Bibliography February 2002- ALSAEID K, HAIDER MZ, KAMAL H, SRIVASTVA BS, AYOUB EM: Prevalence of human leukocyte antigen (HLA) DRB1 alleles in Kuwaiti children with juvenile rheumatoid arthritis. Eur.J.Immunogenet. 2002, 29:1-5.
Abstract: The prevalence of human leukocyte antigen (HLA) DR alleles has been determined in 69 Kuwaiti Arab children with juvenile rheumatoid arthritis (JRA) and compared to that in 212 ethnically matched normal healthy controls using a PCR-sequence specific primers (PCR-SSP) method. A very high incidence of DR3 was detected in JRA patients compared to the controls (P < 0.0001, RR = 2.235). The high incidence of HLA-DR3 in JRA patients was accounted for mainly by an excess of DRB1*0307 (P < 0.05, RR = 3.072) and DRB1*0308 (P < 0.009, RR = 2.663) compared to the controls. Moreover, DR3 was more prevalent when patients with ANA-positive JRA were analysed separately; 73% compared to 58% for the whole JRA patient group. The frequency of DR1 was also higher in the JRA group compared to controls (P = 0.019, RR = 3.585). Although the incidence of some alleles was higher in the control group (DR13 and DR7), none reached a statistically significant level. All the patients with iridocyclitis had either a DR1 or DR3 allele, except for one child. The frequency of DRB1*03 was found to be much higher in the polyarticular subtype of Kuwaiti JRA cases compared to the oligoarticular subgroup and the controls. Also, a non-significant increase in the frequency of the DRB1*04, *11 and *15 alleles was detected in the polyarticular subtype of the Kuwaiti JRA cases compared to the controls
Internet : PM:11841481 - ANDRE M, HAGELBERG S, STENSTROM CH: Education in the management of juvenile chronic arthritis. Changes in self-reported competencies among adolescents and parents of young children. Scand.J.Rheumatol. 2001, 30:323-327.
Organism:Department of Women and Child Health, Karolinska Institutet, Stockholm, Sweden marieandre@homese
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 11 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 : PM:11846049 - ARCHENHOLTZ B, NORDBORG E, BREMELL T: Lower level of education in young adults with arthritis starting in the early adulthood. Scand.J.Rheumatol. 2001, 30:353-355.
Organism:Department of Rheumatology, Sahlgrenska University Hospital, Sahlgrenska University Hospital, Goteborg Sweden birgithaarchenholtz@vgregionse
Abstract: An appropriate education may lead to less work disability in patients with arthritis. The aim of the study was to determine the educational level in two groups of young adults with arthritis. Patients with juvenile arthritis ( JA, n=32) and patients with early adult onset of arthritis (EA, n=47) were examined with the Quality of Life Scale (QOLS) and a questionnaire concerning education and profession counselling. Comparisons with a reference group (n=95) from the general population were made. The EA group had lower level of education (p<0.01), compared to the reference group. Among the EA patients, 62% had not discussed their choice of occupation with anybody, compared to 19% in the JA group. The educational level was lower in patients with rheumatic disease starting in early adulthood. Educational issues and counselling should be focused on the care of young adults with arthritis
Internet : PM:11846054 - ARGUEDAS O, FASTH A, ANDERSSON-GARE B: A prospective population based study on outcome of juvenile chronic arthritis in Costa Rica. Rinsho Ganka 2002, 29:174-183.
Organism:Department of Immunology, National Children's Hospital, San Jose Costa Rica^E-Mail: oarguedas@hnn.sa.cr
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 - 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:Hasbro Children's Hospital, 593 Eddy Street, Potter Building, Suite 200, Providence, RI, 02903 USA
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 multi-dimensional - BULTINK IE, LEMS WF, DIJKMANS BA, VAN SOESBERGEN RM, LINDEMAN J: Severe aortic regurgitation in RF positive polyarticular JIA. Ann.Rheum.Dis. 2002, 61:282-283.
Internet : PM:11830447 - CHEN C-Y, TSAO C-H, OU L-S, YANG M-H, KUO M-L, HUANG J-L: Comparison of soluble adhesion molecules in juvenile idiopathic arthritis between the active and remission stages. Annals of the Rheumatic Diseases 2002, 61:167-170.
Organism:Dr. J.-L. Huang, Division of Allergy, Department of Paediatrics, Chang Gung Children's Hospital, 5 Fu-Hsin Street, Kweishan, Taoyuan
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 : long@adm.cgmh.org.tw - DANNAWI H: T cell leukemia with 5q chromosomal deletion in a patient with juvenile rheumatoid arthritis. Blood 2001, 98:186b
Organism:Pediatrics, MCCG, Macon, GA USA
Abstract: An 11 year old white male was referred for evaluation of Pancytopenia. The patient had a history of JRA since 15 months of age. Initially, he was treated Naprosyne, then with Methotrexate 10mg/week and Prednisone 5mg every other day. The Physical exam was remarkable for the presence of left Supraclavicular node. Hb7.1 Hct20.8, Wbcl.96 Platelet 29000. Chest Xray showed ant.mediastinal mass. Bone marrow aspiration showed involvement with A.L.L. Flow-cytometri showed ccd3+, cd2+, cd7+, cd34+, cd4-, cd5-, cd13+, cd56+. The patient was started on Vancristine Daunomycine predisone and asparaginase. One week later a bone marrow was done and showed no reduction of blast. Another course of treatment was given and one week later another bone marrow was done and showed no reduction of blasts. The treatment was changed to Idarubicine 10mg/M2*3 days and Arac100mg/m2 CI*7/days. Remission was obtained. Cytogenetic studies revealed for the presence of 5q deletion. the patient had a Histocompatible sister and underwent bone marrow transplantation. THe 5q delition has been reported in myelodysplastic syndrome, in de novo A.M.L., and in leukemia and mylodysplastic syndrome secondary to cytotoxic therapy. We speculate that the patient had myelodysplasia and evolved into leukemia or whether the rheumatoid arthritis had developed on the basis of the immune abnormalities of MDS. Cellular immune system dysfunction leads to alteration of the micro enviroment of the BM via abnormally regulated cytokan and other intracellular messenger - DIKENSOY O, BAYRAM N, BINGOL A, FILIZ A: Bronchiolitis obliterans in a case of juvenile rheumatoid arthritis presented with pneumomediastinum. Respiration 2002, 69:100-102.
Organism:Department of Pulmonary Diseases, Gaziantep University, School of Medicine, Gaziantep, Turkey dikensoy@yahoocom
Abstract: We present a case of bronchiolitis obliterans associated with juvenile rheumatoid arthritis in whom pneumomediastinum was the presenting manifestation. Diagnosis of bronchiolitis obliterans was made on the basis of the clinical history, pulmonary function tests and high-resolution computerized tomography scan findings. Pneumomediastinum resolved in a few days following high-dose nasal oxygen. This case shows that bronchiolitis obliterans can occur in patients with juvenile rheumatoid arthritis. Complicating pneumomediastinum in such cases should be considered as an indication of underlying bronchiolitis obliterans
Internet : PM:11844973 - DONN R, ZEGGINI E, SHELLEY E, OLLIER W, THOMSON W, BRITISH PAEDIATRIC RHEUMATOLOGY STUDY GROUP: Lack of association between juvenile idiopathic arthritis and Fas gene polymorphism. Rinsho Ganka 2002, 29:166-168.
Organism:ARC Epidemiology Unit, University of Manchester, Oxford Road, Stopford Building, Manchester, M13 9PT UK^E-Mail: rachelle@fs1.ser.man.ac.uk
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 - 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. British Journal of Ophthalmology 2002, 86:51-56.
Organism:Ophthalmology Department, Ipswich Hospital, Heath Road, Ipswich, IP4 5PD UK^E-Mail: edelsten@easynet.co.uk
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 - FIETTA P, MANGANELLI P: Adult onset Still's disease. Minerva Med. 2002, 93:27-39.
Organism:Unita Operativa di Reumatologia e Medicina Interna, Dipartimento Osteo-Articolare, Azienda Ospedaliera Universitaria, Parma, Italy
Abstract: Adult onset Still's disease (AOSD), the adult variant of the systemic form of the juvenile rheumatoid arthritis, is an uncommon disorder of unknown origin. Although the pathogenesis has not yet been clarified, an immunologically mediated inflammation occurs in active AOSD. High spiking fever, evanescent maculo-papular skin rash, arthralgias/arthritis, neutrophilic leukocytosis, negative rheumatoid factor and antinuclear antibodies, as well as a marked hyperferritinemia are the major features of AOSD. Sore throat, lymphadenopathies, hepato-splenomegaly, abdominal pain, polyserositis, respiratory distress syndrome, multiple organ dysfunction and disseminated intravascular coagulation may also occur. The clinical course of AOSD is extremely variable and unpredictable and can be divided into three main patterns: a self-limited or monocyclic pattern, a polycyclic or intermittent course, with one or more flares of the disease and complete remission among the episodes, and a chronic course, characterized by persistently active disease, usually due to a chronic, destructive arthritis. Since there are not pathognomonic laboratory parameters or histological findings, the diagnosis of AOSD requires the exclusion of infectious, malignant and autoimmune disorders. Some sets of criteria for classification have been proposed, but so far not validated. The prognosis of AOSD is usually considered relatively benign, although a destructive arthritis may cause severe disability and the multisystemic life-threatening complications of the disease may determine a fatal outcome. Treatment usually consists in nonsteroidal anti-inflammatory drugs and corticosteroids, but a more aggressive approach with disease modifying antirheumatic or immunosuppressive drugs may be required
Internet : PM:11850612 - FIGUEIRA AS: Fibromyalgia syndrome. Revista Brasileira de Medicina 2001, 58:215-224.
Organism:S. Figueira Antonio, Encarregado Ambulatorio Patol. Vert., Servico de Reumatologia, Hosp. Serv. Publ. Estadual Sao Paulo, Sa(tilde)o Paulo
Abstract: In the introduction the author discuss the condition nomenclature, following the show the definition and epidemiological data; the subdivision (primary, secondary, regional, elderly and infant-juvenile fibromyalgia) is presented, and followed by the syndrome clinical picture and the diagnostic features in a detailed basis. Relating to the differential diagnosis, the author point out that the syndrome is an exclusion diagnostic, among others, with the myofascial syndrome, rheumatic polymialgia, rheumatoid arthritis, Sjogren syndrome, systemic erithematous lupus, with the possibility to being coexisting with these conditions. The pathophysiology is also presented in details, and in addition the author shows the treatment and its difficulties, classifying the topic in: general measures, non-pharmacologic modalities (cardiovascular fitness, physical exercise, biofeedback, eletroacupunture, hypnotherapy and cognitive behavioral therapy, pharmacologic therapy (analgesics, anti-inflammatory, opioids agents, tryciclic antidepressant serotonin reuptake inhibitors, muscular relaxants), local infiltrations and other medicinal agents - GRATWOHL A, PASSWEG J, GERBER I, TYNDALL A: Stem cell transplantation for autoimmune diseases. Bailliere's Best Practice and Research in Clinical Haematology 2001, 14:755-776.
Organism:Dr. A. Gratwohl, Division of Haematology, Department of Internal Medicine, Kantonsspital Basel, CH-4031 Basel
Abstract: Much progress has been made in the field of haemopoietic stem cell transplants (HSCTs) for severe autoimmune disorders. Theoretical considerations, animal data and anecdotal evidence suggested some time ago that intensive immunoablation followed by autologous HSCT could restore normal immune reactivity in patients with severe autoimmune disorders. Based on a concept statement issued in 1995, two European societies, the European League Against Rheumatism (EULAR) and the European Group for Blood and Marrow Transplantation (EBMT) began collecting phase I/II trial data in an international collaborative network. Sufficient information from more than 350 patients allows a preliminary assessment with level three evidence. Autologous HSCTs can induce remissions in all disease categories tested so far. Remissions can be transient or durable. HSCTs are associated with significant morbidity and mortality. Treatment-related mortality (TRM) is near 10% at 1 year and is associated with the intensity of the conditioning and the stage of the disease at the time of transplant. Marked inter-disease differences exist. There are few data available in haematological autoimmune diseases, more in systemic sclerosis (SSc), systemic lupus erythematosus (SLE), juvenile idiopathic arthritis (JIA) and multiple sclerosis (MS). Patient selection has been recognized as a crucial element from the phase I-II trials. Patients with advanced disease, severely compromised organ function or irreversible organ damage should not be considered as candidates for HSCT. Prospective randomized studies should now determine the value of HSCT compared to standard therapy. Such trials are ongoing for patients with systemic sclerosis (ASTIS trial - Autologous Stem Cell Transplantation International Scleroderma Trial) or are planned for patients with multiple sclerosis (ASTIMS trial - Autologous Stem Cell Transplantation International Multiple Sclerosis Trial) and rheumatoid arthritis (ASTIRA trial - Autologous Stem Cell Transplantation International Rheumatoid Arthritis Trial). More phase II data are needed for other indications such as SLE and JIA - HACIMUSTAFAOGLU M, CIL E, SARISOZEN B, ZINCIRCI M, ILDIRIM I: Bilateral septic arthritis of the knee joint in three children caused by unusual infectious agents. Pediatrics International 2001, 43:697-700.
Organism:Department of Pediatrics, Division of Pediatric Infectious Diseases, Uludag University, Faculty of Medicine, Gorukle, 16059, Bursa Turkey^E-Mail: mkemal@uludag.edu.tr - KIRWAN JF, SHAH P, KHAW PT: Diode laser cyclophotocoagulation: Role in the management of refractory pediatric glaucomas. Ophthalmology 2002, 109:316-323.
Organism:J.F. Kirwan, Paediatric Glaucoma Clinic, Moorfields Eye Hospital, Institute of Ophthalmology, 11-43 Bath Street, London ECIV 9EL
Abstract: Purpose: To report the efficacy and complications of diode laser cyclophotocoagulation (cyclodiode) in the management of refractory pediatric glaucomas. Design: Noncomparative interventional case series. Participants: Pediatric patients with uncontrolled glaucoma. Seventy-seven eyes of 61 patients underwent cyclodiode. Mean age was 7.4 years (range, 0.4-17 years). Main Outcome Measures: Intraocular pressure (IOP), visual acuity, complications. Results: Diagnoses included aphakic glaucoma, congenital glaucoma, juvenile chronic arthritis, aniridia, anterior segment dysgenesis, and Sturge-Weber syndrome. Sixty percent of eyes were aphakic, and 64% had undergone at least one previous surgical procedure for glaucoma. Patients underwent a mean of 2.3 treatment sessions per eye (maximum, 8 sessions). Mean pretreatment IOP was 32.0 mmHg. After one treatment session, 62% had a clinically useful reduction in IOP (<22 mmHg or by 30%), but this had fallen to 37% by 12 months. With repeat cyclodiode, 72% had a clinically useful reduction in IOP for a year or more (mean, 8.4-month interval between treatments). Aphakic eyes had a more sustained IOP reduction (P < 0.01 log rank test). Of treatment failures, 13% had no useful IOP response, and three eyes developed subsequent retinal detachment and loss of vision. No other eyes lost vision because of cyclodiode-related complications. In 5.5% of the treatment sessions there was a significant posttreatment inflammatory episode. Cyclodiode treatment did not enable a reduction in the number of medications. Conclusions: With repeated treatment, cyclodiode can provide effective control of IOP. However, the success rate is lower than with adults, and younger eyes may recover from treatment more rapidly. Although response may be temporary, cyclodiode has a lower rate of severe adverse effects than surgical modalities and has roles as a temporizing measure, as an adjunct to surgery, or in managing selected patients in whom surgery is undesirable because of a high risk of surgical complications. (c) 2002 by the American Academy of Ophthalmology - KITAI N, KREIBORG S, BAKKE M, PAULSEN HU, MOLLER E, DARVANN TA, PEDERSEN H, TAKADA K: Three-dimensional magnetic resonance image of the mandible and masticatory muscles in a case of juvenile chronic arthritis treated with the Herbst appliance. Angle Orthod. 2002, 72:81-87.
Organism:Department of Orthodontics and Dentofacial Orthopedics, Graduate School of Dentistry, Osaka University, Suita, Japan nkitai@dentosaka-uacjp
Abstract: The present report documents, in a case of juvenile chronic arthritis (JCA) with mandibular retrognathia, three-dimensional (3D) changes in the mandible and the relationship between the mandible and the masticatory muscles resulting from treatment with the Herbst appliance after cessation of growth. Magnetic resonance scanning of the whole head was carried out before and after treatment. The mandible, the masseter, and the medial and lateral pterygoid muscles were segmented bilaterally and reconstructed in 3D for both stages. Superimposition of the datasets was carried out according to anatomical structures in the brain (cranial base). Mandibular superimposition was performed according to the mandibular symphysis and the lower mandibular border. The mandible moved forward and downward relative to the anterior cranial base. In addition, bone apposition was observed at the superior and posterior surfaces of both mandibular condyles and at the roof of the glenoid fossa. The masticatory muscles remained relatively stable in position in relation to the anterior cranial base. To our knowledge, such information in JCA patients has not previously been published in the literature. Using magnetic resonance imaging (MRI), it was possible to gain improved insight into the 3D morphology including soft tissues without the overlap of the surrounding tissues observed in the conventional radiographs. Accordingly, it is suggested that 3D magnetic resonance analysis is a more useful method for the follow-up of the JCA patients than radiographic techniques
Internet : PM:11843278 - KURAHARA D, TOKUDA A, GRANDINETTI A, NAJITA J, HO C, YAMAMOTO K, VENU RD, MACPHERSON K, IWAMURO M, YAMAGA K: Ethnic differences in risk for pediatric rheumatic illness in a culturally diverse population. Rinsho Ganka 2002, 29:379-383.
Organism:Dr. D. Kurahara, 734-1319 Punahou Street, Honolulu, HI 96826
Abstract: Objective. To analyze the differences of occurrence of pediatric rheumatic disease among various ethnic groups in a culturally diverse isolated geographic area. Methods. A retrospective study of pediatric rheumatic diseases in a multiethnic area during a 6 year period. Results. A group of 922 patients was categorized based on predominant ethnicity, and their risk of having acute rheumatic fever (ARF), juvenile rheumatoid arthritis (JRA), and systemic lupus erythematosus (SLE) was studied. Odds ratios (OR) were computed for each illness with Caucasians as the reference group. Results indicated that Polynesians were overrepresented among patients with ARF, having elevated OR that were significantly different from Caucasians (22.5-120.7, p < 0.0001). For SLE, the highest OR were obtained for Samoans, Filipinos, and Japanese. In contrast, for JRA, Filipinos and Japanese had OR less than one, and no Samoans were diagnosed with JRA, possibly indicating a protective effect against developing JRA. Conclusion. This unique retrospective study examined the ethnic variations of expression of certain rheumatic diseases in an isolated region. Results reveal that certain ethnic groups are at risk for ARF and SLE, but are protected against JRA. These findings suggest investigating possible immunogenetic similarities and differences in these illnesses
Internet : davidk@kapiolani.org - KYNGAS H, RISSANEN M: Support as a crucial predictor of good compliance of adolescents with a chronic disease. J.Clin.Nurs. 2001, 10:767-774.
Organism:Kagawa Medical University, School of Nursing, Kida-Gun, Kagawa-Ken, Japan kyngas@kmsacjp
Abstract: The purpose of this study was to describe the factors that predict compliance among adolescents with a chronic illness. The data were collected by questionnaires from adolescents with asthma, epilepsy, juvenile rheumatoid arthritis (JRA) and insulin-dependent diabetes mellitus (IDDM). Groups of 300 adolescents with these illnesses were selected from the Finnish Social Insurance Institution's register, giving a total study series of 1200 individuals. The final response percentage was 88% (n = 1061). The data were analysed with the SPSS software. Logistic regression was used to indicate the predictors of good compliance. The compliance of adolescents with a chronic disease was predicted on the basis of support from parents, nurses, physicians and friends, as well as motivation, energy and willpower. The most powerful predictor was support from nurses. The likelihood of adolescents supported by nurses complying with health regimens was 7.28-fold compared to the adolescents who did not receive support from nurses. The next powerful predictor was energy and willpower. Adolescents who had the energy and willpower to take care of themselves complied with health regimens with a 6.69-fold likelihood compared to the adolescents who did not have energy and willpower. Adolescents who had good motivation were 5.28 times more likely to comply than the adolescents who did not have motivation. Support from parents, physicians and friends similarly predicted good compliance with health regimens
Internet : PM:11822848 - LEHMAN TJA, STRIEGEL KH, ONEL KB: Thalidomide therapy for recalcitrant systemic onset juvenile rheumatoid arthritis. Journal of Pediatrics 2002, 140:125-127.
Organism:Dr. T.J.A. Lehman, Hospital for Special Surgery, Sanford Weill Medical Center, Cornell University, 535 E 70th St, New York, NY 10021
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 - MISEROCCHI E, BALTATZIS S, EKONG A, ROQUE M, FOSTER CS: Efficacy and safety of chlorambucil in intractable noninfectious uveitis: The Massachusetts eye and ear infirmary experience. Ophthalmology 2002, 109:137-142.
Organism:Ocular Immunology and Uveitis Service, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA, 02114 USA
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-Behcet'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 - MYERS A, CLARK J, FOSTER H: Tuberculosis and treatment with infliximab. N.Engl.J.Med. 2002, 346:623-626.
Internet : PM:11859881 - ROZIN A, SCHAPIRA D, NACHTIGEL A, MENAHEM NA: [Kashin-Beck disease in Israel?]. Harefuah 2002, 141:20-5, 127.
Organism:B Shine Dpt of Rheumatology, Institute of Technology, Haifa, Israel
Abstract: This is a case report of a 21 year old young man who suffers from severe early bilateral hip joint osteoarthrosis. Due to this surprising finding we discuss the differential diagnosis between Kashin-Beck disease, an endemic disease of the patients prior living area (east Siberia) and severe hip joint damage secondary to juvenile rheumatoid arthritis and other diseases
Internet : PM:11851101 - SAITO Y, SAITO K, HIRANO Y, IKEYA K, SUZUKI H, SHISHIKURA K, MANNO S, TAKAKUWA Y, NAKAGAWA K, IWASA A, FUJIKAWA S, MORIYA M, MIZOGUCHI N, GOLDEN BE, OSAWA M: Hyperzincemia with systemic inflammation: a heritable disorder of calprotectin metabolism with rheumatic manifestations? J.Pediatr. 2002, 140:267-269.
Organism:Department of Pediatrics, Institute of Rheumatology, Tokyo Women's Medical University (TWMU), Japan
Abstract: A boy had infantile-onset systemic inflammation, growth failure, hepatosplenomegaly, anemia, leukocytopenia, progressive muscular dystrophy, and hypercalprotectinemia, resulting in marked hyperzincemia. His mother had a history of chronic arthritis since childhood and also showed hypercalprotectinemia/hyperzincemia. We postulate an inherent defect in calprotectin metabolism
Internet : PM:11865286 - SCOLA MP, THOMPSON SD, BRUNNER HI, TSORAS MK, WITTE D, VAN DIJK MA, GROM AA, PASSO MH, GLASS DN: Interferon-gamma: Interleukin 4 ratios and associated type 1 cytokine expression in juvenile rheumatoid arthritis synovial tissue. Rinsho Ganka 2002, 29:369-378.
Organism:Dr. D.N. Glass, Division of Rheumatology, Pavilion 2-129, Children's Hospital Medical Center, 3333 Burner Avenue, Cincinnati, OH 45229-3039
Abstract: Objective. To compare synovial tissue cytokine mRNA expression between patients with juvenile rheumatoid arthritis (JRA) and a heterogeneous group of non-autoimmune arthropathies (controls) with respect to type 1/type 2 balance. Methods. Thirty-five JRA (average 9.1 years' disease duration) and 13 control synovial tissues were studied. As a measure of the type 1/type 2 cytokine balance in a subset of the JRA and control tissues, interferon-gamma(IFN-gamma) and interleukin 4 (IL-4) mRNA levels were measured by competitive fragment reverse transcription-polymerase chain reaction. To quantitate additional cytokines relevant to this balance, multiprobe ribonuclease protection assays were employed measuring IL-5, IL-10, IL-13, IL-15, IL-18, and IL-12 (p35 and p40 subunits). Immunohistochemistry was performed on JRA tissues using antibodies specific for IL-15 and IL-18. Results. A higher IFN-gamma:IL-4 ratio (p = 0.034) was found in JRA tissues compared to controls. JRA tissues also displayed higher mRNA levels of IL-12p35 (p = 0.021), IL-15 (p = 0.002), and IL-18 (p = 0.017), but not IL-4 and IL-10. IFN-gamma expression in JRA, but not controls, correlated strongly with IL-12p35 (r = 0.63) and IL-12p40 (r = 0.73) levels. A subset of IL-15+ and IL-18+ cells was detected in JRA synovial tissues, largely within perivascular aggregates. Conclusion. JRA synovial tissue cytokine expression patterns indicate a type 1 bias, even in the later stages of disease. The strong correlation between IFN-gamma and IL-12 in JRA suggests a prominent role for IL-12 in promoting the type 1 bias, while IL-15 and IL-18 may also indirectly increase IFN-gamma expression and further bias the immune response
Internet : glasdO@chmcc.org - SIMON D, LUCIDARME N, PRIEUR AM, RUIZ JC, CZERNICHOW P: Linear growth in children suffering from juvenile idiopathic arthritis requiring steroid therapy: natural history and effects of growth hormone treatment on linear growth. J.Pediatr.Endocrinol.Metab 2001, 14 Suppl 6:1483-1486.
Organism:Service d'Endocrinologie et de Diabetologie Pediatriques, Hjpital Robert Debre, Paris, France dominiquesimon@rdbap-hop-parisfr
Abstract: We assessed linear growth and final height retrospectively in a group of 24 patients suffering from juvenile idiopathic arthritis (JIA) during childhood who had received steroid therapy. In these patients, there was a significant loss of height of more than 2 standard deviations during the first years of the disease, which correlated positively with the duration of prednisone therapy. After remission of the disease and discontinuation of prednisone treatment, 70% of the patients achieved catch-up growth, although 30% showed a persistent loss of height. Their mean final height was strongly correlated with their mean height at the end of steroid therapy and was significantly different between the group of patients with catch-up growth and the group without catch-up growth. This pattern of growth observed in patients with JIA should help us to define strategies of growth hormone (GH) treatment in these patients in order to improve their final height. We have previously reported the beneficial effects on growth and body composition of 1 year of GH treatment in a group of 14 growth-retarded patients suffering from JIA who received glucocorticoid therapy. These patients (n = 13) were treated again with GH at the same dosage (0.46 mg/kg/week [0.07 mg/kg/day]) for another 3-year period. GH treatment markedly increased growth velocity in these patients, but had a minor effect on height SDS, suggesting that these children will remain short when adults. Starting GH therapy in these patients earlier after the onset of the disease may prevent growth deterioration and metabolic complications induced by chronic inflammation and long-term steroid therapy
Internet : PM:11837503 - TASKIRAN E, TOROS T: Chronic synovitis caused by a date palm thorn: An unusual clinical picture. Arthroscopy 2002, 18:E7
Organism:Ege University School of Medicine Department of Orthopaedics and Traumatology, Izmir, Turkey taskiran@medegeedutr
Abstract: Synovitis related to plant thorns is a rare pathology. As in this case, thorn prick injury, which is particularly caused by thin palm thorns, is frequently missed in clinical history. Clinical presentation of cases may simulate juvenile rheumatoid arthritis with monoarticular involvement and septic arthritis. Removal of all thorns with partial or total synovectomy is essential in definitive treatment. Arthroscopy is the most valuable method of meeting both diagnostic and therapeutic purposes as it provides the best visibility of the joint with lower morbidity
Internet : PM:11830822 - ULLRICH G, MATTUSSEK S, DRESSLER F, THON A: How do adolescents with juvenile chronic arthritis consider their disease related knowledge, their unmet service needs, and the attractiveness of various services? Eur.J.Med.Res. 2002, 7:8-18.
Organism:Kinderklinik der MHH, Hannover Medical School, Carl-Neuberg-Str1, D-30623 Hannover, Germany UllrichGerald@MH-Hannoverde
Abstract: AIMS: To explore the information needs of adolescents with juvenile chronic arthritis (JCA) with respect to patient education and other measures to promote self-management. METHODS: Standardized cross-sectional inquiry concerning disease-related knowledge, perceived importance of information giving, unmet needs as well as perceived attractiveness of a range of services (lecture, structured patient education, support group, self-help group) to promote self-management. SAMPLE: N = 48 adolescents (68% of all adolescents with JCA of our outpatient clinic); mean age x = 14.9 (+/- 2.1) years; 56% female; 17% had the oligoarthritis form of JCA, 40% juvenile spondylarthritis, 25% polyarthritis and systemic form, 19% other rheumatic diseases. RESULTS: The majority of adolescents considered themselves as sufficiently well-informed and voted in favour of detailed information giving. However, 30% were unsatisfied with their current information and knowledge. Information needs predominantly related to the prognosis, course, and treatment of JCA, whereas the psychosocial impact (except sports and job matters) were judged as less important. Adolescents with a lower level of education were generally more interested than those with a high level of education. As for the attractiveness of services nearly half of the adolescents judged all of them as not very attractive. CONCLUSIONS: The majority of adolescents is interested in detailed information giving and some of them point to unmet needs, but nearly half of them is hesitant towards services which are delivered in a group format (such as structured patient education or support groups)
Internet : PM:11827835