Bibliography September2001

  1. BAKKE M, ZAK M, JENSEN BL, PEDERSEN FK, KREIBORG S: Orofacial pain, jaw function, and temporomandibular disorders in women with a history of juvenile chronic arthritis or persistent juvenile chronic arthritis. Oral Surg.Oral Med.Oral Pathol.Oral Radiol.Endod. 2001, 92:406-414.
    Organism:UNIVERSITY OF COPENHAGEN AND RIGSHOSPITALET
    Abstract:     OBJECTIVES: We sought to study the long-term outcome of juvenile chronic arthritis (JCA) in the temporomandibular joint (TMJ).Study Design: Temporomandibular disorders, including TMJ involvement, were assessed in 42 women with pauciarticular or polyarticular JCA-on average 25.8 years from disease onset-and compared with those found in matched control subjects. Disease-related parameters associated with temporomandibular disorders were identified. RESULTS: The TMJ was involved in 66.7% of the patients, most severely in extended pauciarticular JCA. Temporomandibular disorders were more frequent in the patients than in the control subjects, especially in those with persistent disease. The TMJ involvement was positively correlated with disease duration and negatively correlated with jaw opening and occlusal support. Duration of active JCA and history of functional pain were identified as predictors of present TMJ involvement. CONCLUSION: In a long-term follow-up, TMJ involvement proved frequent in the studied patients and was associated with long disease duration and previous pain on jaw opening. The findings suggest that patients with JCA should undergo orofacial evaluation on a regular basis
    Internet : PM:11598575

  2. BARRON KS, WALLACE C, WOOLFREY A, LAXER RM, HIRSCH R, HORWITZ M, SIEGEL J, FILIPOVICH L, WULFFRAAT N, PASSO M, RIDER LG: Autologous Stem Cell Transplantation for Pediatric Rheumatic Diseases. Rinsho Ganka 2001, 28:2337-2358.
    Organism:Dr. K.S. Barron, Div. of Intramural Research, Natl. Inst. Allergy/Infectious Dis., National Institutes of Health, Bethesda, MD 20892-1356
    Abstract:     The National Institute of Allergy and Infectious Disease, National Institutes of Health, convened a workshop entitled The Next Step: Protocol Development for Autologous Stem Cell Transplantation for Pediatric Rheumatic Disease, June 2000, co-chaired by Drs. Karyl Barron and Carol Wallace. The goal of the workshop was to focus on the scientific rationale for stem cell transplantation therapy in the pediatric diseases, unique aspects of this therapy in the pediatric rheumatic diseases, transplantation issues and options, regulatory issues, and development of a DNA repository for these diseases
    Internet : Kbarron@nih.gov

  3. BEREND KR, VAIL TP: Hip arthroscopy in the adolescent and pediatric athlete. Clinics in Sports Medicine 2001, 20:763-778.
    Organism:Dr. K.R. Berend, Division of Orthopaedic Surgery, DUMC Box 3000, Durham, NC 27710
    Abstract:     The current literature offers only sparse reports of the use of hip arthroscopy in the pediatric patient injured during athletics. In contrast, the role of this technique in the diagnosis and treatment of multiple childhood hip conditions including pyarthrosis, Legg-Calve-Perthes disease, slipped capital femoral epiphysis, coxa vara, juvenile chronic arthritis, chondrolysis, and avascular necrosis is well described.SUP1, 3, 14, 20, 25, 34, 35, 37 The application of this relatively uncommon technique to the young athlete has only recently become more attractive. The ability to examine and treat traumatic intra-articular pathology with minimal morbidity and prompt recovery is mandated by the young age of these patients and their demanding activity levels. Hip arthroscopists are now beginning to correlate preoperative physical exam findings and history with diagnosis and expectations for outcome. As our combined experience with this technique grows, the specific indications for its use in the young athlete become increasingly better defined. In pediatric and adolescent patients, the new onset of hip pain should warrant a high level of suspicion for the more common causes of pain such as infection, Legg-Calve-Perthes disease, slipped capital femoral epiphysis, or developmental dysplasia. When these have been evaluated, further differential diagnosis should include labral tears, loose bodies, synovitis, and chondral lesions. As this review begins to elucidate, these conditions are amenable to arthroscopic evaluation and treatment. At this time, the presence of reproducible mechanical symptoms after a twisting or axial loading injury during athletics should prompt the orthopaedic surgeon to consider arthroscopic examination of the hip if conservative therapy fails. Satisfying and reproducible results have been achieved when using hip arthroscopy within these parameters
    Internet : kberend@mindspring.com

  4. BERNTSON L, SVENSSON E: Pain assessment in children with juvenile chronic arthritis: A matter of scaling and rater. Acta Paediatrica, International Journal of Paediatrics 2001, 90:1131-1136.
    Organism:L. Berntson, Department of Paediatrics, Hospital of Falun, SE-79182 Falun
    Abstract:     Twenty-six children with juvenile chronic arthritis and their parents participated in the study. We compared the assessments of pain perceived by the children on a visual analogue scale (VAS) with assessments on a 4-point verbal descriptor scale (VDS-4). In children aged 10 y and older, the pain assessment results of the children and the parents were compared. The discordance in paired data from the scale comparisons and from the inter-rater comparisons was evaluated using a statistical method developed for ordinal data. The preference of scale and motivation of the older children were summarized. Conclusion: The parents and children used the VAS and the VDS-4 differently. The children showed a higher inter-scale discordance compared with the parents and they gave their pain a higher rating on the VAS but a lower rating on the VDS-4 than their parents. The VAS was preferred by the children but did not show the most reliable results
    Internet : lillemor.berntson@falun.mail.telia.com

  5. BROPHY S, CALIN A: Ankylosing spondylitis: Interaction between genes, joints, age at onset, and disease expression. Rinsho Ganka 2001, 28:2283-2288.
    Organism:Dr. A. Calin, Royal National Hosp. Rheum. Dis., Upper Borough Walls, Bath BA1 1RL
    Abstract:     Objective. Ankylosing spondylitis (AS) is a chronic inflammatory disorder with symptom onset generally occurring in the late teens/mid-twenties. In women, a younger age at onset enhances disease susceptibility in the next generation. We examined the influence of age at symptom onset on phenotypic expression. Methods. Patients were divided into cohorts according to age of symptom onset. The primary outcome measure was radiological progression (by Bath AS Radiology Index, BASRI). Secondary measures were disease activity (Bath AS Disease Activity Index, BASDAI), function (Bath AS Functional Index, BASFI), numbers undergoing AS related surgery, and percentage with secondary disorders. Results. Age at onset had no significant effect on radiological progression (young onset vs late onset, 8.0, 8.6, respectively) disease activity (young vs late, 4.4, 4.4), need for non-hip surgical intervention (9%, 8%, respectively), or prevalence of secondary disorders (iritis, 40%, 41%; psoriasis, 20%, 19%; inflammatory bowel disease, 7.5%, 8.9%). By contrast, there was a striking increase in prevalence of total hip replacement in those with juvenile onset (18%, 8%, respectively; p<0.001). Regardless of age at onset, spinal progression determined radiologically was greater in those with hip arthritis compared to those without [young onset hip involvement vs non-hip involvement, 9.7 (2.4), 7.2 (3.0) (p<0.001); late onset hip involvement vs non-hip involvement, 10.1 (2.5), 7.1 (3.0), respectively]. Function deteriorates with age (young onset vs late onset, 3.7, 4.5, respectively; p<0.01). Conclusion. (1) Hip disease (young or late onset) is a major prognostic marker for longterm severe disease (patients with hip disease have a spinal score increased by 2.5-3 points or 35-40% more change). (2) Hip involvement is more prevalent among patients with young age at onset. (3) Young onset patients without hip involvement do not have more severe disease. Thus, age at onset, itself, does not influence disease severity. (4) Since hip involvement and not age at onset is associated with worse outcome, patients with a young age at onset may be assumed to have an increased susceptibility load (i.e., genetic component or environmental trigger) rather than more severity genes. The lack of association between severity and age at onset implies that the determinants of susceptibility and severity are independent
    Internet : andrei.calin@virgin.net

  6. BYWATERS EG: George Frederic Still (1868-1941): his life and work. J.Med.Biogr. 1994, 2:125-131.
    Internet : PM:11639933

  7. DAVIES K, STIEHM ER, WOO P, MURRAY KJ: Juvenile idiopathic polyarticular arthritis and IgA deficiency in the 22q11 deletion syndrome. Rinsho Ganka 2001, 28:2326-2334.
    Organism:Dr. K.J. Murray, Department of Rheumatology, Great Ormond Str. Hosp. Sick Child., Great Ormond Street, London WC1N 3EJ
    Abstract:     Five patients with the 22q11 deletion syndrome (velocardiofacial syndrome) developed chronic inflammatory polyarticular arthritis. These new cases add to 8 previously reported and confirm the association. The arthritis in all cases was moderate to severe, but at least partially responsive to methotrexate and/or corticosteroids, and was clinically indistinguishable from juvenile idiopathic arthritis (JIA). Analysis of the total 13 patients indicates that 2 are rheumatoid factor positive, 6 are antinuclear antibody positive, 5 have subtle T cell deficiencies, and 6 have hypergammaglobulinemia. Of particular interest is the occurrence of IgA deficiency in 4 patients, including 2 from our own series. Although IgA deficiency is seen in both JIA (2-4%) and 22q11 deletion syndrome (2-4%), the prevalence of low IgA in this series (31%) is much greater than expected. This phenomenon and the true association of inflammatory arthritis and a chromosome deletion disorder provides further evidence of important genetic factors in the pathogenesis of JIA
    Internet : K.Murray@ich.ucl.ac.uk

  8. FUJIKAWA S: [Japanese children with rheumatic diseases are not satisfied]. Ryumachi 2001, 41:723-725.
    Internet : PM:11577400

  9. HUPPERTZ H-I: Lyme disease in children. Current Opinion in Rheumatology 2001, 13:434-439.
    Organism:Dr. H.-I. Huppertz, Childrens' Hospital, Zentralkrankenhaus Sankt-Jurgen-Str., 98205 Bremen
    Abstract:     Lyme borreliosis is a multisystem disorder caused by Borrelia burgdorferi and transmitted by ticks in the northern hemisphere. The disease is common in children. In addition to frequently recognized manifestations such as erythema migrans, neuroborreliosis, and Lyme arthritis, rarer manifestations, including eye and ear disease, are increasingly understood. Clinical diagnosis is supported by serologic confirmation. Improvement of laboratory methodology, especially polymerase chain reaction-based tests, is continuing. Actual treatment recommendations based on controlled studies reflect expanding scientific knowledge. In the United States, license of a vaccine to prevent infection in children is awaited. Lyme borreliosis is an intriguing human example of bacterial persistence in the presence of the host immune system. Chronic Lyme arthritis is a model of chronic arthritis resembling forms of arthritis of unknown cause, such as rheumatoid arthritis and juvenile idiopathic arthritis. (c) 2001 Lippincott Williams & Wilkins, Inc
    Internet : huppertz.bremen@t-online.de

  10. KULAS DT, SCHANBERG L: Juvenile idiopathic arthritis. Current Opinion in Rheumatology 2001, 13:392-398.
    Organism:Dr. L. Schanberg, Duke University Medical Center, Department of Pediatrics, Box 3212, Durham, NC 27710
    Abstract:     A significant amount of work has been performed over the past 2 years further defining the pathophysiology of juvenile idiopathic arthritis (JIA). The development of the biologic agent etanercept represents successful translational research. This genetically engineered fusion protein targets tumor necrosis factor, binding and inactivating this important component of inflammation. This advance has been accompanied by refinements in classification, as well as further elucidation of the natural disease course of JIA. Recent work continues to demonstrate the widespread nature of JIA, as well as its severity in adulthood. (c) 2001 Lippincott Williams & Wilkins, Inc
    Internet : schan001@mc.duke.edu

  11. LOTITO APN, LAURINDO IMM, NOVAES GS, KISS MH, MELLO SB, V: Nitric oxide production is greatly enhanced in inflamed joint of juvenile rheumatoid patients. Inflammation Research 2001, 50:S167
    Organism:Rheumatology, School of Medicine, USP, Av. Dr. Arnaldo 455, Sao Paolo, SP Brazil

  12. MCDONAGH JE: Osteoporosis in juvenile idiopathic arthritis. Current Opinion in Rheumatology 2001, 13:399-404.
    Organism:Dr. J.E. McDonagh, Institute of Child Health, Birmingham Children's Hospital, Steelhouse Lane, Birmingham 4 6NH
    Abstract:     Osteoporosis is characterized by loss of both bone mass and microarchitectural integrity, resulting in an increased risk of fractures with associated morbidity and mortality. Awareness of this condition is increasing in pediatrics, including pediatric rheumatology. Reduced bone mineral density is now well recognized in children and young adults with juvenile idiopathic arthritis and is multifactorial in origin. The problems of interpretation of bone analysis techniques during childhood and adolescence are highlighted. Recent studies have reported on the use of newer methods of imaging, including quantitative ultrasound and bone single photon emission computed tomography techniques. Attempting to disentangle the relative effects of disease activity, corticosteroids, nutrition, and physical activity in the development of osteoporosis in juvenile idiopathic arthritis is the focus of several studies. Finally, early optimistic reports of the use of bisphosphonates in juvenile idiopathic arthritis are welcome additions to the growing body of literature in this area. (c) 2001 Lippincott Williams & Wilkins, Inc
    Internet : janetmcd@doctors.org.uk

  13. NIEWENHUIS MK, GONZALEZ RV, VAN DER NJ, KUIS W, BEEK FJA, BUCHANAN TS, HELDERS PJM: The role of the forearm muscles related to wrist malalignment in Juvenile Chronic Arthritis. Advances in Physiotherapy 2001, 3:108-119.
    Organism:Dr. P.J.M. Helders, Dept. of Pediatric Physical Therapy, Univ. Med. Ctr./Children's Hospital, P.O. Box 85090, 3508 AB Utrecht
    Abstract:     The development of malalignment of the wrist is understood to be a combination of pathological changes induced by the disease process, compressive forces on the carpus exerted by the forearm muscle tendons crossing the wrist and the geometry of the articular surfaces. Understanding how changes in compressive forces affect malalignment is important, in view of improving physiotherapeutic treatment of the wrist in children with juvenile chronic arthritis (JCA). Quantitative analysis, taking into account the various factors involved, has recently been made possible by the development of interactive graphics based musculoskeletal models. Therefore, individual models of wrists from one healthy as well as three JCA children, with different expressions and stages of malalignment, were made. Joint moments were calculated and shear forces were estimated by applying rigid body spring modeling (RBSM) to the modified wrist models. In the radial direction, the joint moment for all JCA subjects was lower than found in the healthy subject. Furthermore, in the ulnar direction, joint moments of the three JCA subjects showed substantial differences. For the two JCA subjects with ulnar-carpal translation, shear forces were large compared to the shear force found in the healthy subject, suggesting that translation may still increase. The interactive graphics wrist model in combination with RBSM seems to be a powerful tool for analyzing how changes in compressive forces affect and interact with malalignment of the wrist in children with JCA
    Internet : P.J.M.Helders@wkz.azu.nl

  14. PRAHALAD S, KINGSBURY DJ, GRIFFIN TA, COOPER B-L, GLASS DN, MAKSYMOWYCH WP, COLBERT RA: Polymorphism in the MHC-encoded LMP7 gene: Association with JRA without functional significance for immunoproteasome assembly. Rinsho Ganka 2001, 28:2320-2325.
    Organism:Dr. R.A. Colbert, Division of Rheumatology, Children's Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH 45229-3039
    Abstract:     Objective. To determine if a polymorphism in the immunoproteasome subunit LMP7 was associated with juvenile rheumatoid arthritis (JRA) and had functional significance. Methods. The frequency of LMP7QQ+ vs
    Internet : bob.colbert@chmcc.org

  15. RAVELLI A, BARTOLI M, TRAIL L: Inhibitors of tumor necrosis factor and juvenile idiopathic arthritis. Occhio Clinico Pediatria 2001, 5:22-24.
    Organism:A. Ravelli, Universita Di Pavia, IRCCS Policlinico San Matteo, Pavia
    Abstract:     In recent years, patients affected by rheumatoid arthritis have gained new therapeutic options with the introduction of two different tumour necrosis factor (TNF) blockers: a chimeric antibody named infliximab and a soluble receptor known as etanercept. At present, however, only etanercept has been approved for curing juvenile rheumatoid arthritis. Further experimental studies are in fact needed for infliximab. TNF inhibitors are given to patients with juvenile rheumatoid arthritis who have not responded to second-line medications such as methotrexate. Nonetheless, before these novel agents can be used on a large scale, a few issues need to be addressed. First, it is necessary to find out if they can actually influence the natural progression of the illness, whether it is better to administer them alone or in combination with methotrexate, and at what stage of the disease they should be given to patients. Furthermore, at present there are no sufficient data for determining their tolerability and the potential risks of long-term therapies for children

  16. RUSSO RAG, KATSICAS MM: Chronic infantile neurological cutaneous and articular syndrome: Two new cases with rare manifestations. Acta Paediatrica, International Journal of Paediatrics 2001, 90:1076-1079.
    Organism:R.A.G. Russo, Service of Immunology, Hosp. Pediat. Prof. Dr. JP. Garrahan, Combate de los Pozos 1881, 1245 Buenos Aires
    Abstract:     CINCA/IOMID is a systemic inflammatory disorder of unknown aetiology that resembles congenital infection and systemic juvenile chronic arthritis (JCA). This disorder is characterized by neonatal onset, persistent rash, ocular inflammatory lesions, and progressive articular and neurological involvement. We report two new patients with this syndrome. Both children presented periodic bouts of cutaneous rash, fever, organomegaly, articular involvement with typical radiological features, and developmental delay. One of the patients presented neonatal jaundice and elevation of liver enzymes; inflammatory infiltrates were observed in the liver biopsy. The other patient showed retinal vasculitis detected at age 18 mo on fundoscopy and fluorescent angiography. Therapy with azathioprine was associated with prolonged remission of this complication. In both cases, the disease was diagnosed after some delay. Conclusion: Early hepatitis and retinal vasculitis are rare features of CINCA/IOMID that may help differentiate this syndrome from JRA. Azathioprine may have induced the remission of vasculitis in one case
    Internet : rrusso@garrahan.gov.ar

  17. SAWHNEY S, WOO P: Diagnosis and Management of Juvenile Idiopathic Arthritis: Current Status. Indian Pediatr. 2001, 38:1083-1089.
    Organism:Consultant Pediatric Rheumatologist, Sir Ganga Ram Hospital, New Delhi, India sujatasawhney@netkrackercom
    Internet : PM:11677297

  18. SAWHNEY S, WOO P, MURRAY KJ: Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Arch.Dis.Child 2001, 85:421-426.
    Organism:Department of Rheumatology, Great Ormond Street Hospital, London WC1N 3JH, UK
    Abstract:     AIMS: To review the precipitating events, clinical features, treatment, and outcome of macrophage activation syndrome (MAS). METHODS: Retrospective review of cases of MAS from a prospectively collected database of children with rheumatic diseases from 1980 to 2000. RESULTS: Nine patients (eight girls) were considered to have evidence of MAS. The primary diagnosis was systemic onset juvenile idiopathic arthritis in seven, enthesitis related arthritis in one, and chronic infantile neurological cutaneous articular syndrome in one. Mean age of onset was 5.7 years, and duration prior to MAS, 4.2 years. No medication was identified as a trigger. Eight had infections prior to MAS; specific infectious agents were identified in four. High grade fever, new onset hepatosplenomegaly, and lymphadenopathy were common clinical features. Platelet counts fell dramatically, from an average of 346 to 99 x 10(9)/l. Mean erythrocyte sedimentation rate (in three patients) fell from 115 to 28 mm/h. Eight had abnormal liver function during the disease course, and six had coagulopathy. Bone marrow examination supported the diagnosis with definite haemophagocytosis in four of seven. All received high dose steroids (eight intravenous, one oral), five cyclosporin, two cyclophosphamide, and one antithymocyte globulin. Two of three patients with significant renal impairment died. CONCLUSION: MAS is a rare and potentially fatal complication of childhood rheumatic disorders. Most of our patients were female, and most cases were preceded by infection. Bone marrow studies support the diagnosis. Deranged renal function may be a poor prognostic sign. Aggressive early therapy is essential
    Internet : PM:11668110

  19. SCHILLING F, KESSLER S: [Chronic recurrent multifocal osteomyelitis-- I. Review]. Klin.Padiatr. 2001, 213:271-276.
    Organism:Klinikum der Johannes Gutenberg-Universitat Mainz, Germany
    Abstract:     Juvenile and adolescent "Chronic Recurrent Multifocal Osteomyelitis" (CRMO) is described on the basis of literature and analysis of 43 own cases (23 cases in children or adolescents).This systemic, non-purulent inflammatory disease occurs mainly metaphyseal in long bones, in pelvic bones or as spondylitis and is not as rare as it seemed. Basis of the disease is a primarily chronic, sterile, in phase of onset often monotopic (e.g. clavicle) and later frequently polytopic osteomyelitis, possibly triggered by an immuno-pathological process (e.g. Proprionibacterium acnes), and showing histologically plasmacellular invasion and a sclerosing process in different stages. Association with pustulous dermatosis (psoriasis, acne, palmo-plantar pustulosis) is found in about 25 % of children and adolescents and in more than 50 % of the adult patients. 5 differents types of distribution of osteomyelitic lesions can be found by using Te99m-bone scan primarily, of which the "pelvic type" is the most common. Because of the close neighbourhood of meta-/epiphyseal osteomyelitic focuses, "sympathetic arthritis" with synovitis is seen frequently. A therapeutic approach with azithromycine and calcitonine is presented
    Internet : PM:11582525

  20. SCHULTZ R, MATTILA J, GAPPA M, VERRONEN P: Development of progressive pulmonary interstitial and intra-alveolar cholesterol granulomas (PICG) associated with therapy-resistant chronic systemic juvenile arthritis (CJA). Pediatr.Pulmonol. 2001, 32:397-402.
    Organism:Department of Pediatrics, University Hospital of Tampere, Tampere, Finland
    Abstract:     A girl aged 5 years with therapy-resistant chronic systemic juvenile arthritis (CJA) developed progressive fibrosing lung disease. Histology of an open lung biopsy revealed pulmonary interstitial and intra-alveolar cholesterol granulomas (PICG). Since treatment with steroids and immunosupressive drugs did not prevent progression of lung fibrosis, an experimental treatment with a tumor necrosis factor alpha (TNFalpha) antagonist etanercept was started. Although development of chronic changes in the lung parenchyma could not be prevented, this treatment brought considerable relief and markedly improved the child's physical capacity. By ruling out other causes for development of PICG, we concluded that the primary disease had caused the development of cholesterol granulomata by macrophage activation. We suggest, therefore, that a trial with etanercept in children with otherwise therapy-resistant CJA should be considered, especially if pulmonary complications have developed
    Internet : PM:11596165

  21. SNOWDEN JA, BROOKS PM: Hematopoietic stem cell transplantation in rheumatic diseases. Current Opinion in Rheumatology 1999, 11:167-172.
    Organism:Royal Hallamshire Hospital, Glossop Road, Sheffield, S10 2JF UK

  22. SVENSSON B, ADELL R, JOHANSSON C, ALBREKTSSON T, HOLM S: Revascularisation of costochondral grafts: An experimental study in domestic pigs. Scandinavian Journal of Plastic and Reconstructive Surgery and Hand Surgery 2001, 35:247-259.
    Organism:B. Svensson, Department of Oral Surgery, Orebro Medical Centre Hospital, SE 701 85 Orebro
    Abstract:     Mandibular condyles are reconstructed immediately with costochondral grafts (CCGs) in children for several temporomandibular conditions, including ankylosis, benign neoplasia, and chronic arthritis. A prerequisite for growth of CCGs is that they are properly revascularised, but the revascularisation process has not to our knowledge so far been examined. The aim of the present study was therefore to investigate the revascularisation of CCGs when they were used for immediate reconstruction of the mandibular condyle in juvenile domestic pigs. Eleven mandibular condyles were experimentally resected and immediately reconstructed with CCGs. Microangiograms with an Indian ink solution were done 2, 4, 8, and 12 weeks after the reconstructions. The density of vessels was higher in measurement zones facing soft tissues than in those facing mandibular bone at all time points. Revascularisation of the growth plate originated from the surrounding recipient soft tissues and not from an endosteal blood supply from the host mandible ramus

  23. TYNDALL A: Autologous hematopoietic stem cell transplantation for severe autoimmune disease with special reference to rheumatoid arthritis. Rinsho Ganka 2001, 28:5-7.
    Organism:Prof. A. Tyndall, Felix Platter Spital, Burgfelderstrasse 101, Basel 4012
    Abstract:     In 1996 an international collaboration began to explore the use of immunoablation and stem cell rescue in the treatment of severe autoimmune disease. Around 500 patients have been so treated according to consensus guidelines, the majority being registered in the combined European League Against Rheumatism and European Group for Blood and Marrow Transplantation (EULAR/EBMT) data registry. Results in terms of toxicity and benefit are different in the different autoimmune diseases, e.g., for rheumatoid arthritis (RA) a low transplant related mortality (TRM) of one patient out of 43 but high relapse rate (around two-thirds), whereas for systemic sclerosis (SSc) a higher TRM (12%) but less relapse. More aggressive immunoablative regimes were associated with more procedure related toxicity, but so far a clear therapeutic advantage has not been demonstrated. An overall actuarial TRM of 9% was observed. Randomized, prospective controlled phase III trials have begun in SSc and will soon commence in RA and multiple sclerosis. More phase I and II data are required for systemic lupus erythematosus and juvenile idiopathic arthritis
    Internet : alan.tyndall@fps-basel.ch

  24. UNSAL E, BUYUKGEBIZ B, CEVIK N: Assessment of protein-energy malnutrition in children with chronic arthritis. Turk.J.Pediatr. 2001, 43:191-195.
    Organism:Department of Pediatrics, Dokuz Eylul University Faculty of Medicine, Izmir, Turkey
    Abstract:     Protein-energy malnutrition (PEM) has been estimated to occur in 10 to 50% of children with juvenile chronic arthritis (JCA). Thirty-eight children with JCA were evaluated and their nutritional status determined, and they were compared with 23 healthy sex and age-matched children as controls. A standardized, 9-parameter comprehensive nutritional assessment profile was used. The simple anthropometric measurements, height and weight for age, were abnormal in 30% and 27% of the patients, respectively. A detailed evaluation revealed that 71% had abnormal somatic protein stores, and that they also had significantly low levels of visceral protein stores, when compared to their healthy peers. The results were consistent with the fact that inflammation put the JCA patients at significant risk for developing complicated malnutrition and it might result in PEM without any obvious signs of malnutrition. A nutritional screening test would be very useful in detecting early PEM in children with chronic arthritis
    Internet : PM:11592507

  25. VENCOVSKY J, JAROSOVA K, RUZICKOVA S, NEMCOVA D, NIEDERLOVA J, OZEN S, ALIKASIFOGLU M, BAKKALOGLU A, OLLIER WE, MAGEED RA: Higher frequency of allele 2 of the interleukin-1 receptor antagonist gene in patients with juvenile idiopathic arthritis. Arthritis Rheum. 2001, 44:2387-2391.
    Organism:Institute of Rheumatology, Prague, Czech Republic venc@revmacz
    Abstract:     OBJECTIVE: An increased incidence of allele 2 of the interleukin-1 receptor antagonist gene (IL1RN*2) in several inflammatory diseases has recently been reported. The aim of this study was to examine a variable number tandem repeat (VNTR) polymorphism of the IL1RN gene in patients with juvenile idiopathic arthritis (JIA). METHODS: Findings in 185 Czech patients with JIA were compared with those in 168 Czech controls, 50 JIA patients and 52 controls of Turkish origin, and 79 controls from central England. VNTR polymorphism analysis of IL1RN was performed by polymerase chain reaction using 2 flanking primers to amplify an 86-bp tandem repeat region in intron 2. RESULTS: The frequency and carriage rate of IL1RN*2 were significantly increased in Czech JIA patients compared with the Czech controls (frequency 27.6% versus 15.8%; carriage rate 44.3% versus 26.2%). Increased frequency and carriage rate of IL1RN*2 were found in 23.3% and 40.0% of Turkish JIA patients and in 17.3% and 34.6% of ethnically matched controls. The high representation of IL1RN*2 in 52.3% of the 22 patients with extended oligoarthritis, 31.3% of the 56 patients with enthesitis-related arthritis, and 42.9% of the 14 patients with other arthritis was particularly responsible for the increased frequency of IL1RN*2 in the Czech JIA patients. We found no association of IL1RN*2 with disease activity or severity parameters. CONCLUSION: Inheritance of IL1RN*2 may contribute to genetic susceptibility in several forms of autoimmune diseases, including JIA. The IL1RN*2 allele may be useful as a prognostic indicator of the evolution of an extended oligoarticular course of JIA
    Internet : PM:11665981

  26. VON KOSKULL S, TRUCKENBRODT H, HOLLE R, HORMANN A: Incidence and prevalence of juvenile arthritis in an urban population of southern Germany: A prospective study. Annals of the Rheumatic Diseases 2001, 60:940-945.
    Organism:Dr. St. Von Koskull, Rheuma-Kinderklinik Rummelsb. Anst., Gehfeldstrasse 24, D-82467 Garmisch-Partenkirchen
    Abstract:     Objective - To ascertain the incidence and prevalence of juvenile arthritis in a German urban population. Methods - All 766 paediatricians, orthopaedists, and rheumatologists working in practices or outpatient clinics in 12 south German towns were asked to report all patients who consulted them for juvenile arthritis during the year 1995. Patients with continuing symptoms were followed up for 9-12 months to obtain a final diagnosis. Extended measures of quality control were taken to control for known biases. Results - Of 457 reported cases, 294 were diagnosed with para-/postinfectious arthritis (PPA), 78 with juvenile chronic arthritis (JCA), and 18 with other forms of arthritis. Half of the PPA cases were classified as transient synovitis of the hip (SH). For JCA the reported annual incidence was 6.6 and the prevalence 14.8 per 100 000 subjects under 16 years of age. For PPA the reported incidence was 76 and the prevalence 4.4 per 100 000 subjects under 16. The incidence of rheumatic fever was clearly below 1 per 100 000 people under 16. A correction model was used to control for known biases and to adjust the estimates accordingly. Conclusions - The results of this first prospective study on the incidence and prevalence of juvenile arthritis in Germany are consistent with a retrospective study performed in the Berlin area. Based on these results it was estimated that the annual frequency of juvenile arthritis in Germany is as follows: 750-900 incident JCA cases, 21 000 incident SH cases, and 21 000 incidence cases of other forms of PPA a year. The number of incidence cases of rheumatic fever is expected to be markedly lower than 150 a year. The total prevalence is expected to be 3600-4350 JCA cases, 2250-3000 SH cases, and the same number of other forms of PPA
    Internet : rheuma-kinderklinik@t-online.de

  27. WU C-J, HUANG J-L, YANG M-H, YAN D-C, OU L-S, HO H-H: Clinical characteristics of juvenile rheumatoid arthritis in Taiwan. Journal of Microbiology, Immunology and Infection 2001, 34:211-214.
    Organism:Dr. J.-L. Huang, Division of Allergy, Department of Pediatrics, Chang Gung Children's Hospital, 5, Fu-Hsin Street, Kueishan, Taoyuan
    Abstract:     This study aimed to investigate the clinical features of juvenile rheumatoid arthritis among Taiwan children. The medical records of 228 children who had juvenile rheumatoid arthritis treated in the Chang Gung Medical Center in Taiwan from 1978 through 1998 were retrospectively reviewed. A total of 146 boys and 82 girls (M:F ratio, 1.8:1) were included in this study. Clinical and laboratory data of these patients were collected from medical charts. Pauciarticular onset (56%) was the most common type of juvenile rheumatoid arthritis, followed by polyarticular (36%) and systemic (8%) type. The positive rate for rheumatoid factor, human leukocyte antigen B27, and antinuclear antibody were 9.2%, 55.2%, and 16.2%, respectively. Uveitis was observed in 5.7% of patients. Compared with previous reports in other regions and populations, remarkably different features of juvenile rheumatoid arthritis were found in this study, which included a higher prevalence among boys than girls, a high positive rate of human leukocyte antigen B27, and a low rate of uveitis