probability of having IIM. Subclassification is performed using a classification tree. A probability cut-off of 55%, corresponding to a score of 5.5 (6.7 with muscle biopsy) ‘probable IIM’, had best sensitivity/specificity (87%/82% without biopsies, 93%/88% with biopsies) and is recommended as a minimum to classify a patient as having IIM. A probability of ≥90%, corresponding to a score of ≥7.5 (≥8.7 with muscle biopsy), corresponds to ‘definite IIM’. A probability of ConclusionsThe European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for IIM have been endorsed by international rheumatology, dermatology, neurology and paediatric groups. They employ easily accessible and operationally defined elements, and have been partially validated. They allow classification of ‘definite’, ‘probable’ and ‘possible’ IIM, in addition to the major subgroups of IIM, including juvenile IIM. They generally perform better than existing criteria.

Introduction Presence of sleep onset REM episodes often raises concerns of narcolepsy. However other conditions have shown to have presence of sleep on REM episodes which include but not limited to obstructive sleep apnea, sleep wake schedule disturbance, alcoholism, neurodegenerative disorders, depression and anxiety Report of Case Here we present a case of 30 year old female with history of asthma, patent foraman ovale, migraine headache, and anxiety who presented with daytime sleepiness, falling asleep while at work, occasional scheduled naps, non-restorative sleep, sleep paralysis, and hypnopompic hallucination. Pertinent physical exam included; mallampati score of 4/4, retrognathia, high arched hard palate, crowded posterior oropharynx. She had a score of 16 on Epworth sleepiness scale. Patient previously had multiple sleep latency test at outside facility which revealed 4/5 SOREM, with mean sleep onset latency of 11.5 minutes. She however was diagnosed with narcolepsy and tried on modafinil which she failed to tolerate. She was tried on sertraline as well which was discontinued due to lack of benefit. She had repeat multiple sleep latency test work up which revealed 2/5 SOREM, with mean sleep onset latency was 13.1 minutes. Her overnight polysomnogram prior to repeat MSLT showed SOREM with sleep onset latency of 10 minutes. Actigraphy showed consistent sleep pattern overall with sufficient sleep time but was taking hydroxyzine and herbal medication. Patient did not meet criteria for hypersomnolence disorder and sleep disordered breathing. Conclusion There is possibility her medication may have played pivotal role with her daytime symptoms. We also emphasize SOREMs can be present in other disorders such as anxiety in this case and not solely in narcolepsy

Objective To compare the criteria for Wegener's granulomatosis (WG) of the American College of Rheumatology (ACR) with those of the European League Against Rheumatism/Pediatric Rheumatology European Society (EULAR/PRES) in a cohort of children with WG and other antineutrophil cytoplasmic antibody (ANCA)–associated vasculitides (AAVs), and to describe the interval to diagnosis, presenting features, and initial treatment for WG. Methods Eligible patients had been diagnosed by site rheumatologists (termed the “MD diagnosis”) since 2004. This diagnosis was used as a reference standard for sensitivity and specificity testing of the 2 WG classification criteria. Descriptive analyses were confined to ACR-classified WG patients. Results MD diagnoses of 117 patients (82 of whom were female) were WG (n = 76), microscopic polyangiitis (n = 17), ANCA-positive pauci-immune glomerulonephritis (n = 5), Churg-Strauss syndrome (n = 2), and unclassified vasculitis (n = 17). The sensitivities of the ACR and EULAR/PRES classification criteria for WG among the spectrum of AAVs were 68.4% and 73.6%, respectively, and the specificities were 68.3% and 73.2%, respectively. Two more children were identified as having WG by the EULAR/PRES criteria than by the ACR criteria. For the 65 ACR-classified WG patients, the median age at diagnosis was 14.2 years (range 4–17 years), and the median interval from symptom onset to diagnosis was 2.7 months (range 0–49 months). The most frequent presenting features by organ system were constitutional (89.2%), pulmonary (80.0%), ear, nose, and throat (80.0%), and renal (75.4%). Fifty-four patients (83.1%) commenced treatment with the combination of corticosteroids and cyclophosphamide, with widely varying regimens; the remainder received methotrexate alone (n = 1), corticosteroids alone (n = 4), or a combination (n = 6). Conclusion The EULAR/PRES criteria minimally improved diagnostic sensitivity and specificity for WG among a narrow spectrum of children with AAVs. Diagnostic delays may result from poor characterization of childhood WG. Initial therapy varied considerably among participating centers.

During the past decade, the critical role of sleep in health and disease has been underscored by research that further defines the relationship between sleep and myriad physiologic and psychological functions as well as quality of life. For many years, there was little exploration of the significance of sleep and sleep disorders in cancer patients; however, the past decade has seen a steady growth of inquiry in this area. These investigations have demonstrated the high frequency and significance of sleep disturbance as a symptom in cancer patients. They have also explored the complex interaction between sleep and other common cancer symptoms, most notably fatigue, depression, and pain, and have identified risk factors associated with the development of sleep problems in this population. Although treatment studies lag behind, reports of effective psychological and behavioral interventions for insomnia in cancer patients are increasing. Several studies are addressing pharmacotherapeutic intervention for hot flashes as a potential source of sleep disturbance. Other sleep disorders, most notably obstructive sleep apnea, also occur with some regularity in cancer patients.

Pediatric nephrology and rheumatology patients with steroid-induced osteopenia are at risk of skeletal fracture. Bisphosphonate therapy has not been routinely advocated as a primary or secondary intervention for steroid-associated fractures in this population. This case control study evaluates the role of pamidronate therapy as a secondary fracture intervention. Children with symptomatic pathological fractures of the axial spine or ribs were treated with pamidronate 1 mg/kg/dose (n=17) IV at 60-day intervals for 1 yr (n=15) or 2 yr (n=2). Bone mineral density of L1-L4 (BMD) was assessed prior to treatment and at six-month intervals, and compared to 17 disease-age-gender-steroid dose-matched control patients. Alkaline phosphatase, calcium, phosphate, PTH, renal biochemistry, and 24-hr urine collections for CrCl, N-telopeptide/creatinine ratio, phosphate excretion, and calcium excretion were obtained every two months in the pamidronate population. Pamidronate caused a first exposure transient flu-like illness lasting

Objective The Childhood Health Assessment Questionnaire (CHAQ), Juvenile Arthritis Functional Assessment Report (JAFAR), and Juvenile Arthritis Functional Status Index (JASI) are widely used functional measures for juvenile idiopathic arthritis (JIA) that differ in content, format, and completion time. We compared the responsiveness and child-parent agreement of the JAFAR, CHAQ, and JASI in a prospective, multicenter study. Methods Children and adolescents from 5 rheumatology centers were enrolled. Subjects were about to undergo therapy (intraarticular corticosteroid injections [IAS] and methotrexate or hip surgery (MTX/hip]) expected to produce a functional improvement. All subjects were studied before the intervention and at 6 weeks and 6 months posttreatment. At each study visit, the 3 measures were administered in randomized, balanced order to both parents and children. Results A total of 92 subjects (mean age 12.8 years) were enrolled in the study, 74 of which were in the IAS group. The responsiveness of all 3 measures was moderate to strong. The standardized response mean at 6 weeks for the IAS group on the JAFAR, CHAQ, and JASI was 0.41 (95% confidence interval [95% CI] 0.18, 0.64), 0.70 (95% CI 0.47, 0.93), and 0.36 (95% CI 0.13, 0.59), respectively. The CHAQ was somewhat more responsive to change at 6 weeks (IAS group: relative efficiency 0.34 [JAFAR], 0.27 [JASI]), but less responsive at 6 months (MTX/hip group: relative efficiency 5.1 [JAFAR], 3.9 [JASI]). All 3 questionnaires showed acceptable parent-child agreement, and overall, there were few differences between the 3 questionnaires. Conclusion The functional outcome measures currently used for JIA are all adequately responsive for use in trials or in the clinic setting. The choice of which measure to use should therefore be based on the time available for completion, the intended clinical/research use, and the depth of content required.

Parent-child interactions during pain-inducing exercise tasks among children (11-17 years old) with fibromyalgia, juvenile rheumatoid arthritis, and pain-free controls were examined and the contribution of parent-child interactions to disability was tested. Fifteen children in each of the three diagnostic groups and their parents completed 5-min exercise tasks and completed questionnaire measures of disability (Functional Disability Inventory) and coping (Pain Coping Questionnaire). There were few group differences in parent-child interactions. After controlling for children's ratings of pain evoked by the exercise, group differences in interactions during exercise tasks were no longer significant. Sequential analyses, controlling for group and exercise task, revealed that when parents made statements discouraging coping following children's negative verbalizations about the task or pain, children were less likely to be on task, compared to when parents made statements encouraging coping or when parents made any other statements. Children's general pain coping strategies were not related to parent-child interactions. Parent-child interactions were generally not related to disability. Across the groups, more pain and less time on task during the exercises were related to Functional Disability Inventory scores and more school absences. Parent-child interaction patterns influence children's adaptation to pain during experimental tasks. Parents' discouragement of coping in response to their children's negative statements related to the pain or the pain-evoking task are counter productive to children's ability to maintain activity in a mildly painful situation.

Objective: To describe the methodology used to develop new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups. Methods: An international, multidisciplinary group of myositis experts produced a set of 93 potentially relevant variables to be tested for inclusion in the criteria. Rheumatology, dermatology, neurology, and pediatric clinics worldwide collected data on 976 IIM cases (74% adults, 26% children) and 624 non-IIM comparator cases with mimicking conditions (82% adults, 18% children). The participating clinicians classified each case as IIM or non-IIM. Generally, the classification of any given patient was based on few variables, leaving remaining variables unmeasured. We investigated the strength of the association between all variables and between these and the disease status as determined by the physician. We considered three approaches: (1) a probability-score approach, (2) a sum-of-items approach criteria, and (3) a classification-tree approach. Results: The approaches yielded several candidate models that were scrutinized with respect to statistical performance and clinical relevance. The probability-score approach showed superior statistical performance and clinical practicability and was therefore preferred over the others. We developed a classification tree for sub-classification of patients with IIM. A calculator for electronic devices, such as computers and smart phones, facilitates the use of the EULAR/ACR classification criteria. Conclusions: The new EULAR/ACR classification criteria provide a patient’s probability of having IIM for use in clinical and research settings. The probability is based on a score obtained by summing the weights associated with a set of criteria items.

We developed a rapid in vitro antibiotic susceptibility test to screen double- and triple-antibiotic combinations for bactericidal activity against 75 multiresistant Pseudomonas aeruginosa isolates referred from 44 cystic fibrosis (CF) patients. When used alone, the most effective intravenous antibiotic, meropenem, was bactericidal against only 44% of the isolates. High-dose tobramycin (200 microg/ml; concentrations achievable by aerosol administration) was bactericidal against 72% of isolates. Adding a second antibiotic significantly improved bactericidal activity. The most effective double-antibiotic combinations contained high-dose tobramycin plus meropenem, piperacillin/tazobactam, or ciprofloxacin, and were bactericidal against 88 to 94% of the isolates. Excluding high-dose tobramycin, the most effective intravenous double-antibiotic combinations contained meropenem plus ciprofloxacin, tobramycin (4 microg/ml), or cefipime, and were bactericidal against 85%, 71%, and 70% of isolates, respectively. Adding a third antibiotic did not significantly improve inhibition in vitro. We conclude that double-antibiotic combinations containing meropenem or high-dose tobramycin show the best bactericidal activity in vitro against multiresistant strains of P. aeruginosa. Addition of a third antibiotic to these double-antibiotic combinations may be unnecessary.

Objective To examine the ability of a previously described set of criteria to predict poor functional outcome in a large, multicenter cohort of children with systemic-onset juvenile rheumatoid arthritis (JRA). Methods All children who were diagnosed with systemic-onset JRA since 1980 at the Hospital for Sick Children (Toronto), since 1983 at the Isaac Walton Killam Hospital for Children (Halifax), and since 1981 at the Children's Hospital of Eastern Ontario (Ottawa) were evaluated. Patients were included in the study if they had been evaluated clinically within 6 months of diagnosis and had been followed up for at least 2 years. Patients were divided into 4 cohorts according to their length of followup: 2–4 years, 4–7 years, 7–10 years, and >10 years. Using previously described criteria for destructive arthritis in children with systemic-onset JRA, the patients were classified as either high risk or low risk for poor functional outcome based on the data from their 6-month visit. High-risk patients had active systemic disease (persistent fever or corticosteroid requirement for control of systemic disease) and a platelet count ≥600 × 109/liter. Poor outcome was defined as moderate or severe disability (defined as a score of ≥0.75 on the Childhood Health Assessment Questionnaire) or disease-associated death. Results Among 122 eligible patients with systemic-onset JRA, we were able to contact 111 (91%) for outcome data. The mean followup period was 7.7 years (SD 3.7). The mean age at outcome assessment was 13.5 years (SD 5.3). There were 51 boys and 60 girls. Twenty-four patients (22%) had moderate-to-severe disability and 2 patients died; these 26 patients were considered to have had a poor outcome. We could determine risk classification for 104 patients. Twenty-four patients (23%) met the criteria for high risk at the 6-month visit. Overall, the risk of a poor functional outcome was significantly higher in the high-risk group (relative risk 3.3, 95% confidence interval [95% CI] 1.73–6.43, P = 0.0004). This risk was most marked in the cohort with >10 years of followup (relative risk 4.3, 95% CI 1.82–10.29, P = 0.006). Conclusion The presence of active systemic disease at 6 months, as characterized by fever or the need for corticosteroids, and thrombocytosis strongly predicted the development of a poor functional outcome in these patients. This was especially apparent with long-term followup. Our study validates the previously developed prognostic criteria for systemic-onset JRA.

We examined the adhesion of monocytes and polymorphonuclear leukocytes (PMNLs) to the neuroblastoma (NB) cell lines SK-N-SH and SK-N-MC, which have some distinct differentiation characteristics. Monocytes adhered to SK-N-SH and SK-N-MC to the same extent (20 +/- 1.4% and 24 +/- 0.8% of monocytes added). Monocyte adhesion to SK-N-SH but not SK-N-MC was partially inhibited by treating monocytes with a mAb to the CD18 (beta2) integrin chain. The adhesion was further inhibited when monocytes were treated with a combination of mAb to CD18 and VLA-4. Treatment of both NB cell lines with interleukin-1alpha (0.5 ng/ml), tumor necrosis factor alpha (100 U/ml), interferon gamma (200 U/ml), or their combinations increased monocyte adhesion to SK-N-SH and SK-N-MC. With each condition, monocyte adhesion to SK-N-SH was partially blocked by mAb to CD18. The inhibition of adhesion to IL-1alpha- or TNFalpha-treated SK-N-SH cells was greater when the monocytes were treated with mAb to both CD18 and VLA-4. In contrast, monocyte adhesion to IL-1alpha or IFNgamma treated SK-N-MC was only slightly inhibited with a combination of mAb to CD18 + VLA-4 and there was no inhibition at all to TNFalpha-treated SK-N-MC. Spontaneous PMNL adhesion to SK-N-SH was almost negligible but increased by treating the cell line with IL-1alpha, TNFalpha, IFNgamma or their combinations. A mAb to CD18 blocked this increase in each case. The pattern of adhesion of PMNLs to SK-N-MC was totally different. PMNL adhesion to unstimulated SK-N-MC was very high (24 +/- 1.3%), was not inhibited by mAb to CD18, and did not increase by stimulating the cell line with IL-1alpha, TNFalpha, IFNgamma or their combinations. Overall, these results suggest two distinct patterns of monocyte and PMNL interaction with neural cells, such as the SK-N-SH and MC cell lines. While monocyte and PMNL adhesion to SK-N-SH is mainly via CD18/VLA-4 or the CD18 mechanisms, respectively, leukocyte adhesion to SK-N-MC is CD18- and VLA-4-independent. Thus, leukocyte-neural cell interactions share some mechanisms common also to leukocyte-endothelium interaction, but there are also unique mechanisms which may be neural cell and differentiation specific.

We investigated the effect of TNF alpha, IL-1alpha and IFN gamma on two neuroblastoma (NB) cell lines (SK-N-SH and SK-N-MC). These lines responded differentially to IL-1alpha, TNF alpha and IFN gamma for MCP-1 and IL-8 production and expression of the ICAM-1 and VCAM-1 adhesion molecules. None of the cytokines induced MCP-1 or IL-8 on SK-N-MC cells. Both chemokines were produced in response to IL-1alpha by SK-N-SH cells, while TNF alpha induced mainly MCP-1 production. Addition of IFN gamma decreased IL-8, but not MCP-1 production. These responses correlated with monocyte and neutrophil chemotactic activity in NB culture supernatants. This activity was neutralized by antibodies to IL-8 and MCP-1. The expression of ICAM-1 on SK-N-MC was up-regulated by TNF alpha or IFN gamma, while IL-1alpha also upregulated ICAM-1 on SK-N-SH cells. VCAM-1 expression on SK-N-SH was induced by IL-1alpha and TNF alpha and IFN gamma synergized with TNF alpha in this respect on both NB cell lines. These results suggest that mechanisms for chemokine production and VCAM-1 and ICAM-1 upregulation by inflammatory cytokines differ and IFN gamma, in conjunction with TNF alpha, stimulate neural cell responses (high MCP-1 and VCAM-1 and decreased IL-8) favouring mononuclear cell recruitment.

Monocytes migrate through vascular endothelium, and then in connective tissue. As a model of this process, we investigated adhesion molecules involved in monocyte migration through HUVEC and a barrier of human synovial fibroblasts (HSF). Minimal spontaneous monocyte migration (6–7%) occurred through either cell barrier, but this increased markedly (27–35% of added monocytes) when a C5a chemotactic gradient was present. Migration across unstimulated HUVEC was partially inhibited (40%) by mAb to CD18 (β2 integrin) and completely blocked by anti-CD18 plus anti-α4 (CD49d; very late Ag-4 (VLA-4)) mAbs. In contrast, migration across HSF induced by C5a or monocyte chemoattractant protein-1 was not inhibited by mAb to CD18 and was only partially inhibited (33%) in combination with anti-α4 mAb. The CD18- and VLA-4-independent migration across HSF was completely inhibited by mAb to α5 of VLA-5. The inhibitory effect of mAbs to VLA-4 and VLA-5 was on the monocyte and required blockade of CD11/CD18 to be observed. In contrast to HSF, no role for VLA-5 in monocyte transendothelial migration was detected. Both HSF and IL-1-stimulated HUVEC expressed vascular cell adhesion molecule-1 (VCAM-1). However, VLA-4-mediated monocyte migration across HSF was only partially dependent on VCAM-1, in contrast to transendothelial migration, which was completely blocked by anti-VCAM-1 mAbs. In conclusion, unlike transendothelial migration, for which VLA-4 is the alternative mechanism to CD11/CD18 on monocytes, both VLA-4 and VLA-5 can mediate monocyte migration through fibroblast barriers. In addition to VCAM-1, other ligand(s) on HSF are also involved in the VLA-4-mediated migration.

Objectives. 1) To determine the importance of psychological adjustment and family functioning in primary juvenile fibromyalgia by assessing these factors in children with fibromyalgia and in their parents, compared with children with juvenile rheumatoid arthritis (JRA) and with pain-free control children and their parents. 2) To examine which of these factors predict functional disability. Methods. Fifteen children in each of the 3 study groups, and their parents, completed self-report questionnaires and pain diaries. A medical evaluation of each child was performed, including assessment of tender points by palpation and by dolorimetry. Results. All children in the fibromyalgia group met the Yunus and Masi criteria for fibromyalgia, and 11 met the American College of Rheumatology criteria. There were almost no significant group differences in the children's or parents' psychological adjustment, ratings of family functioning, or coping strategies. Significant group differences in functional disability, pain, fatigue, tender point threshold, and control point tolerance were found. A number of the psychological adjustment, pain, fatigue, and coping variables were significantly associated with functional disability. Conclusion. The notion that fibromyalgia is a psychogenic condition is not supported by these results. Fibromyalgia is associated with disability of a magnitude comparable to that of other chronic pain conditions. Disability among children with fibromyalgia or JRA is a function of the children's psychological adjustment and physical state, and of the parents' physical state and method of coping with pain.

Corticosteroid related changes in body mass index in children and adolescents with rheumatic diseases Natalie Shiff, Rollin Brant, David A Cabral, Jaime Guzman, Peter B Dent, Janet E Ellsworth, Kristin M Houghton, Adam Huber, Roman Jurencak, Bianca A Lang, Maggie Larche, Claire MA LeBlanc, Paivi M Miettunen, Kiem G Oen, Johannes Roth, Claire Saint-Cyr, Rosie Scuccimarri, Leanne M Ward, Canadian STOPP Consortium

Pseudoporphyria, a cutaneous disorder characterized by skin fragility, vesiculation, and scarring, has been reported as a side effect of naproxen therapy in children with juvenile rheumatoid arthritis (JRA). We report the results of a 6-month prospective study to determine the prevalence of pseudoporphyria in our JRA population. All the patients with pseudoporphyria had received naproxen foror = 4 weeks at the time of the study. Of the patients treated with naproxen, 12% (9/74) developed this complication. No patient had significant elevation of free erythrocyte protoporphyrin, excluding the diagnosis of true erythropoietic protoporphyria. We conclude that pseudoporphyria is a common side effect of naproxen therapy in children with JRA, even in geographic areas without high sun exposure. Because of the risk of facial scarring with pseudoporphyria, physicians and parents of children with JRA should be aware of this complication.

Children with neurodevelopmental disabilities, such as cerebral palsy, frequently have associated oral motor dysfunction, which leads to feeding difficulties, risk of aspiration, prolonged feeding times, and malnutrition with its attendant physical compromise. The authors propose a comprehensive multidisciplinary assessment, including neurological and dysphagia examination and ear, nose, and throat examination, to evaluate clinical indicators and severity of feeding impairment in children affected by neurodevelopmental disorders. A representative sample of 40 children with cerebral palsy (spastic, dyskinetic, or mixed), intellectual disability, and feeding problems was included in the study. A specific multidisciplinary evaluation and standardized mealtime observation in patients with cerebral palsy appear feasible and appropriate to recognize proactive indicators of dysphagia and to establish personalized programs of gastric and rehabilitative interventions.

Background/Purpose: To determine the incidence of vertebral fracture (VF) in the three years following glucocorticoid (GC) initiation, and the effect of GC on the risk of incident VF in children with rheumatic disorders (RD). Methods: Children with RD were enrolled within 30 days of GC initiation and followed for 3 years, with VF assessed at baseline and then annually using the Genant method on lateral spine radiographs. An incident VF was defined as a new fracture in a previously normal vertebral body or worsening of an existing VF. The annual and the 3 year cumulative incidences were calculated as the number of new cases divided by the number of subjects who completed VF assessment at the specified time periods. Six different time-dependent GC exposure measures were constructed: cumulative dose, average daily dose, duration on GC therapy, dose intensity (cumulative dose divided by duration on GC therapy) over 3 years, recent average daily dose and duration on GC therapy over the preceding 12 months. Extended Cox's models were used with alternative time-dependent variables, adjusting for factors at baseline including age, gender, body mass index, vitamin D and calcium supplementation, disease activity, bone mineral density, physical activity, and presence of prevalent VF. Results: 136 enrolled children (mean ± SD age 9.9 ± 4.4 years, 65% girls) held the following diagnoses: 22% juvenile dermatomyositis (JDM), 21% non-systemic juvenile idiopathic arthritis (JIA), 19% systemic lupus erythematosus (SLE), 18% systemic JIA, 12% systemic vasculitis, and 9% other RD. 9 children had VF at baseline (3 JDM, 2 systemic JIA, 2 SLE, 1 systemic vasculitis, 1 localized scleroderma), with an estimated prevalence of 6.6% (95% confidence interval (CI) 2.4–10.8%). 18 incident VF were identified in 15 children during the 3 years following GC initiation (6 JDM, 4 SLE, 2 systemic vasculitis, 2 systemic JIA, and 1 non-systemic JIA). The 3-year cumulative VF incidence was 13.0%, at a rate of 4.9 per 100 person-years. Of the 18 incident VF events, 7 occurred during the first year at an annual incidence of 6.0% (95% CI 1.7–10.3%); 6 VF occurred during the second year (annual incidence 5.6%; 95% CI 1.2–9.9%); 5 VF occurred during the third year (annual incidence 4.3%; 95% CI 1.0–8.1%). 64% of patients had mild incident VF as the worst grade, 36% had moderate fractures, and none had severe fractures. Of those with incident fractures, 21% had no complaints of back pain in the preceding 12 months. Multivariable models showed that every 100 mg/kg increase in cumulative GC dose was associated with a 30% increased incident VF risk (hazard ratio (HR) = 1.3, 95% CI 1.1, 1.6). Every 0.5mg/kg increase in average daily dose over the 3 year period was associated with a 4.2 fold increased VF risk (HR = 4.2, 95% CI 2.3, 7.7), and every 0.5 mg/kg increase in daily dose during the preceding 12 months was associated with a 2.7 fold increased incident VF risk (HR =2.7, 95% CI 1.3, 5.2). Conclusion: We have shown that 13.0% of children with RD sustained VF within three years followingGC initiation, of which 36% had moderate fractures. Cumulative dose, average daily GC dose, and recent average daily GC dose were associated with a significantly increased VF risk. Funded by CIHR FRN 64285

purpose: The mainstay of pharmacologic therapy in patients with dermatomyositis is corticosteroids. However, because patients sometimes become refractory to these drugs and because these drugs have potential short- and long-term toxicities, alternate therapy is highly desirable. Therefore, a pilot study was initiated using high-dose intravenous gammaglobulin (IVGG) in the treatment of dermatomyositis. patients and methods: IVGG was administered to five patients with juvenile dermatomyositis. Prior to IVGG treatment, all patients had persistent muscle weakness despite daily corticosteroids and three patients had developed unacceptable steroid toxicity. Two of the patients had previously developed toxicity while receiving immunosuppressive therapy. results: IVGG therapy resulted in improved muscle strength and ameliorated skin rash in all patients. The percentage increase in muscle strength as measured by sphygmomanometry following the 9-month course of IVGG ranged from 56% to 606% in the proximal lower extremities and from 30% to 186% in the proximal upper extremities. Following IVGG therapy, prednisone could be discontinued or the dose reduced in all patients. conclusion: This study suggests that IVGG may allow steroid sparing in dermatomyositis and may provide a safe alternative to cytotoxic therapy.

Objective. To determine, in a case-control study, if patients with new-onset juvenile dermatomyositis (juvenile DM) have increased symptoms prior to onset, exposure to certain environmental conditions, frequency of familial autoimmune diseases, or antibody titers, compared with 2 control groups. Methods. A structured interview with the families of 80 children with juvenile DM, 40 children with juvenile rheumatoid arthritis (JRA), or 23 healthy children, from the same geographic area as the children with juvenile DM, was conducted. All children's sera were tested for antibody to Toxoplasma gondii, herpes simplex virus (HSV), or coxsackievirus B (CVB). Results. A high proportion of children with juvenile DM had constitutional symptoms 3 months before the disease-onset date (P = 0.013 versus control children). Children with JRA had more relatives with rheumatoid arthritis (P = 0.0001) and pernicious anemia (P = 0.003) than did children with juvenile DM or healthy children. Among children ⩽7 years of age, elevated enteroviral titers were more frequent in those with juvenile DM (81%) and in healthy controls (90%) than in those with JRA (64%), suggesting a common environmental exposure. Titers to T gondii, HSV, or CVB 1-6 were normal. Conclusion. Frequencies of familial autoimmune disease, exposure to environmental factors, or elevated antibody titers to T gondii, HSV, or CVB are not increased in juvenile DM. Children with juvenile DM do have symptoms of illness 3 months before the disease-onset date, and young patients have elevated enteroviral titers, as do young geographic controls.

OBJECTIVE: This study was undertaken to investigate the recent finding of a seasonal difference in the onset of systemic-onset juvenile rheumatoid arthritis (SoJRA). We hypothesized that a seasonal onset pattern might implicate an infectious agent as a cause of SoJRA. METHODS: The date of onset was collected from the records of all patients with SoJRA from 1980 to 1992 at presentation to pediatric rheumatology clinics across Canada. The onset pattern of SoJRA was then compared with incidence data on viral infections obtained for the same period. RESULTS: Across Canada the onset of SoJRA was constant across the seasons. However, in the Prairie region there was a statistically significant seasonal pattern, with peaks in autumn and early spring. We could find no evidence that viral incidence correlated with disease incidence either throughout Canada or in the Prairie region. CONCLUSIONS: If a seasonal infectious agent causes SoJRA, then it is likely only one of several causes and may act only in certain regions. Future studies should be carried out in those areas where SoJRA does have a seasonal onset pattern. (J P EDIATR 1996:129:513-8)

Incident vertebral fractures 12 months following glucocorticoid initiation in children with rheumatic disorders Bianca A Lang, Celia Rodd, Timothy Ramsay, David A Cabral, Peter B Dent, Janet E Ellsworth, Kristin M Houghton, Adam Huber, Roman Jurencak, Maggie Larche, Claire MA LeBlanc, Brian Lentle, MaryAnn Matzinger, Paivi M Miettunen, Kiem Oen, Johannes Roth, Claire Saint-Cyr, Rosie Scuccimarri, Nazih Shenouda, Leanne M Ward, Canadian STOPP Consortium

Systemic lupus erythematosus (SLE) is an autoimmune disorder resulting in inflammation of multiple organ systems. Although SLE is not common in children, it must be considered in the differential diagnosis of any child who has multisystem disease. The onset of SLE in children may be acute, with severe disease affecting one or more major organ systems, or it may be insidious, with intermittent symptoms of fever, malaise, rashes, arthritis, or pleuritis. SLE also must be considered if a child is found to have a positive antinuclear antibody (ANA) test result. Although almost all children who have SLE have a positive result, this laboratory test alone does not establish the diagnosis of SLE. Classification Criteria Eleven disease manifestations are included in the revised classification criteria for SLE established by the American College of Rheumatology (Table 1). These criteria were established as a classification tool to guide in selecting patients for clinical and laboratory studies rather than as a diagnostic system. Nevertheless, they have proven useful in the assessment of both pediatric and adult patients who may or may not have SLE. Epidemiology Childhood onset of SLE accounts for approximately 20% of all cases. The annual incidence in the United States is approximately 0.6 cases per 100 000 total population, with higher rates found in the African-American, Oriental, and Hispanic populations.

rhagic colitis in a nursing home. N Engl J Med 1987;317:1496500. 2. Karmali MA, Petrie M, Lira C, et al. The association between idiopathic hemolytic-uremic syndrome and infection by vetotoxin-producing Escherichia coli. J Infect Dis 1985;151:77582. 3. Butler T, Islam MR, Azad MAK, et al. Risk factors for development of hemolytic-uremic syndrome during shigellosis. J PEDIATR 1987;110:894-7. 4. Cimolai N, Carter JE, Morrison B J, Anderson JD. The progression of Escheriehia eoli O157:H7 enteritis to hemolyticuremic syndrome: anti-diarrheal agent use and age as risk factors? Clin Invest Med 1988;ll(suppl):C71. 5. Gransden WR, Damm MAS, Anderson JD, et al. Further evidence associating hemolytic-uremic syndrome with infection by verotoxin-producing Eseherichia cob O 157:H7. J Infect Dis 1986;154:522-4. 6. Ryan CA, Tauxe RV, Hosek GW, et al. Eseherichia coli O 157:H7 diarrhea in a nursing home: clinical, epidemiological, and pathological findings. J Infect Dis 1986;154:631-8. 7. Dupont HL, Hornick RB. Adverse effect of LomotiI therapy in shigellosis. JAMA 1973;226:1525-8. 8. Pittman FE. Adverse effect of Lomotil. Gastroenterology 1974;67:408-10. 9. Novak E, Lee JG, Seckman CE, et al. Unfavourable effect of atropine-diphenoxylate (Lomotil) therapy in lincomycin. caused diarrhea. JAMA 1976;235:1451-4. 10. Sprinz H. Pathogenesis of intestinal infections. Arch Pathol 1969;87:556-62. 11. Smith FW, Law DH, Nickel WF, et al. Fulminant ulcerative colitis with toxic dilation of the colon: medical and surgical management of eleven cases with observations regarding etiology. Gastroenterology 1962;421:233-43. 12. Johnson PC, Ericsson CD, Dupont HL, et al. Comparison of loperamide with bismuth subsalicylate for the treatment of acute travelers' diarrhea. JAMA 1986;255:757-60. 13. Kent TH, Formal SB, Labrec EH. Acute enteritis due to Salmonella typhimurium in opium-treated guinea pigs. Arch Pathol 1966;81:501-8. 14. Formal SB, Abrams GD, Schneider H, et al. Experimental Shigella infections VI: role of the small intestine in an experimental infection in guinea pigs. J Bacteriol 1963;85:119-25. 15. Cimolai N, Anderson JD, Morrison BJ. Antibiotics for Escherichia coti O157:H7 enteritis? J Antimicrob Chemother 1989;23:807-8. 16. Spika JS, Parsons JE, Nordenberg D, et al. Hemolytic-uremic syndrome and diarrhea associated with Escherichia coli O157:H7 in a daycare center. J PEDIATR 1986;109:287-91.

We report a case of Behcet's syndrome with myositis in a pediatric patient, emphasizing the importance of muscle involvement in the differential diagnosis of calf pain and swelling in Behcet's syndrome. A review of the English-language literature from 1965 to the present suggests that the clinical picture of Behcet's syndrome in children differs from that in adults, in that there is a lower frequency of ocular disease, and unusual manifestations appear to be more common.

Juvenile Dermatomyositis at Onset: Demographics, Clinical Characteristics, and Access to Care. ♦ 1844

To determine in a case-control study if new onset JDMS patients had increased 1) symptoms, 2)environmental conditions, 3) familial autoimmune diseases, or 4) antibody titers, compared with two control groups. The families of 80 JDMS and 62 JRA or healthy children had a structured interview. All childrens' sera were tested for antibody to Toxoplasma gondii, Herpes simplex virus (HSV), or Coxsackievirus B (CVB). Children with JDMS had symptoms 3 months before disease index date, p=0.013. JRA families had more rheumatoid arthritis (p=.0001) and pernicious anemia (p=.003) than those of JDMS or healthy children. Enteroviral titers were higher in JDMS children ≤ 7 years of age (84%) and controls (89%) than JRA (53%), suggesting an environmental exposure. Titers to T. gondii, HSV or CVB 1-6 were normal. Conclusion: Familial autoimmune disease, environmental factors, or antibody titers to T. gondii, HSV or CVB are not increased in JDMS. JDMS children have symptoms three months before disease index date; young patients have elevated enteroviral titers as do regional controls.