Bibliography December 2000- ARKACHAISRI T, LEHMAN TJ: Use of Biologics in the Treatment of Childhood Rheumatic Diseases. Curr.Rheumatol.Rep. 2000, 2:330-336.
Organism:Division of Pediatric Rheumatology, The Hospital for Special Surgery, 535 East 70th Street, New York, NY 10021, USA
Abstract: Over the last two decades, progression in the knowledge of molecular biologic techniques has led to a better understanding of immunopathogenesis and identification of several cytokines, which propagate chronic arthritis in both adult and pediatric patients. A number of biologic agents have been developed in targeting different immune markers or mediators with the hope that they may help in controlling the inflammation in the group of patients who are resistant to disease-modifying antirheumatic drugs (DMARDs) and may alter the natural history of the diseases. Tumor necrosis factor (TNF)-alpha is the first to be targeted and its antagonists have been approved. Other biologic agents targeting different markers/mediators have followed and have been tested in clinical trials especially in adult rheumatoid arthritis (RA). Experiences in pediatric use are limited and agents proved to be effective in adult RA are not always transferred the same efficacy in different subtypes of juvenile idiopathic arthritis (JIA). It is reasonable to see the efficacy, and more importantly the safety profiles of each agent before the decision is made to use them in children. This article reviews the published and anecdotal reports of biologic agents that have been used in children with JIA and also focuses on the potential use of other biologic agents in JIA that have been used in trials to combat adult RA
Internet : PM:11123079 - ARSLANOGLU S, MURAT H, FERAH G: Spondyloepiphyseal dysplasia tarda with progressive arthropathy: An important form of osteodysplasia in the differential diagnosis of juvenile rheumatoid arthritis. Pediatrics International 2000, 42:561-563.
Organism:Dr Behcet Uz Children's Hospital, Inolu Cad. No. 556/3, 35290, Izmir Turkey - BLANCHONG CA, OLSHEFSKI R, KAHWASH S: Large Granular Lymphocyte Leukemia: Case Report of Chronic Neutropenia and Rheumatoid Arthritis-like Symptoms in a Child. Pediatr.Dev.Pathol. 2001, 3:94-99.
Organism:Division of Hematology/Oncology, Columbus Children's Hospital, the Ohio State University, 700 Children's Drive, Columbus, OH 43205, USA
Abstract: Lymphoproliferative disorders of large granular lymphocytes (LGL) are heterogeneous, with a clinical/pathologic spectrum ranging from a benign polyclonal expansion to an aggressive clonal disease. Often these lymphoproliferative disorders are associated with autoimmune disease. The clonal form of the disorder, LGL leukemia, typically occurs in older adults with a median age of 55 years at diagnosis. Pediatric cases are referred to in review articles; however, no detailed reports of T-cell LGL leukemia in children exist. This report illustrates a case of a child who presented initially at age 2 and 1/2 years with psoriasis, juvenile rheumatoid arthritis-like symptoms, and neutropenia. Bone marrow examinations obtained throughout his course have demonstrated progressive hypercellularity with increased reticulin fibers and replacement of the normal marrow elements by lymphocytes, which were later identified as large granular lymphocytes. Further testing with immunophenotyping by flow cytometry and T-cell receptor gene rearrangement studies revealed a monoclonal proliferation of large granular lymphocytes and confirmed a diagnosis of LGL leukemia. Although rare, large granular lymphocyte leukemia should be included in the differential diagnosis of chronic neutropenia in children
Internet : PM:11116299 - BREIT W, FROSCH M, MEYER U, HEINECKE A, GANSER G: A subgroup-specific evaluation of the efficacy of intraarticular triamcinolone hexacetonide in juvenile chronic arthritis. Rinsho Ganka 2000, 27:2696-2702.
Organism:Dr. G. Ganser, Abt. Kinder/und Jugendrheumatol, St. Josef Stift, Westtor 7, 48324 Sendenhorst
Abstract: Objective. To determine the subgroup-specific differences of intraarticular triamcinolone hexacetonide (TH) in the treatment of joint inflammation in patients with juvenile chronic arthritis (JCA). Methods. A retrospective review of 194 children of all subgroups of JCA, treated by a single or repeated TH injection between 1989 to 1994. Efficacy and duration of benefit were evaluated after a mean duration of 3, 15, 30, and 64 weeks. Results. In all, 1439 TH injections were given to 194 patients; 368 of these were reinjections. The median duration of improvement of all injections was 74 weeks. Responses were significantly different among subgroups (p = 0.0001): there were 121 weeks of efficacy in early-onset pauciarticular JCA type I (223 injections), 47 weeks in late-onset pauciarticular JCA type II (190 injections), 105 weeks in rheumatoid factor negative polyarticular JCA (445 injections), 63 weeks in rheumatoid factor positive polyarticular JCA (127 injections), and 36 weeks in systemic JCA (413 injections). Forty-one injections were done in other rheumatic diseases. In relation to this result there were also differences with regard to joint groups, antinuclear antibody (ANA) and HLA-B27 status, and sex. Side effects were rare: infections of skin or joints were not noted; skin and lipoatrophy were seen after 15 injections, necrosis of the hip in one case, luxation of 2 shoulders of one patient, and periarticular calcification in 3 patients. Conclusion. Intraarticular TH is an effective therapy for inflammatory joint disease in all subgroups of JCA. The risk of major complications is low. The median duration of improvement depends on the subgroup of the disease
Internet : ganser@st-josef-stift.de - BREIT W, FROSCH M, MEYER U, HEINECKE A, GANSER G: A subgroup-specific evaluation of the efficacy of intraarticular triamcinolone hexacetonide in juvenile chronic arthritis. Rinsho Ganka 2000, 27:2696-2702.
Organism:Abteilung fuer Kinder- und Jugendrheumatologie, St. Josef Stift, Westtor 7, 48324, Sendenhorst Germany
Abstract: Objective: To determine the subgroup-specific differences of intraarticular triamcinolone hexacetonide (TH) in the treatment of joint inflammation in patients with juvenile chronic arthritis (JCA). Methods: A retrospective review of 194 children of all subgroups of JCA, treated by a single or repeated TH injection between 1989 to 1994. Efficacy and duration of benefit were evaluated after a mean duration of 3, 15, 30, and 64 weeks. Results: In all, 1439 TH injections were given to 194 patients; 368 of these were reinjections. The median duration of improvement of all injections was 74 weeks. Responses were significantly different among subgroups (p = 0.0001): there were 121 weeks of efficacy in early-onset pauciarticular JCA type I (223 injections), 47 weeks in late-onset pauciarticular JCA type II (190 injections), 105 weeks in rheumatoid factor negative polyarticular JCA (445 injections), 63 weeks in rheumatoid factor positive polyarticular JCA (127 injections), and 36 weeks in systemic JCA (413 injections). Forty-one injections were done in other rheumatic diseases. In relation to this result there were also differences with regard to joint groups, antinuclear antibody (ANA) and HLA-B27 status, and sex. Side effects were rare: infections of skin or joints were not noted; skin and lipoatrophy were seen after 15 injections, necrosis of the hip in one case, luxation of 2 shoulders of one patient, and periarticular calcification in 3 patients. Conclusion: Intraarticular TH is an effective therapy for inflammatory joint disease in all subgroups of JCA. The risk of major complications is low. The median duration of improvement depends on the subgroup of the disease - CUMMINS R, WAGNER-WEINER L, PALLER A: Pseudoporphyria induced by celecoxib in a patient with juvenile rheumatoid arthritis [2]. Rinsho Ganka 2000, 27:2938-2940.
Organism:Dr. R. Cummins, Children's Memorial Hospital, La Rabida Hospital, University of Chicago, Chicago, IL - CUMMINS R, WAGNER-WEINER L, PALLER A: Pseudoporphyria induced by celecoxib in a patient with juvenile rheumatoid arthritis. J.Rheumatol. 2000, 27:2938-2940.
Internet : PM:11128692 - CUNNINGHAM MJ, CHIU EJ, LANDGRAF JM, GLIKLICH RE: The health impact of chronic recurrent rhinosinusitis in children. Archives of Otolaryngology - Head and Neck Surgery 2000, 126:1363-1368.
Organism:Dr. M.J. Cunningham, Massachusetts Eye and Ear Infirmary, 243 Charles St., Boston, MA 02114
Abstract: Objectives: To report and quantify the health-related quality of life of children who require surgical intervention for chronic recurrent rhinosinusitis and to assess the perspective of the child vs that of the parent. Design: Prospective, observational. Patients and Intervention: Twenty-one of a consecutive sample of 35 children undergoing endoscopic sinus surgery for infectious indications completed, along with their parents, the Child Health Questionnaire. The Child Health Questionnaire measures in parallel both child and parent perceptions of health by means of separate parent proxy report (Child Health Questionnaire-Parent Form 50) and child self-report (Child Health Questionnaire-Child Form 87) questionnaires concerning physical and psychosocial functioning. Main Outcome Measures: Tabulated scores from both the Child Health Questionnaire-Parent Form 50 and Child Health Questionnaire-Child Form 87 were compared with published data from age-matched normative populations and several pediatric chronic disease groups. Results: Significant decrements in the general health of children with chronic recurrent rhinosinusitis compared with a normative sample were observed for both child- and parent-reported data, particularly in the physical domains. Children with rhinosinusitis were perceived by their parents to have significantly more bodily pain (P<.001) and to be more limited in their physical activities (P<.05) than children with asthma, juvenile rheumatoid arthritis, and other chronic disorders. Parent-child perceptions did vary, with parents reporting more pain and general behavioral effects relative to their children's reports in these areas. Conclusion: The health impact of chronic recurrent rhinosinusitis as reported by the subjective evaluations of pediatric patients and their parents is severe - DE BENEDETTI F, RAVELLI A: Juvenile idiopathic arthritis: Will etanercept be an improvement over current therapies? BioDrugs 2000, 14:93-98.
Organism:Dipartimento di Scienze Pediatriche, IRCCS Policlinico San Matteo, Universita degli Studi di Pavia, 27100, Pavia Italy - DE SILVA B, BANNEY L, UTTLEY W, LUQMANI R, SCHOFIELD O: Pseudoporphyria and nonsteroidal antiinflammatory agents in children with juvenile idiopathic arthritis. Pediatr.Dermatol. 2000, 17:480-483.
Organism:Department of Dermatology, Royal Infirmary of Edinburgh, and Department of Paediatric Rheumatology, Royal Hospital for Sick Children, Edinburgh, Scotland BDeS2excitecom
Abstract: Pseudoporphyria is characterized by erythema, blistering, and scarring on sun-exposed skin. Nonsteroidal antiinflammatory drugs (NSAIDs) are implicated in the etiology of this condition. In a 1-year prospective study of children attending the pediatric rheumatology clinic in Edinburgh we found a prevalence of pseudoporphyria of 10.9% in children taking NSAIDs for juvenile idiopathic arthritis. Naproxen was the most commonly implicated NSAID, independent of dosage. Blue/gray eye color was an independent risk factor for the development of pseudoporphyria. We would advise caution in prescribing naproxen in these children to prevent disfiguring facial scarring
Internet : PM:11123786 - DOLLFUS H, HAFNER R, HOFMANN HM, RUSSO RICARDO AG, DENDA L, GONZALES LD, DECUNTO C, PREMOLI J, MELO-GOMEZ J, JORGE JP, VESELY R, STUBNA M, DUFIER J, PRIEUR A, INTERNATIONAL CHRONIC INFANTILE NEUROLOGICAL CUTANEOUS AND ARTICULAR/NEONATAL ONSET MULTISYSTEM INFLAMMATORY DISEASE: Chronic Infantile Neurological Cutaneous and Articular/Neonatal Onset Multisystem Inflammatory Disease syndrome: Ocular manifestations in a recently recognized chronic inflammatory disease of childhood. Arch.Ophthalmol. 2000, 118:1386-1392.
Organism:Service de Genetique Medicale et Service d'Ophtalmologie, Hopitaux Universitaires de Strasbourg, 67089, Strasbourg France
Abstract: Objective: To report on the ocular manifestations of the Chronic Infantile Neurological Cutaneous and Articular/Neonatal Onset Multisystem Inflammatory Disease (CINCA/NOMID) syndrome, a rare, recently identified, pediatric multisystem inflammatory disease with chronic cutaneous, neurological, and articular manifestations. Design: Descriptive case-report study. Setting: International collaborative study based on a questionnaire. Results: We included 31 patients. The mean age at onset of eye manifestations was 4.5 years. Optic disc changes were the most common feature, occurring in 26 patients (83%), including optic disc edema, pseudopapilledema, and optic atrophy. Anterior segment manifestations varying from mild to severe were seen in 13 patients (42%); chronic anterior uveitis, in 17 patients (55%). Moderate to severe visual acuity loss in at least 1 eye was seen in 8 patients (26%) as a consequence of the disease. Posterior synechia, glaucoma, and white iritis were not observed in any patient. Conclusion: Ocular manifestations with potentially sight-threatening complications occur commonly in the CINCA/NOMID syndrome. The distinctive nature of these complications may assist the ophthalmologist in recognizing this rare disorder and distinguishing it from juvenile rheumatoid arthritis - FADILAH SAW, CHEONG SK, SHAHDAN S: The pale and limping child. Postgrad.Med.J. 2000, 76:717
Organism:Department of Medicine, Hospital Universiti Kebangsaan Malaysia (HUKM), Jalan Yacob Latiff, Bandar Tun Razak, 56000, Cheras, Kuala Lumpur: sfadilah@mail.hukm.ukm.my Malaysia - HOSSNY E, HADY HA, MABROUK R: Anti-centromere antibodies as a marker of Raynaud's phenomenon in pediatric rheumatologic diseases. Pediatr.Allergy Immunol. 2000, 11:250-255.
Organism:Dr. E. Hossny, Ain Shams University - Cairo, 40 A Baghdad St., Cairo 11341
Abstract: To examine the possible relationship between anti-centromere antibodies (ACA) and pediatric rheumatologic diseases, we investigated the presence of ACA (using enzyme immunoassay) in the sera of 45 children and adolescents with such diseases and compared the results with a group of 42 age- and gender-matched healthy subjects. ACA were present (>= 10 U/ml) in three out of five patients (60%) with scleroderma (SCD), in seven out of 16 (43.8%) patients with systemic lupus erythematosus (SLE), in two out of five patients (40%) with mixed connective tissue disease (MCTD), in one out of four patients (25%) with dermatomyositis (DMS), and in two out of 14 patients (14.3%) with juvenile rheumatoid arthritis (JRA). ACA were also detected in a single patient with anti-phospholipid syndrome (APL) who had digital gangrene and hemiparesis, as well as in two healthy subjects. ACA positivity was related to the presence of Raynaud's phenomenon in the studied sample, as 86% of patients suffering from the phenomenon were ACA positive. ACA positivity was associated with older age, high blood pressure and high erythrocyte sedimentation rate (ESR) values, and lower hemoglobin and weight and height percentile values. It was also higher among anti-nuclear antibody-positive subjects. Raynaud's phenomenon and ACA positivity shared almost the same clinical and laboratory associations in the studied patients. Thus, ACA are probably among the markers of Raynaud's phenomenon in pediatric rheumatologic diseases. Their value as predictors of future development of the phenomenon needs further evaluation. (C) Munksgaard, 2000
Internet : elhossny@yahoo.com - JUAREZ-ECHENIQUE JC, ORDAZ-FAVILA JC: Ophthalmological features in subjects with juvenile rheumatoid arthritis. Revista Mexicana de Oftalmologia 2000, 74:117-120.
Organism:Dr. J.C. Juarez-Echenique, Instituto Nacional de Pediatria, Insurgentes Sur 1300-C, Mexico City
Abstract: A retrospective study was performed in the Instituto Nacional de Pediatria from 1993 to 1998 in subjects with the diagnose of juvenile rheumatoid arthritis. Uveitis was found in 13, being females with pauciarticular rheumatoid arthritis and positive antinuclear antibodies the high risk group. Males with uveitis had the worse visual prognosis and complications. Early detection and treatment in subjects with juvenil rheumatoid arthritis determines a better visual outcome
Internet : jcje@prodigy.net.mx - KONO T, ISHII M, TANIGUCHI S, NEGORO N: Psoriasis in a patient with juvenile rheumatoid arthritis. Dermatology 2000, 201:275-276.
Organism:Dr. T. Kono, Department of Dermatology, Osaka City University Medical School, 1-4-3, Asahimachi, Abeno-ku, Osaka 545-8585
Abstract: A 20-year-old woman had polyarticular-onset type of juvenile rheumatoid arthritis with a chronic and destructive course since 6 years of age. She had developmental retardation, deformity and disability. Asymptomatic erythematous scaly plaques developed on the trunk. A skin biopsy specimen revealed psoriasis. This is the first report of psoriasis developing in a patient with juvenile rheumatoid arthritis. Copyright (C) 2000 S. Karger AG, Basel - LAXER RM, CLARKE HM: Rheumatic disorders of the hand and wrist in childhood and adolescence. Hand Clin. 2000, 16:659-671.
Organism:Division of Rheumatology, University of Toronto, Ontario, Canada
Abstract: Rheumatic diseases are common in the pediatric population. Because the hand surgeon is often the first specialist to whom children with rheumatic disease involving the upper extremity are referred, it is important they are aware of the wide variety of disorders that can present with joint complaints to facilitate prompt referral and treatment of these patients
Internet : PM:11117055 - LEHMAN TJ: Clinical Trials for the Treatment of Systemic Onset Juvenile Rheumatoid Arthritis-Juvenile Idiopathic Arthritis. Curr.Rheumatol.Rep. 2000, 2:313-315.
Organism:Division of Pediatric Rheumatology, Hospital for Special Surgery, and Sanford Weill Medical College of Cornell University, 535 East 70th Street, New York, NY 10021, USA goldscout@aolcom
Internet : PM:11123076 - LEPORE L: Cutaneous manifestations in rheumatic disorders. Medico e Bambino 2000, 19:505-511.
Organism:L. Lepore, Clinica Pediatrica, IRCCS 'Burlo Garofolo', Trieste
Abstract: Cutaneous involvement is frequently present in rheumatic disorders. Usually the cutaneous lesions are typical and are useful for the diagnosis. In some cases the cutaneous involvement is present since the beginning (Schoenlein-Henoch purpura, Kawasaki disease, CIN-CA, scleroderma, Behcet disease), in others (LES, Lyme disease, dermatomiositis, Juvenile Chronic Arthritis) the cutaneous signs can appear later, during the course of the disease - LEPORE L, KIREN V: Autologous bone marrow transplantation versus alternative drugs in pediatric rheumatic diseases. Haematologica 2000, 85:89-92.
Organism:L. Lepore, Clin. Pediatrica Universita Trieste, Ospedale Infantile Burlo Garofolo, Trieste
Abstract: A minority of children suffering from severe rheumatic diseases are unresponsive to conventional treatments. These patients can now be managed with a variety of immunosuppressive therapies. Methotrexate is considered the first choice diseasemodifying agent for adult and juvenile rheumatoid arthritis. In patients unresponsive to low doses of methotrexate, medium or high-doses can be useful. Instead of methotrexate, a recently develped immunosuppressive drug, mycophenolate-mofetil, which inhibits T- and B-lymphocyte proliferation, can be used. Another possibility for refractory rheumatic diseases, with no increase in toxicity, is combination therapy, for example methotrexate plus cyclosporine, or methotrexate plus salazopyrine or intravenous pulses of cyclophosphamide and methylprednisone. More recently two distinct inhibitors of tumor necrosis factor (etanercept and infliximab have been used successfully for intractable rheumatic diseases (juvenile idiopathic arthritis, psoriatic arthritis, spondyloarthropathies) but the followup is still too short to establish their long-term effectiveness. If all these treatments are unsuccessful, an autologous bone marrow transplantation can be proposed to selected patients. Interesting results have been obtained in pediatric rheumatic diseases such as juvenile idiopathic arthritis, systemic lupus erythematosus and systemic sclerosis. Further studies are required to assess the best procedures able to induce remission with a minimal risk of fatal events. (C) 2000, Ferrata Storti Foundation - MCHUGH K, GUPTA R, MURRAY K: Imaging in juvenile chronic arthritis. Imaging 1999, 11:91-97.
Organism:K. McHugh, Department of Radiology, Great Ormond St. Hosp. for Children, London WC1N 3JH
Abstract: - 80% of children with JCA become symptomatic by the age of seven. - Patients may present with a systemic illness with delayed onset of arthritis (Still's disease). - The four major types of arthritis are Still's disease, oligoarthritis, polyarthritis and enthesitis arthritis. - Plain films are usually sufficient for diagnosis and assessing progression. - The degree of synovial hypertrophy and inflammation can be assessed with MRI and iv gadolinium. - MRI has the potential of improving management by quantifying disease progression and response to treatment - MEYER O: Oral immunomodulation therapy in rheumatoid arthritis. Joint Bone Spine 2000, 67:384-392.
Organism:O. Meyer, Service de Rhumatologie, Hopital Bichat, 46, rue Henri-Huchard, 75018 Paris
Abstract: Because the gastrointestinal mucosa is a vast interface between the body and the environment, it is the main entry site for many environmental antigens. Enterocytes can cleave environmental antigens into peptides, bind these peptides to their CD1 receptor, and present them to T cells. Intact antigens can penetrate through specialized Peyer's patch enterocytes called 'M cells'; they are then degraded and presented by dendritic cells to Peyer's patch T cells. The influx of multiple antigens through the gastrointestinal mucosa usually results in tolerance. High-dose tolerance is due to T cell deletion or anergy, whereas low-dose tolerance involves activation of TGFbeta-producing Th2 or Th3 cells. TGFbeta inhibits lymphocyte proliferation and the production of antibodies to ingested antigens; in addition, it blocks the proliferation of lymphocytes in organs to which gastrointestinal Th3 lymphocytes migrate. This 'innocent bystander' effect has been used to try to induce oral tolerance, For instance, pretreatment with oral bovine type II collagen has proved capable of modulating several models of experimental polyarthritis. Arthritis severity was considerably reduced. Preliminary attempts in humans with rheumatoid arthritis have yielded promising results. (C) 2000 Editions scientifiques et medicales Elsevier SAS - NEIMAN AR, LEE LA, WESTON WL, BUYON JP: Cutaneous manifestations of neonatal lupus without heart block: Characteristics of mothers and children enrolled in a national registry. Journal of Pediatrics 2000, 137:674-680.
Organism:Dr. J.P. Buyon, Department of Rheumatology, Hospital for Joint Diseases, 301 East 17th St, New York, NY 10003
Abstract: Objective: To extend the information base on cutaneous manifestations of neonatal lupus erythematosus (NLE) with regard to maternal disease, sex of child, onset, localization, influence of UV light, prognosis, and recurrence rates in subsequent pregnancies. Methods: Review of records from the Research Registry for Neonatal Lupus. Results: The cohort includes 47 mothers (83% white) whose sera contain anti-SSA/Ro, anti-SSB/La, and/or anti-U1-ribonucleoprotein antibodies and their 57 infants (20 boys and 37 girls) diagnosed with cutaneous NLE (absent heart disease) between 1981 and 1997. At detection of the child's rash, 13 mothers were asymptomatic, 11 had an undifferentiated autoimmune syndrome (UAS), 9 had systemic lupus erythematosus (SLE), 7 Sjogren's syndrome (SS), 6 SLE/SS, and 1 rheumatoid arthritis/SS; 20 reported photosensitivity. Within 5 years, 7 asymptomatic mothers experienced disease progression: 1 developed photosensitivity, 2 SLE, 3 SS, 1 SLE/SS; in 2 mothers UAS progressed to SLE; and 2 mothers with SS developed SLE. The infant's rash often followed UV light exposure; mean age at detection was 6 weeks, and mean duration was 17 weeks. All had facial involvement (periorbital region most common) followed by the scalp, trunk, extremities, neck, and intertriginous areas. In 37, the rash resolved without sequelae, 43% of which were untreated. A quarter had residual sequelae that included telangiectasia and dyspigmentation. One child developed Hashimoto's thyroiditis, and 2 developed systemic-onset juvenile rheumatoid arthritis. Of 20 subsequent births, 7 children were healthy, 2 had congenital heart block (CHB) only, 4 CHB and skin rash, and 7 skin rash only. Conclusions: Future pregnancies should be monitored by serial echocardiograms, given the substantial risk for heart block. Affected children should be observed for later development of a rheumatic disease - NJEH CF, SHAW N, GARDNER-MEDWIN JM, BOIVIN CM, SOUTHWOOD TR: Use of quantitative ultrasound to assess bone status in children with juvenile idiopathic arthritis: A pilot study. Journal of Clinical Densitometry 2000, 3:251-260.
Organism:Dr. C.F. Njeh, Osteoporosis/Arthritis Res. Group, Department of Radiology, University of California, 350 Parnassus Avenue, San Francisco, CA 94143-1349
Abstract: Periarticular osteoporosis around inflamed joints and generalized osteoporosis have been shown to be markers of disease activity and severity in children with juvenile idiopathic arthritis (JIA). Bone mineral density (BMD) in adults can be assessed precisely by dual X-ray absorptiometry (DXA), but this technique has not been used widely in children. Quantitative ultrasound (QUS) may provide an alternative method for assessment of bone status. The aim of this pilot study was to compare QUS to DXA in assessing generalized osteoporosis in a cohort of patients with JIA. Twenty-two Caucasian children (15 females, 7 males) with JIA of duration of 19-142 (mean 71 mo) and age 7-17 yr were recruited. Total body and lumbar spine BMD and bone mineral content (BMC) were measured by DXA using standard procedures on a Lunar DPX-L scanner. QUS was performed using Myriad SoundScan 2000. Speed of sound (SOS) was measured at the right midtibia. The DXA results were compared to QUS using linear regression analysis. Spine and total body BMD measured by DXA correlated significantly with tibia SOS (spine: r = 0.57, p < 0.007; total body: r = 0.68, p < 0.001). Spine BMC was similarly related to SOS as BMD (r = 0.58, p < 0.007). Individual patient weight and height were strong predictors of BMD, but only moderate predictors of SOS. The mean spine BMD was lower in the JIA patients compared to the normal ranges (mean Z-score of -1.19). BMD Z-scores were negatively associated with duration of disease. Patients taking steroids were associated with lower Z-scores. In conclusion, SOS shows a significant correlation with BMD as measured by DXA, albeit with wide 95% confidence intervals in this small pilot study. QUS was also well tolerated and was technically easy to perform in these children. With the added advantage that it is free from radiation risk, further assessment of this potentially valuable tool for measuring bone status in children is warranted
Internet : christopher.njeh@oarg.ucsf.edu - PAEDIATRIC RHEUMATOLOGY ES: VII European Paediatric Rheumatology Congress: Annual Scientific Meeting of the Paediatric Rheumatology European Society, Geneva, Switzerland, September 23-27, 2000. Annals of the Rheumatic Diseases 2000, 59:713-749.
Abstract: This meeting contains abstracts of 166 papers, written in English, covering basic research, the classification of juvenile idiopathic arthritis, connective tissue disorders, epidemiology and outcome, infection and arthritis, juvenile dermatomyositis, juvenile idiopathic arthritis, mechanical and orthopedic problems, rare diseases, scleroderma, treatment of pediatric rheumatic diseases, vasculitis, and allied health professionals - PICCO P, GATTORNO M, MARCHESE N, VIGNOLA S, SORMANI MP, BARABINO A, BUONCOMPAGNI A: Increased gut permeability in juvenile chronic arthritides. A multivariate analysis of the diagnostic parameters. Clinical And Experimental Rheumatology 2000, 18:773-778.
Organism:Dr. P. Picco, 2nd Division of Pediatrics, G. Gaslini Scientific Institute, Largo G. Gaslini 5, 16147 Genoa
Abstract: Objective: This study was aimed at evaluating intestinal permeability (IP) in patients with oligoarticular juvenile idiopathic arthritis (o-JIA), spondyloarthropathy (SpA) associated with inflammatory bowel disease (IBD) and other forms of juvenile-onset chronic arthritidis (OIA) using the lactulose/mannitol (L/M) test in comparison with other noninvasive parameters of gut involvement. Methods: A series of 26 children affected with o-JIA and 14 with either SpA/IBD or OIA were assessed for IP. The urinary L/M ratio was measured by gas chromatography. The erythrocyte sedimentation rate (ESR), C reactive protein (CRP), and faecal alpha1 antitrypsin concentrations were also evaluated. Ten o-JIA patients displayed active arthritis while in 16 the disease was under control. Among the OIA patients, 11 were affected with psoriatic arthritis and the remaining 3 with chronic reactive arthritis. 14 patients with SpA-IBD had active synovitis or spine inflammation. 14 eo-pJCA and 22 OIA and SpA-IBD patients, respectively, were receiving NSAID therapy. Results: The mean L/M ratios for the Spa-IBD (0.07 +/- 0.02, mean +/- SD), OIA (0.05 +/- 0.02) and o-JIA (0.04 +/- 0.02) patients were significantly higher (19 < 0.001, p = 0.022 and p = 0.01, respectively) than those found in controls (0.02 +/- 0.01). Logistic regression analysis disclosed a positive correlation between the L/M ratio and the presence of gastrointestinal manifestations (p = 0.011). The type of disease (p = 0.28), the disease activity in the JCA patient group (p = 0.24) and NSAID administration (p = 0.210) did not seem to significantly influence the L/M ratio. Conclusions: All of the subtypes of juvenile chronic arthritides that we studied displayed an increased IP. Hence, gut wall inflammation (albeit asymptomatic) may also be present in o-JIA patients. The SpA-IBD patients with gastrointestinal symptoms displayed the highest mean L/M ratio values. The L/M test seemed to correlate with histopathological features of the gut mucosa. The L/M ratio was shown to be a highly sensitive but poorly specific test for predicting gut inflammatory disease compared to other non-invasive screening tests - PRABALAD S, ROWE WS: Atopy, autoimmunity, and the TH1/TH2 balance. Journal of Pediatrics 2000, 137:446-449.
Organism:Division of Rheumatology Children's Hospital Medical Center Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, 45229-3039 USA - RABUSIN M, ANDOLINA M, MAXIMOVA N, LEPORE L, PARCO S, TUVERI G, JANKOVIC G: Immunoablation followed by autologous hematopoietic stem cell infusion for the treatment of severe autoimmune disease. Haematologica 2000, 85:81-85.
Organism:M. Rabusin, Department of Pediatrics, Inst. of Maternal and Child Health, via dell'lstria 65/1, 34100, Trieste
Abstract: Background and Objectives. The aim of this study was to evaluate the tolerability and effectiveness of a non-myeloablative conditioning regimen followed by autologous hematopoietic stem cell infusion for the treatment of severe autoimmune diseases. Design and Methods. From 1996 patients with severe autoimmune disease not responsive to conventional immunosuppressive treatment were selected. The patients' blood or marrow cells were harvested after incubation with vincristine and methylprednisolone. Two different immunoablative conditioning regimens were employed. The first used cyclophosphamide (2500 mg/msup 2 in one day) and antilymphocyte globulin (ALG) (15 vials/msup 2 in three days) and the second used fludarabine (300 mg/msup 2 in two courses of 5 days) plus ALG (25 vials/msup 2 in 5 days). Results. Nineteen patients (14 female, 5 male) with severe autoimmune diseases were treated. Nine had a rheumatologic disorder (5 juvenile chronic arthritis, 1 rheumatoid arthritis, 1 systemic vasculitis, 1 Sjogren's syndrome, 1 Behcet's disease), 4 a neurologic disorder (3 multiple sclerosis, 1 myasthenia), 3 a haematologic disease (2 pure red cell aplasia, 1 autoimmune thrombocytopenia), 2 had a gastrointestinal disease (1 Crohn's disease, 1 autoimmune enteropathy) and 1 had a multiple autoimmune disorder. There was no regimen-related toxicity and no opportunistic infections occurred. Ninety percent of the patients improved and/or had a complete remission after the procedure. Fifty percent of the subjects went into complete or partial remission after a median follow-up of 15 months (range 3-25) while 50% relapsed after a median follow-up of 11 months, (range 6-16). The incidence of relapse in the group treated with fludarabine was lower (30%). Interpretation and Conclusions. A non-myeloablative conditioning regimen was able to induce persistent remission in some patients with severe autoimmune diseases. There was no mortality or morbidity related to the procedure. The extent of remission does, however, remain to be established. (C) 2000, Ferrata Storti Foundation
Internet : rabusin@burlo.trieste.it - ROBINSON RF, NAHATA MC, RENNEBOHM R, HIGGINS G: Safety and efficacy of etanercept in the juvenile rheumatoid arthritis. Pharmacotherapy 2000, 20:1268
Organism:Children's Hospital, Ohio State University, Columbus, OH USA - TAUBERT H, THAMM B, MEYE A, BARTEL F, ROST A-K, HEIDENREICH D, JOHN V, BRANDT J, BACHE M, WURL P, SCHMIDT H, RIEMANN D: The p53 status in juvenile chronic arthritis and rheumatoid arthritis. Clinical and Experimental Immunology 2000, 122:264-269.
Organism:H. Taubert, Institute of Pathology, Faculty of Medicine, University Halle-Wittenberg, Magdeburger Strasse 14, D-06097 Halle/Saale
Abstract: The aim of this study was to investigate the p53 status in two autoimmune diseases; juvenile chronic arthritis (JCA) and rheumatoid arthritis (RA). In a PCR-sequencing analysis of exons 4-9 of the p53 gene, no mutation was identified, except for the case of an RA synovectomy sample with two mutations of intron 7. p53 gene polymorphisms for codons 36, 47, and 213 were not detected. Codon 72 polymorphism showed an indication of an increased occurrence of the Pro/Pro allelotype in JCA. Expression of P53 protein was comparable for JCA and RA synovectomy samples. For all RA samples P53 protein was detectable, whereas one sample of a JCA patient failed to express P53 protein
Internet : helge.taubert@medizin.uni-halle.de - TORISU T: Should we change some medical terms im rheumatology? Ryumachi 2000, 40:857-860.
- TSUKAHARA M, TSUNEOKA H, TATEISHI H, FUJITA K, UCHIDA M: Bartonella infection associated with systemic juvenile rheumatoid arthritis. Clin.Infect.Dis. 2001, 32:E22-E23
Organism:Faculty of Health Sciences, Yamaguchi University School of Medicine, Ube City, Japan masato@poccyamaguchi-uacjp
Abstract: A 4-year-old girl with systemic juvenile rheumatoid arthritis had Bartonella infection diagnosed serologically. This case suggested that Bartonella (most probably Bartonella henselae) infection may in part be responsible for the development of systemic juvenile rheumatoid arthritis
Internet : PM:11112671 - TUCKER LB: Outcome Measures in Childhood Rheumatic Diseases. Curr.Rheumatol.Rep. 2000, 2:349-354.
Organism:Division of Rheumatology, BC Children's Hospital, 4480 Oak Street, Room 1A21, Vancouver, BC, Canada V6H 3V4 ltucker@cwbcca
Abstract: The childhood rheumatic diseases are a group of chronic disorders with variable courses, morbidity, and outcomes ranging from complete remission to long-term disability. The major childhood rheumatic disorders are juvenile idiopathic arthritis (systemic onset, polyarticular, oligoarticular, enthesitis-related, psoriatic), juvenile dermatomyositis, systemic lupus erythematosus, and the vasculitides. In this review, the current status of outcomes measurement application to pediatric rheumatology will be discussed
Internet : PM:11123082 - VAN DER VOORT CHARLOTTE ROUPE, HEIJNEN CJ, WULFFRAAT N, KUIS W, KAVELAARS A: Stress induces increases in IL-6 production by leucocytes of patients with the chronic inflammatory disease juvenile rheumatoid arthritis: A putative role for alpha1-adrenergic receptors. J.Neuroimmunol. 2000, 110:223-229.
Organism:Laboratory of Pediatric Immunology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Lundlaan 6, Room KC03.068.0, 3584 EA, Utrecht: c.heijnen@wkz.azu.nl Netherlands
Abstract: Juvenile rheumatoid arthritis (JRA) is characterized by chronic inflammation of the joints. In the present study we demonstrate that exposure of JRA patients to a noradrenergic stressor (cold pressor test) results in enhanced LPS-induced IL-6 production by peripheral blood cells of these patients. Healthy, age-matched controls had the same rise in norepinephrine, but do not respond with changes in IL-6 production after exposure to the cold pressor test. Moreover, PBMC of patients with JRA express mRNA encoding alpha1-adrenergic receptors (AR), predominantly of the alpha1d-AR subtype. In contrast, we could not detect mRNA encoding for alpha1-AR in PBMC of healthy controls. The results of this study suggest that expression of alpha1-AR mRNA in PBMC during chronic inflammation is associated with altered responses of the immune system to stress - WISE CA, BENNETT LB, PASCUAL V, GILLUM JD, BOWCOCK AM: Localization of a gene for familial recurrent arthritis. Arthritis And Rheumatism 2000, 43:2041-2045.
Organism:Dr. C.A. Wise, Research Department, TX Scottish Rite Hosp. for Children, 2222 Welborn Street, Dallas, TX 75219
Abstract: Objective. To localize the gene for familial recurrent arthritis via a genome-wide linkage scan in an extended kindred with the disease. Methods. A 3-generation family in which 9 members were diagnosed with juvenile idiopathic arthritis (JIA) was ascertained. In this family the disease was of very early onset and included episodic inflammation leading to eventual destruction of joints, muscle, and skin. We treated this disorder as a distinct clinical entity that we have named 'familial recurrent arthritis.' A genome-wide linkage scan with polymorphic microsatellites at 10 - 15-cM resolution was initiated. Results. The genome-wide scan generated a maximum 2-point logarithm of odds score with D15S211 (Z(max) = 3.27 at theta(max) = 0.0010). Haplotype reconstruction defined a candidate region of ~20 cM flanked proximally by D15S983 and distally by D15S127 on human chromosome 15. Conclusion. A gene for familial recurrent arthritis was localized to 15q22-24, as a result of a genomewide linkage scan in a large, multiply affected kindred. Identification of the altered gene will provide insights into the pathogenesis of autoimmune joint destruction that is reminiscent of JIA - WULFFRAAT NM, SANDERS LA, KUIS W: Autologous Hemopoietic Stem-cell Transplantation for Children with Refractory Autoimmune Disease. Curr.Rheumatol.Rep. 2000, 2:316-323.
Organism:Department of Pediatric Immunology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, PO 85090, 3508AB Utrecht, The Netherlands NWulffraat@WKZAZUNL
Abstract: Autologous stem cell transplantation (ASCT) has been proposed as a possible treatment for severe autoimmune diseases such as rheumatoid arthritis (RA), multiple sclerosis (MS), systemic sclerosis, and systemic lupus erythematosus (SLE). To date, more than 250 patients with various autoimmune disorders have undergone an ASCT since 1996. Among them, there is a very limited number of children. This review summarizes the experience with ASCT for pediatric rheumatic diseases. Most reported cases concern juvenile idiopathic arthritis (JIA). Experience with ASCT for childhood SLE, Scleroderma, or Dermatomyositis is very limited. To date, 12 children with severe systemic or polyarticular JIA, all with progressive disease activity despite the use of corticosteroids, MTX, CsA, or Cyclophosphamide were treated in our center with ASCT. Rheumatologic follow-up at 3-month intervals up to 36 months showed a marked decrease in arthritis severity as expressed by the core-set criteria for juvenile chronic arthritis (JCA) activity. However, these children remain at risk for severe viral infections due to the prolonged lymfopenia. ASCT in this severely ill patient group induces a very significant and drug-free remission of the disease, but carries a significantly risk of developing fatal MAS
Internet : PM:11123077 - YAMAMOTO T, ABE T, MASTUYA M, KOBAYASH K, AKAIKE J, IKEDA Y, HOSOKAWA A, SHIMOJI H, SAKAI H, YONEZAWA K, TOSAKA M, IMAI K, MATSUMOTO H: A case with juvenile rheumatoid arthritis who developed cerebral vasculitis and venovascular hypertension. Ryumachi 2000, 40:818-823.
Organism:T. Yamamoto, Department of Internal Medicine, Kushiro City General Hospital, Sapporo Medical University, Ogawahigashi-machi, Kodaira-city, Tokyo
Abstract: On November, 1997, a 15-year-old boy visited our hospital because of headache, fever and arthralgia. He was treated with 5 mg/day of prednisolone thereafter. On October 21, 1998, he was admitted because of remittent fever and multiple arthralgia and diagnosis of juvenile rheumatoid arthritis (JRA) was made. He was also found to have hypertension of 210/110 mmHg, and soon developed ptosis of the eye, facial paresis and perceptive deafness of the right side. Cerebrospinal fluid showed protein of 98 mg/dl and mildly elevated IgG, IgA and IgM levels with normal cell count. Brain MRI examination revealed multiple cerebral lesions in the frontal, parietal and cerebellar areas on the right, whose cause was thought to be vasculitis. Renal angiography demonstrated a right renal artery stenosis, compatible with renovascular hypertension. He was treated with 60 mg of prednisolone per day, which brought about a satisfactory improvement of the above rheumatic and neurologic signs. On November 17, 1998, he received a follow-up study of MRI, which failed to show any cerebral lesions, supporting the effectiveness of prednisolone. An angiotensin converting enzyme inhibitor successfully normalized hypertension and renin activity in serum, although renal blood flow did not increase