Bibliography January 2002- ANDRE M, HAGELBERG S, STENSTROM CH: Education in the management of juvenile chronic arthritis. Scandinavian Journal Of Rheumatology 2001, 30:323-327.
Organism:M. Andre, Department of Physical Therapy, Children's Hospital, Karolinska Hospital, SE-171 76 Stockholm
Abstract: Objective: The purpose was to evaluate changes in self-reported competencies following an education program among parents of children with juvenile chronic arthritis (JCA) and among adolescents with JCA. Methods: The self-reported, 24-item MEPS questionnaire was used for evaluating the program. Fifty-five parents and ll adolescents completed the questionnaire before, directly after, and four months after the eight-hour program. Results: Parents in the education program improved significantly concerning their self-reported competencies on medical, exercise, pain and social support issues, while the adolescents showed only minor improvement. The parents' positive development in some comparisons was also significant in relation to that of a non-educated group, whose responses remained mainly unchanged over the four months. Conclusion: Given the advantages of the education program indicated in the study, parent education should be a self-evident part of the treatment in JCA
Internet : marie.andre@home.se - ARGUEDAS O, FASTH A, ANDERSON-GARE B: A prospective population based study on outcome of juvenile chronic arthritis in Costa Rica. Rinsho Ganka 2002, 29:174-183.
Organism:Dr. O. Arguedas, Department of Immunology, National Children's Hospital, PO Box 1654-1000, San Jose
Abstract: Objective. To study the disease process and outcome in an unselected group of patients with juvenile chronic arthritis (JCA). Methods. From a population based study in San Jose, Costa Rica, 47 patients with JCA with disease onset from 1993 through 1995 were investigated after median duration of 4.1 yrs (range 2.9-4.9) (incidence group). Another 49 children with disease onset prior to 1993 and younger than 16 years of age on December 31, 1995 (cross sectional group) were also followed. Results. In the incidence group, 4/47 children changed subtype during the course of the disease. All did so within 2 years from disease onset, and the same observation was made in the cross sectional group. Uveitis was described at onset in a single case, and no child developed uveitis later. In patients from the incidence group in the process of being transferred to adult rheumatology clinics, 48% were still taking medication. Patients who had involvement of proximal interphalangeal (PIP) joints at onset had an increased risk of being active or stable at followup (RR 12.3, 95% CI 1.4-108.3). A higher chance of no continuing disease activity at followup was observed in children with oligoarticular disease than in the other subtypes (RR 2.8, 95% CI 1.2-6.9). Conclusion. Uveitis associated with antinuclear antibody positive JCA and psoriatic arthritis in Costa Rican children is uncommon, and the risk of developing uveitis remains low during the course of the disease. Involvement of PIP joints predicts an increased risk of continuing disease. The course of JCA in Costa Rican children is not milder than in Caucasian populations, since 48% of the patients showed persistent disease activity at the transition to adult care
Internet : oarguedas@hnn.sa.cr - BALCI S, AYPAR E, KASAPC c, TUYSUZ B, ARISOY N: An eleven-year-old female Turkish patient with progressive pseudorheumatoid dysplasia mimicking juvenile idiopathic arthritis [3]. Clinical And Experimental Rheumatology 2001, 19:759
Organism:Prof. S. Balci, Dept. of Clinical Genetics, Ihsan Dogramaci Children's Hospital, Hacettepe University, Kibris sok. 17/8, 06690 Kavaklidere Ankara - BERNTSON L, FASTH A, ANDERSSON-GARE B, KRISTINSSON J, LAHDENNE P, MARHAUG G, NIELSEN S, PELKONEN P, SVENSSON E: Construct validity of ILAR and EULAR criteria in juvenile idiopathic arthritis: a population based incidence study from the Nordic countries. International League of Associations for Rheumatology. European League Against Rheumatism. J.Rheumatol. 2001, 28:2737-2743.
Organism:Department of Pediatrics, Hospital of Falun, Sweden lillemorberntson@falunmailteliacom
Abstract: OBJECTIVE: New classification criteria (ILAR) have been proposed for juvenile idiopathic arthritis (JIA). They are more descriptive than those formerly used [American College of Rheumatology (ACR), European League Against Rheumatism (EULAR)], but require validation against classifications already in use. We validated the ILAR criteria in relation to the EULAR criteria in a prospective, incidence, and population based setting, and analyzed their feasibility. METHODS: Construct validity of ILAR and EULAR classification criteria refers to how closely the 2 instruments are related and how each of them operates in classifying subgroups/categories. Twenty doctors in 5 Nordic countries collected data from the incidence cases within their catchment areas during an 18 month period beginning July 1, 1997. Clinical and serological data from the first year of disease were collected. RESULTS: A total of 322 patients were included. Classification according to the ILAR criteria was possible in 321 patients; 290 patients had a disease duration > or = 3 months and were classified according to the EULAR criteria. One child could only be classified according to the EULAR criteria. Thus, 31/322 (9.6%) children were classified according to the ILAR criteria only. Forty-eight of 321 (15%) patients did not fit into any category and 6% (20/321) fulfilled criteria for2 categories. In the ILAR classification 5 out of 7 categories/subgroups have 2 to 5 specified exclusion criteria that highly discriminate the definition of each patient. In our study the exclusion criteria were fulfilled to only a small extent. CONCLUSION: The EULAR and ILAR criteria differ concerning the operational definitions of the subvariables involved, which complicates their comparison. By using ILAR rather than EULAR criteria the number of cases with juvenile arthritis increased by 10%, considering the first half-year after onset. The validity of the ILAR criteria is low since they often exclude patients from subgroup classification and the possibility of having more than one diagnosis is not negligible. The specified exclusion criteria for some of the subgroups are difficult to fulfill in clinical work and variables involved could be questioned with regard to their consistency
Internet : PM:11764226 - BLOOM BJ, OWENS JA, MCGUINN M, NOBILE C, SCHAEFFER L, ALARIO AJ: Sleep and its relationship to pain, dysfunction, and disease activity in juvenile rheumatoid arthritis. Rinsho Ganka 2002, 29:169-173.
Organism:Dr. B.J. Bloom, Hasbro Children's Hospital, Potter Building, 593 Eddy Street, Providence, RI 02903
Abstract: Objective. To determine what sleep abnormalities may exist in children with juvenile rheumatoid arthritis (JRA), and their relationship to pain, dysfunction, and disease activity. Methods. Twenty-five children with active JRA (11 pauciarticular, 9 polyarticular, 5 systemic) had their sleep assessed by parallel, validated patient and parent questionnaires (Sleep Self-Report, SSR, and Children's Sleep Habits Questionnaire, CSHQ). Disease activity was assessed by parent and physician global assessments (on a 5 point scale: 0 = no disease activity to 4 = very severe disease), erythrocyte sedimentation rate (ESR), and numbers of swollen and limited joints. Functional assessment was based on parental completion of the Juvenile Arthritis Functional Assessment Report (JAFAR). Pain was assessed by the average pain visual analog scale of the Varni Pediatric Pain Questionnaire. Results were compared to those from 45 healthy age and sex matched controls by Mann-Whitney U tests, and correlated with variables of JRA disease activity, function, and pain using Spearman correlations. Results. Patients with JRA had higher total score on the CSHQ (p < 0.0001), as well as subscales assessing night wakings, parasomnias, sleep anxiety, sleep-disordered breathing, and morning wakening/daytime sleepiness (p < 0.0001-0.05). There were no correlations between CSHQ scores and JRA disease activity or pain variables, but the total score on the SSR did correlate with pain (r = 0.56, p = 0.005). Conclusion. We conclude that sleep abnormalities are common in children with JRA, and are multidimensional - CHEN CY, TSAO CH, OU LS, YANG MH, KUO ML, HUANG JL: Comparison of soluble adhesion molecules in juvenile idiopathic arthritis between the active and remission stages. Ann.Rheum.Dis. 2002, 61:167-170.
Organism:Division of Allergy, Asthma, and Rheumatology, Department of Paediatrics, Chang Gung Children Hospital, Chang Gung University, Taoyuan, Taiwan
Abstract: OBJECTIVE: To determine the serum levels of soluble adhesion molecules in patients with juvenile idiopathic arthritis (JIA), and to determine whether the levels of these molecules differ between active disease and remission in the same JIA subtype, and whether differences in these levels exist between controls and the three JIA subtypes. METHODS: The serum levels of soluble E-selectin (sE-selectin) and soluble intercellular adhesion molecule-1 (sICAM-1) were determined by enzyme linked immunosorbent assay (ELISA) in 40 patients with JIA (12 systemic, 13 polyarticular, and 15 oligoarticular) who had active disease or were in clinical remission and 16 healthy controls. Differences in the levels of adhesion molecules of the same JIA subtype during different disease activity were determined by the paired t test, and differences between the disease and control groups were calculated by one way analysis of variance. A value p<0.01 was considered significant. RESULTS: During the same disease stage (active or in remission), systemic JIA was associated with a significantly higher sE-selectin level than the oligoarticular JIA subtype, whereas this was not found for sICAM-1. Although the mean levels of sE-selectin and sICAM-1 in active systemic and polyarticular JIA were higher than those in remission, this did not reach statistical significance. The levels of sE-selectin and sICAM-1 of the three JIA subtypes, in both the active stage and clinical remission, were still significantly higher than in normal controls. CONCLUSIONS: Systemic JIA is associated with a higher sE-selectin level than oligoarticular JIA both in active disease and in clinical remission. This may explain why the morbidity of systemic JIA is greater than that of oligoarticular JIA-namely, owing to increased endothelial cell activation. As significantly higher levels of sE-selectin and sICAM-1 were found in the active and remission stages of the three JIA subtypes compared with those in the control group, JIA may recur even when clinical remission has been achieved
Internet : PM:11796405 - CLEARY AG, MCDOWELL H, SILLS JA: Polyarticular juvenile idiopathic arthritis treated with methotrexate complicated by the development of non-Hodgkin's lymphoma. Arch.Dis.Child 2002, 86:47-49.
Organism:Royal Liverpool Children's Hospital NHS Trust, Eaton Road, Liverpool L12 2AP, UK gavincleary@talk21com
Internet : PM:11806884 - CUENDE E, VESGA JC, PEREZ LB, ARDANAZ MT, GUINEA J: Macrophage activation syndrome as the initial manifestation of systemic onset juvenile idiopathic arthritis [9]. Clinical And Experimental Rheumatology 2001, 19:764-765.
Organism:Dr. E. Cuende, Unidad de Reumatologia, Hospital Txagorritxu, Jose Atxotegi s/n, 01009 Vitoria
Internet : ecuende@htxa.osakidetza.net - DAGAN-FRIEDMAN BH, TARASIUK A, TAL A: [Impaired daytime functions in children with sleep disorders]. Harefuah 2001, 140:1204-8, 1227.
Organism:Division of Pediatrics, Unit of Sleep Research, Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University, Hanegev, Israel
Abstract: Sleep in adequate amount and quality is essential for normal child development. Sleep disorders may affect the childs daytime functions resulting in behavioral problems such as attention deficit, aggressiveness and hyperactivity. Recent reports have suggested that chronic sleep disturbance can cause neurocognitive deficits and impaired learning abilities. Obstructive sleep apnea syndrome, asthma, atopic dermatitis and juvenile rheumatoid arthritis--are common childhood diseases that can impair normal sleep. This article reviews the sleep characteristics in these diseases and the relation between sleep quality and the social and intellectual performance of the child during the day
Internet : PM:11789310 - DONN R, ZEGGINI E, SHELLEY E, OLLIER W, THOMSON W, ABINUN M, BECKER M, BELL A, CRAFT A, CRAWLEY E, DAVID J, FOSTER H, GARDENER-MEDWIN J, GRIFFIN J, HALL A, HALL M, HERRICK A, HOLLINGWORTH P, HOLT L, JONES S, POUNTAIN G, RYDER C, SOUTHWOOD T, STEWART I, VENNING H, WOO P, WYATT S: Lack of association between juvenile idiopathic arthritis and Fas gene polymorphism. Rinsho Ganka 2002, 29:166-168.
Organism:Dr. R. Donn, ARC Epidemiology Unit, Stopford Building, University of Manchester, Oxford Road, Manchester M13 9PT
Abstract: Objective. Juvenile idiopathic arthritis (JIA) is a complex genetic disease of autoimmune etiology. Fas is a molecule with a pivotal role in apoptosis and hence in immune regulation. Elevated transcriptional levels of Fas in the synovial fluid of patients with JIA suggest that it might be implicated in disease etiopathogenesis. We investigated whether a polymorphism in the Fas promoter region (-670) confers susceptibility to JIA. Methods. In this association study, 342 UK patients with JIA and 255 healthy individuals were genotyped for the polymorphism using polymerase chain reaction restriction fragment length polymorphism. Comparisons of the genotypic frequencies were made using chi-square analysis. Results. No statistically significant differences were found when the genotype frequencies of the -670 Fas polymorphism were compared between the JIA cases and the control panel. Similarly, no differences were seen between the JIA subgroups, or when the patients were divided on the basis of rheumatoid factor or antinuclear antibody positivity. Conclusion. The -670 polymorphism of Fas does not appear to be associated with susceptibility to JIA
Internet : rachelle@fsl.ser.man.ac.uk - EDELSTEN C, LEE V, BENTLEY CR, KANSKI JJ, GRAHAM EM: An evaluation of baseline risk factors predicting severity in juvenile idiopathic arthritis associated uveitis and other chronic anterior uveitis in early childhood. Br.J.Ophthalmol. 2002, 86:51-56.
Organism:Medical Ophthalmology Department, Great Ormond Street Hospital and Prince Charles Eye Unit, King Edward VII Hospital, Windsor, UK edelsten@easynetcouk
Abstract: BACKGROUND/AIMS: The clinical course for childhood chronic anterior uveitis can vary from mild, self limiting disease to bilateral blindness. The purpose of this study was to identify those risk factors at onset that predict disease severity. METHODS: A retrospective case note review of all patients with painless anterior uveitis diagnosed from 1982 to 1998. Patients were divided into two cohorts based on route of referral, diagnosis, and compliance with treatment. The standard cohort consisted of only those diagnosed from routine screening of juvenile idiopathic arthritis. RESULTS: Complications-cataract surgery, ocular hypertension treatment, and visual acuity <6/24. Remission: inactive uveitis on no topical treatment for >6 months. Results-163 patients were included. 34 patients (21%) developed at least one complication. The most significant predictor of complications was severe disease at onset (p = 0.001). Other factors included uveitis at the first examination (p = 0.034), membership of the non-standard cohort (p = 0.0001), non-oligoarticular disease (p = 0.02), and late onset arthritis (p = 0.024). Male sex was associated with increased complications in the standard cohort (p = 0.001). Factors predisposing to remission included membership of the standard cohort (p = 0.003), onset after 1990 (p = 0.016), white race (p = 0.015), mild disease onset (p = 0.003), and a long gap between arthritis and uveitis onset (p = 0.015). CONCLUSIONS: It is possible to characterise the severity of those with childhood chronic anterior uveitis at the onset of disease. The majority of patients remit without visually disabling complications. It may be possible to reduce the complication rate by targeting aggressive immunosuppression on high risk patients before complications develop
Internet : PM:11801504 - HASHKES PJ, FRIEDLAND O, UZIEL Y: New treatments for juvenile idiopathic arthritis. Isr.Med.Assoc.J. 2002, 4:39-43.
Organism:Department of Pediatrics and Pediatric Rheumatology Clinics, Rebecca Sieff Hospital, Safed, Israel
Internet : PM:11802310 - HOFER M, SAURENMANN T, BOLZ D, SAUVAIN MJ: Epidemiology of childhood arthritis in Switzerland. Soz.Praventivmed. 2001, 46:285-287.
Internet : PM:11759334 - HONKANEN V, LAHDENNE P: [Juvenile rheumatoid arthritis]. Duodecim 2000, 116:839-843.
Organism:Helsingin yliopisto, lasten ja nuorten klinikka ja HYKS:n lasten ja nuorten sairaala PL 281, 00029 HYKS
Internet : PM:11787127 - HULL RG: Guidelines for management of childhood arthritis. Rheumatology (Oxford) 2001, 40:1309-1312.
Organism:Queen Alexandra Hospital, Cosham, Portsmouth, Hampshire, PO6 3LY UK - ILOWITE NT: Current treatment of juvenile rheumatoid arthritis. Pediatrics 2002, 109:109-115.
Organism:N.T. Ilowite, Schneider Children's Hospital, 269-01 76th Ave, New Hyde Park, NY 11040
Abstract: Prognostic factors in juvenile rheumatoid arthritis (JRA) include polyarticular onset, polyarticular disease course, and rheumatoid factor positivity; in the systemic onset subtype, persistence of systemic features at 6 months after onset confers a worse prognosis. Timely diagnosis and appropriate aggressive treatment of patients with poor prognostic features improve quality of life and outcome. After nonsteroidal anti-inflammatory drugs, methotrexate is the most commonly used second-line agent. However, approximately one third of patients do not respond to methotrexate adequately. Randomized, placebo-controlled, clinical trials in patients with JRA are few, but one such trial with the tumor necrosis factor inhibitor etanercept shows that this drug is effective and well-tolerated. Other recently approved agents for rheumatoid arthritis, including infliximab, leflunomide, celecoxib, and rofecoxib, have not been adequately studied in pediatric patients, and the role of these agents in children with JRA remains to be determined
Internet : ilowite@lij.edu - JOHNSON K, GARDNER-MEDWIN J: Childhood arthritis: classification and radiology. Clin.Radiol. 2002, 57:47-58.
Organism:Department of Paediatric Radiology, Birmingham Children's Hospital, Birmingham, UK karljohson@bhamchildrenswmidsnhsuk
Abstract: Childhood arthritis has now been reclassified into a single internationally recognized entity of juvenile idiopathic arthritis (JIA). Radiology provides an important role in the management of JIA, in helping in the differential diagnosis, monitoring disease progression and detecting complications. Traditionally, plain radiographs have been the imaging investigation of choice but magnetic resonance imaging (MRI) and ultrasound are now providing a more effective and safer alternative. The appropriate use of sequences in MR imaging is important in the early detection of joint abnormalities in JIA
Internet : PM:11798203 - KIETZ DA, PEPMUELLER PH, MOORE TL: Therapeutic use of etanercept in polyarticular course juvenile idiopathic arthritis over a two year period. Ann.Rheum.Dis. 2002, 61:171-173.
Organism:Division of Rheumatology, Departments of Internal Medicine and Pediatrics, St Louis University Health Sciences Center, St Louis, MO, USA
Abstract: OBJECTIVE: To analyse the treatment response to etanercept in patients with polyarticular course juvenile idiopathic arthritis (JIA). METHODS: 22 patients with polyarticular course JIA (19 females, three males; mean age 13.9 years; mean disease duration 6.3 years; 15 with polyarticular onset, seven with systemic onset, one with residual systemic activity; eight rheumatoid factor positive; eight with erosive disease) were treated with etanercept for up to 24 months. Etanercept was given subcutaneously at 0.4 mg/kg twice a week. Treatment response was ascertained in an open prospective study. RESULTS: All patients showed impressive clinical improvement, with a decrease in swollen joint count by an average of 10.1 joints (mean of 49% decrease), a decrease in tender joint count by 9.3 joints (mean of 94%), and decrease in total joint count by 11.2 joints (mean of 48%). Duration of morning stiffness decreased to less than 10 minutes. Furthermore, haemoglobin concentration increased on average by 14 g/l (mean of 15.3%) and packed cell volume increased by 0.035 (mean increase of 12%), and erythrocyte sedimentation rate decreased on average by 42.8 mm/1st h (mean decrease of 64%). No major side effects were noted. CONCLUSION: Etanercept continues to be clinically effective and well tolerated in patients with polyarticular course JIA over a two year period
Internet : PM:11796406 - KORKMAZ C, OZDOGAN H, KASAPCOPUR O, YAZICI H: Acute phase response in familial Mediterranean fever. Ann.Rheum.Dis. 2002, 61:79-81.
Organism:Department of Rheumatology, Medical School of Osmangazi University, Eskisehir, Turkey
Abstract: OBJECTIVE: To test the hypothesis that not all acute phase reactants respond in the same way during attacks of familial Mediterranean fever (FMF) and that there is a subclinical acute phase response (APR) in a proportion of patients during the interval between attacks. METHODS: Blood and urine samples were obtained from 49 patients with FMF during an attack and the attack-free period that followed, to test for erythrocyte sedimentation rate, C reactive protein (CRP), fibrinogen, white blood cell count, platelet count, factor VIII related antigen, haptoglobin, protein electrophoresis, ferritin, proteinuria, and haematuria. Control groups comprised 29 patients with juvenile idiopathic arthritis, 10 patients with various infectious diseases, and 19 healthy subjects. RESULTS: A marked APR was seen during the FMF attacks which was comparable with that obtained in the diseased control groups. CRP was the only acute phase protein that was raised during all attacks. Neither thrombocytosis nor an increase in ferritin levels (except one) was noted in any attack. Serum albumin levels remained unchanged. In two thirds of the patients with FMF a continuing APR was seen in between the attacks. CONCLUSION: Platelet, ferritin, and albumin responses are not part of the significant APR seen during short lived attacks of FMF, and inflammation continues in about two thirds of the patients during an attack-free period
Internet : PM:11779767 - KURAHARA DK: Complementary medicine techniques to help reduce muscular pain in the pediatric rheumatic illnesses. Hawaii Med.J. 2001, 60:289
Organism:Kapi'olani Children's Specialty Center, John A Burns School of Medicine, University of Hawaii at Manoa, USA
Internet : PM:11797494 - LEHMAN TJ, STRIEGEL KH, ONEL KB: Thalidomide therapy for recalcitrant systemic onset juvenile rheumatoid arthritis. J.Pediatr. 2002, 140:125-127.
Organism:Division of Pediatric Rheumatology, Hospital for Special Surgery, and the Department of Pediatrics, Sanford Weill Medical Center of Cornell University, New York
Abstract: Systemic onset juvenile rheumatoid arthritis unresponsive to nonsteroidal anti-inflammatory drugs may be controlled with corticosteroids, but these drugs have significant side effects. We report 2 steroid-dependent children with systemic onset juvenile rheumatoid arthritis who did not respond to multiple nonsteroidal anti-inflammatory drugs, methotrexate, azathioprine, cyclosporine, and etanercept. Both children had significant improvement with thalidomide therapy
Internet : PM:11815776 - MANZOTTI F, ORSONI JG, ZAVOTA L, CIMINO L, ZOLA E, BONAGURI C: Autoimmune uveitis in children: Clinical correlation between antinuclear antibody positivity and ocular recurrences. Rheumatol.Int. Berlin 2002, 21:127-132.
Organism:J.G. Orsoni, Institute of Ophthalmology, University of Parma, Via Gramsci 14, 43100 Parma
Abstract: Objective. The aim of this study was to identify the correlation between antinuclear antibody (ANA) titre and the onset and clinical course of uveitis in children with juvenile idiopathic arthritis (JIA) or without any other systemic autoimmune disease, i.e., idiopathic uveitis (IU). Methods. Twenty-two patients affected by uveitis were examined. Ten had JIA-associated uveitis, 12 had IU. Follow-up ranged from 7 to 101 months. The ANA were titrated three times per year and additionally in case of ocular recurrences. All patients were treated with immunosuppressive drug combination therapy (IDCT). Results. JIA-associated uveitis: ocular recurrences were noted in three ANA-positive patients and in one ANA-negative patient. IU uveitis: ocular recurrences were noted in one ANA-positive and in one ANA-negative patient. No significant rise in ANA titre was noted in either group during uveitis recurrence. Conclusions. (1) ANA had no value in predicting the recurrence of uveitis. (2) IDCT does not influence ANA production
Internet : jorsoni@unipr.it - MAURAS N: Growth hormone therapy in the glucocorticosteroid-dependent child: Metabolic and linear growth effects. Horm.Res. 2001, 56:13-18.
Organism:Dr. N. Mauras, Division of Endocrinology, Nemours Children's Clinic, 807 Children's Way, Jacksonville, FL 32207
Abstract: Pharmacological doses of glucocorticosteroids given chronically are associated with a variety of negative side effects which impact the prolonged use of these potent anti-inflammatory agents. They have catabolic effects on protein, resulting in poor tissue healing, an increased incidence of infections and accelerated bone loss. Insulin resistance to both hepatic and peripheral tissues is a common consequence of chronic steroid use, leading at times to impaired carbohydrate metabolism. Steroids affect both the release and the effects of growth hormone (GH) at the target sites, hence becoming functional GH antagonists. When administered to growing children the side effects of glucocorticosteroid treatment are further compounded by a potent and significant suppression of linear growth. Ample experimental and clinical data support a role for GH therapy in counteracting some of the effects of glucocorticosteroids. Using isotope dilution methods we have previously shown that both GH and insulin-like growth factor (IGF)-I can decrease the protein wasting effects of prednisone administration in man. IGF-I has also been shown to enhance type I collagen formation in hydrocortisone-treated human osteo-blasts. GH (through IGF-I) significantly enhances linear growth; thus, in states of 'functional' GH deficiency, such as that observed in chronic steroid use, GH may also have a potentially beneficial effect. Studies in children on chronic prednisone doses with cystic fibrosis, chronic renal failure or juvenile rheumatoid arthritis have all shown beneficial effects on linear growth after prolonged GH therapy. Data from a recent study of ours using GH in children with steroid-dependent inflammatory bowel disease showed that GH treatment was associated with increased lean body mass, decreased adiposity and increased linear growth. Marked increases in IGF-I concentrations and in kinetic measures of bone calcium accretion (using calcium tracers) were also observed, without any deterioration of disease activity scores or carbohydrate tolerance. In conclusion, GH therapy may play a role in the treatment of children on chronic steroids both as a growth promoting agent and as an anabolic agent on whole body protein and bone. Longer term studies will be needed to better define the safety and efficacy of this approach. Copyright (c) 2001 S. Karger AG, Basel
Internet : nmauras@nemours.org - MEAZZA C, TRAVAGLINO P, PIGNATTI P, MAGNI-MANZONI S, RAVELLI A, MARTINI A, DE BENEDETTI F: Macrophage migration inhibitory factor in patients with juvenile idiopathic arthritis. Arthritis Rheum. 2002, 46:232-237.
Organism:University of Pavia, IRCCS Policlinico San Matteo, Italy
Abstract: OBJECTIVE: To evaluate serum and synovial fluid (SF) levels of macrophage migration inhibitory factor (MIF) and in vitro MIF production by peripheral blood mononuclear cells (PBMCs) in patients with juvenile idiopathic arthritis (JIA). METHODS: Serum, SF, and culture supernatant levels of MIF were measured by enzyme-linked immunosorbent assay. Production of MIF by PBMCs was investigated by culturing PBMCs in the absence or presence of 2 different concentrations of concanavalin A. RESULTS: Serum MIF levels were increased in patients with JIA, and the highest levels were present in patients with systemic-onset JIA. In systemic-onset JIA, serum levels of MIF correlated with the persistence of systemic features and the number of active joints. PBMCs from patients with systemic-onset JIA, when cultured under unstimulated conditions or at suboptimal stimulation, released higher amounts of MIF compared with those from patients with oligoarticular-onset JIA or healthy controls. MIF levels in the SF of patients with systemic-onset JIA were significantly higher than those in patients with oligoarticular-onset JIA. In individual joints, in both systemic-onset JIA and oligoarticular-onset JIA, SF MIF levels were inversely correlated with the duration of the clinical remission induced by intraarticular administration of triamcinolone hexacetonide. CONCLUSION: MIF appears to be a relevant cytokine in the pathogenesis of JIA, particularly in systemic-onset JIA
Internet : PM:11817596 - MISEROCCHI E, BALTATZIS S, EKONG A, ROQUE M, FOSTER SC: Efficacy and safety of chlorambucil in intractable noninfectious uveitis: The Massachusetts eye and ear infirmary experience. Ophthalmology 2002, 109:137-142.
Organism:Dr. E. Miserocchi, Ocular Immun. and Uveitis Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, 243 Charles St., Boston, MA 02114
Abstract: Purpose: To report our experience with the use of chlorambucil for otherwise treatment-resistant uveitis and to assess its safety and efficacy. Design: Noncomparative interventional case series. Participants: Twenty-eight patients with intractable noninfectious uveitis. Methods: We reviewed the records of 28 patients (56 eyes) with chronic noninfectious uveitis who were treated with chlorambucil from 1987 to 2000. Diagnoses included Adamantiades-Behc(cedil)et's disease (ABD) (7 patients), juvenile rheumatoid arthritis (JRA)-associated uveitis (10 patients), pars planitis (2 patients), sympathetic ophthalmia (1 patient), idiopathic uveitis (6 patients), Crohn's disease (1 patient), and HLA-B27-associated uveitis (1 patient). All patients were refractory to other immunomodulatory therapy and systemic steroids. The median duration of treatment with chlorambucil was 12 months (range, 4-50 months), whereas the median daily dosage was 8 mg (range, 4-22 mg). Patients were followed for a median follow-up period of 46 months (range, 4-166 months) after chlorambucil treatment was begun and continued to be followed for relapse after cessation of therapy. Main Outcome Measures: Visual outcome, response to treatment, treatment-related side effects, drug dosage, previous and final treatment, discontinuation of systemic corticosteroids. Results: Chlorambucil was discontinued in seven patients because of side effects: two females had temporary amenorrhea develop, two patients had unacceptable gastrointestinal intolerance, one patient had infection, and 2 patients had progressive leukopenia. Nineteen patients (68%) showed positive clinical response to the treatment, four (14%) initially responded then relapsed after discontinuation of the drug, three patients with ABD had improvement of ocular disease but worsening of systemic symptoms, and two had persistent inflammation. Visual acuity was improved in 24 eyes (43%), stable in 22 (39%), and worsened in 10 eyes (18%). Systemic prednisone was successfully discontinued in 19 of the 28 patients (68%), and 14 patients were free of inflammation at the end of follow-up without any systemic medication. Conclusions: Chlorambucil can be a safe and effective alternative for preserving vision in patients with otherwise treatment resistant uveitis. (c) 2002 by the American Academy of Ophthalmology - OBATA T, SATO A, MINAMI T, MIZUKOSHI T, MURAKAMI R, YAMAMOTO H, TAKEKAWA M, SUGAYA T, TAKAHASHI H, IMAI K: [Juvenile rheumatoid arthritis associated with aortitis syndrome]. Nippon Naika Gakkai Zasshi 2001, 90:2475-2476.
Organism:First Department of Internal Medicine, Sapporo Medical University School of Medicine, Sapporo
Internet : PM:11808183 - OESTREICH AE: Mega os trigonum in progressive pseudorheumatoid dysplasia. Pediatr.Radiol. 2002, 32:46-48.
Organism:Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229-3039, USA, oestreichae@chmccorg
Abstract: BACKGROUND: Progressive pseudorheumatoid dysplasia (PPRD), a noninflammatory condition, needs to be differentiated diagnostically from juvenile rheumatoid arthritis (JRA). OBJECTIVE: Demonstration of an unusually large and often early-appearing os trigonum helps distinguish PPRD from JRA. MATERIALS AND METHODS: Ankle images in four children with PPRD were reviewed. RESULTS: The os trigonum was abnormally enlarged in all PPRD subjects and was shown to have appeared or fused earlier than normal in two subjects. CONCLUSION: A large and early os trigonum ossification helps differentiate PPRD from JRA
Internet : PM:11819063 - PALOSUO T, NISSINEN R, SAVOLAINEN A, SAILA H, AHO K: Antibody markers in patients with juvenile idiopathic arthritis: Two comments and a reply. Anti-filaggrin antibody in patients with juvenile idiopathic arthritis [6]. Clinical And Experimental Rheumatology 2001, 19:762
Organism:Dr. T. Palosuo, National Public Health Institute, Mannerheimintie 166, FIN-00300 Helsinki
Internet : timo.palosuo@ktl.fi - REIFF A, ZASTROW M, SUN B-C, TAKEI S, MITSUHADA H, BERNSTEIN B, DURDEN DL: Treatment of collagen induced arthritis in DBA/1 mice with L-asparaginase. Clinical And Experimental Rheumatology 2001, 19:639-646.
Organism:Dr. A. Reiff, Childrens Hospital Los Angeles, Division of Rheumatology, 4650 Sunset Boulevard, Los Angeles, CA 90027
Abstract: Objective. To evaluate the safety and efficacy of L-asparaginase as an immunosuppressive agent in a mouse model of rheumatoid arthritis. Methods. Male DBA/1 mice with collagen-induced arthritis (CIA) were treated at different intervals with various doses of native and pegylated L-asparaginase from E. coli. The mice were observed for 4 weeks during which time arthritis was scored. Outcome parameters included effect on severity and progression of established arthritis as well as prevention of disease. In addition, X-rays from the affected joints were obtained for comparison. Results. Both native L-asparaginase at a dose of 50 IU/injection intraperitoneally three days a week and pegylated asparaginase (PEG-L-asparaginase) at a dose of 25 IU/injection twice a week, significantly reduced the mean arthritic score (MAS) in mice with established arthritis (p < 0.001 for PEG-L-asparaginase). When native L-asparaginase was administered before the onset of arthritis (days 14-post immunization) the number of mice developing arthritis as well as the number of arthritic paws and the severity of arthritis in the treatment group were significantly decreased (p < 0.0001). Significant differences were found in the X-ray evaluation between treated and control mice. None of the animals died due to drug related events or showed signs of asparaginase induced toxicity. Conclusion. Our data provide the first direct evidence that L-asparaginase is a potent antiarthritic agent and may represent an effective second line agent for future treatment studies in juvenile and adult rheumatoid arthritis
Internet : reiff@hsc.usc.edu - ROSENBAUM JT, SMITH JR: Uveitis and juvenile arthritis. Br.J.Ophthalmol. 2002, 86:1-2.
Internet : PM:11801490 - TAKANO T, OHNO M, TAKEUCHI Y, MANDAI R, YOSHIDA S: Cytotoxic edema and interleukin-6 in hypertensive encephalopathy. Pediatr.Neurol. 2002, 26:71-73.
Organism:Department of Pediatrics, Shiga University of Medical Science, Seta-Tsukinowa, Otsu, 520-2192, Shiga, Japan
Abstract: We report a female, 10 years of age, with juvenile rheumatoid arthritis accompanied by hypertensive encephalopathy. The patient developed a cytotoxic brain lesion, as revealed by the high signal intensity on diffusion-weighted magnetic resonance imaging, which corresponded to the hypoperfusion area on single-photon emission computed tomography scan using 99mTc-ethylcysteinatedimer. Cerebrospinal fluid interleukin-6 activity was elevated when the hypertensive encephalopathy revealed active central nervous system disease, and its activity decreased when the encephalopathy recovered from the central nervous system manifestations. We speculated that the cytotoxic edema and associated parenchymal damage in hypertensive encephalopathy were closely related to the intrathecal overproduction of interleukin-6
Internet : PM:11814741 - TORTOLANI PJ, MCCARTHY EF, SPONSELLER PD: Bone mineral density deficiency in children. J.Am.Acad.Orthop.Surg. 2002, 10:57-66.
Organism:Department of Orthopaedic Surgery, Johns Hopkins Hospital, 601 North Caroline Street, Baltimore, MD 21287-0881, USA
Abstract: With the development of improved diagnostic and treatment options, reduced bone mineral density in children is receiving increased attention. The etiology of osteopenia in healthy children is multifactorial and incompletely understood, but poor calcium intake during the adolescent growth spurt may be an important (and potentially reversible) factor. Other clinically relevant causes of reduced bone mineral density in children include osteogenesis imperfecta, rickets, juvenile rheumatoid and other chronic arthritides, osteopenia associated with neuromuscular disorders, and idiopathic osteoporosis. To provide effective treatment, it is important to understand the process of normal skeletal mineralization, the techniques of bone mineral density measurement, the pathophysiology of osteopenia, and the evaluation and treatment options for the general pediatric population as well as for patients with specific pediatric disorders
Internet : PM:11809051 - YUNG RL: Etanercept Immunex. Curr.Opin.Investig.Drugs 2001, 2:216-221.
Organism:University of Michigan Medical Center, Division of Rheumatology, Ann Arbor 48109-1065, USA ryung@medumichedu
Abstract: Immunex has developed and launched etanercept, a soluble TNF receptor (TNFR) fusion protein, for the treatment of early and moderate to severely active rheumatoid arthritis (RA). Etanercept was launched as a first-line agent in the US for the treatment of moderate-to-severe active RA in June 2000 [375481]. It can also be used in conjunction with methotrexate (MTX) in patients who do not respond adequately to MTX alone [303266], [310436]. It was launched in the EU in November 2000 [388846]. Enbrel was also launched for the treatment of polyarticular-course juvenile RA (JRA) patients who have an inadequate response to one or more disease-modifying antirheumatic drugs (DMARDs) in May 1999. Additionally, it is in phase III trials for psoriatic arthritis and a BLA filing for this indication is expected for the first half of 2001 [364948]. Etanercept was launched in the US in November 1998, for the treatment of moderate-to-severe RA in patients with inadequate responses to one or more DMARDs, or in combination with MTX in patients who do not respond adequately to MTX alone [306175]. The drug was subsequently approved by the US FDA for use as a first-line therapy to treat patients with moderately to severely active RA [375481]. In February 2000, Wyeth Europe received clearancefor etanercept in 15 EU countries by the EMEA for the treatment of active arthritis in adults when the response to DMARDs has been inadequate [354844]. It has since been launched in the UK (June 2000) [388840], and by October 2000 had been launched in all EU member states [388846]. In November 1998, the company filed a supplemental BLAfor the treatment of children and teenagers with moderately to severely active polyarticular course JRA. In May 1999, etanercept was approvedfor this indication by the US FDA and approvedfor this indication in Europe in February 2000 [307061], [310436], [326379]. The increasing understanding of the role of TNF in a number of other diseases has led to its clinical assessment in these areas. Following positive clinical results in phase II studies [317562], [315793], (320666], (359789], (373980] in patients with chronic heart failure, etanercept entered phase III trials for this indication in June 1999 [330068], and a BLA filing for this indication is expected in 2003 [396110]. Additionally, Immunex initiated a phase III trial of etanercept in psoriatic arthritis in March 2000, and as of May 2000, the company was planning a BLA filing for this indication in the first half of 2001 [364948]. An open-label trialfor the treatment of Crohn's disease is in progress in Belgium [367,039], and results from this trial were presented at Digestive Disease Week in May 2000 [379907]. While WO-09103553 claims the recombinant human receptor, the fusion protein consisting of the etanercept domain and the immunoglobulin region was disclosed in WO-09406476. In February 1997, US-05605690 was issued to Immunex for methods of using etanercept to treat diseases mediated by TNF. The patent also claims methods of using recombinant etanercept to decrease the levels of TNF in RA patients [235456]. In June 1999, Immunex strengthened its patent estate covering the product with a patent licensing agreement for Genentech's immunoadhesin patents covering the product [327250]. A royalty agreement with Serono SA and Immunex on sales of etanercept was agreed in 1999. The agreement reflected the strength of Ares-Serono's intellectual property status [352813]. In June 1999, Lehman Brothers predicted Immunex's sales at US $300 million in 1999, rising to peak annual sales of US $1.5 billion [328701]. Salesfor the drug's first full quarter on the market in 1999 were US $59.7 million [330068]. By November 1999 the drug had made sales of US $500 million; Immunex expects the drug will generate over US $2 billion in annual sales by 2004 [353185]. In September 2000, Merrill Lynch reported that if sales of the drug continue at the present rate then it is likely that demand will temporarily outstrip supply in 2001. Resolution of the supply issue is expected by 2002. Also in September 2000, Merrill Lynch lowered their estimate of ENBREL sales in 2001 from US $1 billion to $927 million. In the long-term, Merrill Lynch believe that the drug has the potential to exceed US $5 billion in sales in the US [382577]
Internet : PM:11816834