Bibliography December 2001

1. BECHTOLD S, RIPPERGER P, MUHLBAYER D, TRUCKENBRODT H, HAFNER R, BUTENANDT O, SCHWARZ HP: GH Therapy in Juvenile Chronic Arthritis: Results of a Two-Year Controlled Study on Growth and Bone. J.Clin.Endocrinol.Metab 2001, 86:5737-5744.
   Organism:Endocrine Division University Children's Hospital, D-80337 Munich, Germany
   Abstract: Disturbance of growth frequently occurs in children suffering from juvenile chronic arthritis (JCA). Recognition of growth impairment is important because reduced final height is one of the permanent consequences. The aim of this study was to evaluate the efficacy and safety of human GH (hGH) in growth-retarded prepubertal children with JCA. Thirty-five children were tested for GH deficiency (GHD) and randomly assigned to a study and an untreated control group; five were GH deficient and were part of the GHD group. All received glucocorticoids. The study group was treated with 1 IU/kg BW.wk hGH; the GHD group was given 0.5 IU. During 2 yr of hGH treatment growth velocity and height SD score increased compared with baseline values. There was a marked increase in growth velocity in the treated groups, but also some increase in the control group. Plasma levels of IGF-I and IGF-binding protein-3 increased with GH treatment. These results suggest that hGH might be useful in the treatment of growth impairment in JCA. GH may counteract the adverse effects of glucocorticoid therapy, but its effect is dependent on the disease activity. Long-term controlled studies are needed to determine the risks and benefits of GH therapy in JCA
   Internet : PM:11739431

2. BERNTSON L, FASTH A, ANDERSSON-GARE B, KRISTINSSON J, LAHDENNE P, MARHAUG G, NIELSEN S, PELKONEN P, SVENSSON E: Construct validity of ILAR and EULAR criteria in juvenile idiopathic arthritis: A population based incidence study from the Nordic countries. Rinsho Ganka 2001, 28:2737-2743.
   Organism:Dr. L. Berntson, Department of Pediatrics, Hospital of Falun, S-79182 Falun
   Abstract: Objective. New classification criteria (ILAR) have been proposed for juvenile idiopathic arthritis (JIA). They are more descriptive than those formerly used [American College of Rheumatology (ACR), European League Against Rheumatism (EULAR)], but require validation against classifications already in use. We validated the ILAR criteria in relation to the EULAR criteria in a prospective, incidence, and population based setting, and analyzed their feasibility. Methods. Construct validity of ILAR and EULAR classification criteria refers to how closely the 2 instruments are related and how each of them operates in classifying subgroups/categories. Twenty doctors in 5 Nordic countries collected data from the incidence cases within their catchment areas during an 18 month period beginning July 1, 1997. Clinical and serological data from the first year of disease were collected. Results. A total of 322 patients were included. Classification according to the ILAR criteria was possible in 321 patients; 290 patients had a disease duration >= 3 months and were classified according to the EULAR criteria. One child could only be classified according to the EULAR criteria. Thus, 31/322 (9.6%) children were classified according to the ILAR criteria only. Forty-eight of 321 (15%) patients did not fit into any category and 6% (20/321) fulfilled criteria for 2 categories. In the ILAR classification 5 out of 7 categories/subgroups have 2 to 5 specified exclusion criteria that highly discriminate the definition of each patient. In our study the exclusion criteria were fulfilled to only a small extent. Conclusion. The EULAR and ILAR criteria differ concerning the operational definitions of the subvariables involved, which complicates their comparison. By using ILAR rather than EULAR criteria the number of cases with juvenile arthritis increased by 10%, considering the first half-year after onset. The validity of the ILAR criteria is low since they often exclude patients from subgroup classification and the possibility of having more than one diagnosis is not negligible. The specified exclusion criteria for some of the subgroups are difficult to fulfill in clinical work and variables involved could be questioned with regard to their consistency
   Internet : lillemor.berntson@falun.mail.telia.com

3. BROSSART DF, CLAY DL, WILLSON VL: Methodological and statistical considerations for threats to internal validity in pediatric outcome data: response shift in self-report outcomes. J.Pediatr.Psychol. 2002, 27:97-107.
   Organism:Texas A&M University The University of Iowa
   Abstract: OBJECTIVE: To examine conceptual models of response shift, research design, and internal validity issues in the context of longitudinal outcome research using self-report measures such as pediatric quality of life. METHODS: Growth modeling was introduced and illustrated using a previously published data set (Clay, Wood, Frank, Hagglund, & Johnson, 1995) of adjustment in children with juvenile rheumatoid arthritis, diabetes, and healthy controls. RESULTS: Demonstrations revealed that growth modeling may detect response shift and may also model the time and shape of the response shift. CONCLUSIONS: Growth modeling provides one avenue to investigate response shift, thereby addressing an important threat to internal validity in longitudinal outcome research such as quality of life in children with chronic illness
   Internet : PM:11726684

4. HACIMUSTAFAOG c, IL 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:Dr. M. Hacimustafaog(caron)lu, Uludag(caron) University, Faculty of Medicine, Department of Pediatrics, 16059 Bursa
   Internet : mkemal@uludag.edu.tr

5. HITOGLOU S, HATZISTILIANOU M, GOUGOUSTAMOU D, ATHANASSIADOU F, KOTSIS A, CATRIU D: Adenosine deaminase activity and its isoenzyme pattern in patients with juvenile rheumatoid arthritis and systemic lupus erythematosus. Journal of developmental and behavioral.pediatrics 2001, 20:411-416.
   Organism:Dr. S. Hitoglou, Aristotle University of Thessaloniki, Zefxidos 3, 546 22 Thessaloniki
   Abstract: Adenosine deaminase (ADA) is involved in purine metabolism and plays a significant role in the mechanisms of the immune system. The aim of this study was to investigate the activity of total ADA (tADA) and its isoenzymes ADA1 and ADA2 in serum and peripheral blood lymphocytes (PBLs) of children with juvenile rheumatoid arthritis (JRA) and systemic lupus erythematosus (SLE) in different phases of the diseases. The study comprised 34 patients with rheumatic disease, 24 with JRA and 10 with SLE, and 64 healthy controls. The tADA activity and its isoenzymes were measured in serum and PBLs of all patients by the method of Giusti and by the presence or absence of EHNA (erythro-9-(2-hydroxy-3-nonyl)adenine) during the active phase of the disease (before treatment), as well as during remission and relapse. Our data show that increased tADA activity in the serum and PBLs of patients with JRA and SLE is correlated mainly to increased levels of ADA2 activity in serum and ADA1 activity in PBLs. It also closely correlates with clinical disease activity and relapse. The cause of this increased tADA/ADA2 activity in serum and tADA/ADA1 activity in PBLs in JRA and SLE remains to be elucidated. Nevertheless, it may be noted that the measurement of tADA activity, together with ADA2 activity in serum and tADA with ADA1 activity in PBLs, could offer a biochemical approach to the assessment of the pathophysiology of JRA and SLE. Also, tADA and its isoenzymes could be used as alternative parameters representing disease activity
   Internet : nontas@topo.auth.gr

6. IQBAL MP, SULTANA F, MEHBOOBALI N, PERVEZ S: Folinic acid protects against suppression of growth by methotrexate in mice. Biopharmaceutics and Drug Disposition 2001, 22:169-178.
   Organism:M.P. Iqbal, Dept. of Biological/Biomedical Sci., The Aga Khan University, Stadium Road, Karachi 74800
   Abstract: The objective of this study was to investigate whether folinic acid supplementation would protect young mice against suppression of growth by methotrexate (MTX). Four equal groups of Balb/c young male mice (5 animals in each group; mean +/- SD body weight 9.64 +/- 0.85 g, in their rapid growth phase) were subjected to the following drug treatment: One group was given MTX (3.5 mg/kg body weight) intraperitoneally on every 2nd day, another received folinic acid (7.0 mg/kg body weight) intraperitoneally every 2nd day. The third group was given both of these drugs (MTX on every 2nd day and folinic acid 8h post-MTX injection). The fourth group was injected with physiological saline every other day to serve as a control group. Total body weight, food and water consumption by animals in each group were monitored every second day for a period of 3 weeks. After this period mice were sacrificed and liver, spleen and kidneys were excised, weighed and analyzed for MTX and dihydrofolate reductase activity. A small segment of the proximal part of small intestine and small pieces of liver and kidney were also removed to study morphological changes. Compared to the groups, which received folinic acid alone, folinic acid plus MTX or physiological saline, mean increase in body weight (6.8 +/- 0.8 g) of mice over a period of 3 weeks was minimal in the group receiving MTX alone (one-way ANOVA p = 0.0001). The mean weights of liver and kidney in this group receiving MTX alone were also found to be significantly less than the mean weights of these organs in the 3 groups (p<0.001). The negative effect on growth of animals appears not only due to malabsorption but inhibition of pathway of de novo DNA synthesis may also be involved. This is supported by loss of villous pattern in small intestine of mice treated with MTX alone and increased accumulation of free MTX and decreased dihydrofolate reductase in the liver of the group receiving MTX alone as compared with the group receiving MTX plus folinic acid. The data indicate that the administration of folinic acid protects mice against suppression of growth by MTX. On the basis of these observations it can be deduced that patients suffering from juvenile rheumatoid arthritis or acute lymphoblastic leukaemia receiving MTX over a long period of time might be at a risk of experiencing short-term suppression of growth, however they could benefit from supplementation with folinic acid. Copyright (c) 2001 John Wiley & Sons, Ltd
   Internet : perwaiz.iqbal@aku.edu

7. JAVIER JIMENEZ-BALDERAS F, TAPIA-SERRANO R, EUGENIA FM, ARELLANO J, BELTRAN A, YAN t, CAMARGO-CORONEL A, FRAGA A: High frequency of association of rheumatic/autoimmune disease and untreated male hypogonadism with severe testicular dysfunction. Arthritis Research 2001, 3:362-367.
   Organism:Dr. F. Javier Jimenez-Balderas, Pregonero 161, CP 01430 Mexico DF
   Abstract: Our goal in the present work was to determine whether male patients with untreated hypogonadism have an increased risk of developing rheumatic/autoimmune disease (RAD), and, if so, whether there is a relation to the type of hypogonadism. We carried out neuroendocrine, genetic, and rheumatologic investigations in 13 such patients and 10 healthy male 46,XY normogonadic control subjects. Age and body mass index were similar in the two groups. Nine of the 13 patients had hypergonadotropic hypogonadism (five of whom had Klinefelter's syndrome [karyotype 47,XXY]) and 4 of the 13 had hypogonadotropic hypogonadism (46,XY). Of these last four, two had Kallmann's syndrome and two had idiopathic cryptorchidism. Eight (61%) of the 13 patients studied had RADs unrelated to the etiology of their hypogonadism. Of these, four had ankylosing spondylitis and histocompatibility B27 antigen, two had systemic lupus erythematosus (in one case associated with antiphospholipids), one had juvenile rheumatoid arthritis, and one had juvenile dermatomyositis. In comparison with the low frequencies of RADs in the general population (about 0.83%, including systemic lupus erythematosus, 0.03%; dermatomyositis, 0.04%; juvenile rheumatoid arthritis, 0.03%; ankylosing spondylitis, 0.01%; rheumatoid arthritis, 0.62%; and other RAD, 0.1%), there were surprisingly high frequencies of such disorders in this small group of patients with untreated hypogonadism (P< 0.001) and very low serum testosterone levels (P= 0.0005). The presence of RADs in these patients was independent of the etiology of their hypogonadism and was associated with marked gonadal failure with very low testosterone levels
   Internet : fjjimenez19@yahoo.com

8. JEDRZEJCZYK-GORAL B, OWCZAREK H, NAHACZEWSKA W, PRUSEK W: Markers of bone turnover in children with juvenile chronic arthritis. Advances in Clinical and Experimental Medicine 2001, 10:157-164.
   Organism:W. Prusek, Klin. Oddzial Pediatr.-Rheumatol., Szpitalu Wojewodzkim J. Babinskiego, pl. 1 Maja 8, 50-043 Wroclaw
   Abstract: Objectives. We studied markers of bone turnover-serum biochemical markers of bone formation (PICP, T-ALP, B-ALP) and of bone degradation (ICTP, DpD) in healthy children and in children with juvenile chronic arthritis of age 3-18. Material and methods. PICP - carboxyterminal propeptide of type I procollagen is reflecting type I collagen formation. About 90% of collagen type I is present in bones. Therefore the serum level of PICP correlated with total bone formation. ICTP - carboxyterminal telopeptide of type I collagen is a degradation product of type I collagen. The serum levels of ICTP correlated with bone degradation. The bone metabolism of children is 12-14 times higher than adults. Therefore the serum levels of biochemical markers of bone turnover are higher than adults. Results. There was differences between serum levels of studed markers in healthy children and in children with juvenile chronic arthritis. In children with disease of age 3-11 there was significantly lower serum levels of T-ALP and B-ALP. In children with active disease of age 3-11 significantly lower serum levels of T-ALP, B-ALP and ICTP were observed than in children with low active disease. In children with active disease in age 12-18 there was only significantly lower B-ALP. The measurement of markers (T-ALP, B-ALP and ICTP) may be used in studing the biochemical and physiological bases of changes in bone turnover in children with juvenile chronic arthritis

9. KUEMMERLE-DESCHNER JB, DAMMANN F, NIETHAMMER D, DANNECKER GE: Stress fractures: Diagnostic pitfalls in juvenile idiopathic arthritis [1]. Rheumatology 2001, 40:1313-1314.
   Organism:J.B. Kuemmerle-Deschner, Universitatskinderklinik Tubingen, Paediatric Rheumatology/Immunology, Department of Paediatrics, Hoppe-Seyler-Strasse 1, 72076 Tubingen

10. LITALIEN C, JACQZ-AIGRAIN E: Risks and Benefits of Nonsteroidal Anti-Inflammatory Drugs in Children: A Comparison with Paracetamol. Paediatr.Drugs 2001, 3:817-858.
    Organism:Service of Pharmacology, Pediatrics and Pharmacogenetics, Hospital Robert Debre, Paris, France
    Abstract: Nonsteroidal anti-inflammatory drugs (NSAIDs) possess antipyretic, analgesic and anti-inflammatory effects. They are frequently used in children and have numerous therapeutic indications, the most common ones being fever, postoperative pain and inflammatory disorders, such as juvenile idiopathic arthritis (JIA) and Kawasaki disease. Their major mechanism of action is through inhibition of prostaglandin biosynthesis by blockade of cyclo-oxygenase (COX). The disposition of most NSAIDs has been mainly studied in infants >/=2 years of age. Compared with adults, the volume of distribution and clearance of NSAIDs such as diclofenac, ibuprofen (infants aged between 3 months and 2.5 years), ketorolac and nimesulide were increased in children. The elimination half-life was similar in children to that in adults. These pharmacokinetic differences might be clinically significant with the need for higher loading and/or maintenance doses in children. Ibuprofen, acetylsalicylic acid (ASA) and acetaminophen are the most frequently used agents for fever reduction in children. Over the past 20 years, because of the association between ASA use and Reye's syndrome, most of the interest has been directed toward ibuprofen and acetaminophen. In view of its comparable antipyretic efficacy, but superior tolerability profile, acetaminophen, when used appropriately with age-adapted formulations, should remain the first-line therapy in the treatment of childhood fever. At the moment, there is no scientific evidence to recommend simultaneous use of these two antipyretic drugs. Most NSAIDs provide mild to moderate analgesia, with the exception of ketorolac which has a strong analgesic activity. The analgesic efficacy of ketorolac, ketoprofen, diclofenac and ibuprofen in the treatment of postoperative pain has been mainly studied following a single dose, in children of >/=1 year of age undergoing minor surgeries. In this setting, when used either alone or in adjunct to caudal or epidural anaesthesia, they were associated with an opioid-sparing effect and were well tolerated. With the exception of ketorolac use in children undergoing tonsillectomy, where controversy exists regarding the risk of postoperative haemorrhage, NSAIDs have not been associated with an increased risk of perioperative bleeding. NSAIDs are the first-line therapy in JIA. They appear to be equally effective and tolerated, with the exception of ASA which is associated with more adverse effects. ASA has been used for many years in the treatment of Kawasaki disease and is part of the standard modality of treatment in combination with intravenous gammaglobulins. More recently, lung inflammation associated with cystic fibrosis (CF) has become a new target for NSAIDs. Despite promising preliminary results with ibuprofen, numerous questions need to be answered before this new strategy becomes part of the conventional treatment of patients with CF. In summary, NSAIDs are effective in reducing fever, alleviating pain and reducing inflammation in children, with a good tolerance profile. Pharmacokinetic studies are needed to characterise the disposition of NSAIDs in very young infants in order to use them rationally. To date, no studies have been published on the disposition, tolerability and efficacy of specific COX-2 inhibitors in children. Further clinical experience with these agents in adults is warranted before undergoing trials with specific COX-2 inhibitors in children
    Internet : PM:11735667

11. MANAL K, LU X, NIEUWENHUIS MK, HELDERS PJ, BUCHANAN TS: Force transmission through the juvenile idiopathic arthritic wrist: a novel approach using a sliding rigid body spring model. J.Biomech. 2002, 35:125-133.
    Organism:Center for Biomedical Engineering Research, University of Delaware, 126 Spencer Laboratories, 19716, Newark, DE, USA
    Abstract: Force transmission across the wrist during a grasping maneuver of the hand was simulated for three children with juvenile idiopathic arthritis (JIA) and for one healthy age-matched child. Joint reaction forces were estimated using a series of springs between articulating bones. This method (i.e., rigid body spring modeling) has proven useful for examining loading profiles for normally aligned wrists. A novel method (i.e., sliding rigid body spring modeling) designed specifically for studying joint reaction forces of the malaligned JIA wrist is presented in this paper. Loading profiles across the wrist for the unimpaired child were similar using both spring modeling methods. However, the traditional fixed-end method failed to converge to a solution for one of the JIA subjects indicating the sliding model may be more suitable for investigating loading profiles of the malaligned wrist. The results of this study suggest that a larger proportion of force is transferred through the ulno-carpal joint of the JIA wrist than for healthy subjects, with a less than normal proportion of force transferred through the radio-carpal joint. In addition, the ulnar directed forces along the shear axis defined in this study were greater for all three JIA children compared to values for the healthy child. These observations are what were hypothesized for an individual with JIA of the wrist
    Internet : PM:11747891

12. MERINO R, DE INOCENCIO J, GARCIA-CONSUEGRA J: Evaluation of ILAR classification criteria for juvenile idiopathic arthritis in Spanish children. Rinsho Ganka 2001, 28:2731-2736.
    Organism:Dr. R. Merino, Unit of Pediatric Rheumatology, University Hospital La Paz, Po de la Castellana 265, 28046 Madrid
    Abstract: Objective. To evaluate the proposed International League of Associations for Rheumatology (ILAR) classification criteria for juvenile idiopathic arthritis in a cohort of Spanish children. Methods. One hundred twenty-five patients with chronic arthritis were categorized according to one of the traditional classifications and the proposed ILAR classification system after at least 6 months of disease. The traditional classifications included the European League Against Rheumatism (EULAR) criteria for pauciarticular, polyarticular rheumatoid factor (RF) negative, and systemic juvenile chronic arthritis (JCA), as well as for RF+ polyarthritis; the Vancouver criteria for juvenile psoriatic arthritis (JPsA); and the European Spondylarthropathy Study Group (ESSG) preliminary criteria for juvenile spondyloarthropathy (JSpA). Results. The ILAR criteria classified 106/125 patients (84.8%). All patients with systemic and polyarticular JCA, RF+ polyarthritis, and definite juvenile psoriatic arthritis were reclassified in the corresponding ILAR category. In contrast, only 80% of pauciarticular JCA and 47% of JSpA patients could be allocated to the ILAR oligoarthritis (47/59 patients, 35 persistent and 12 extended) and enthesitis related arthritis (ErA, 8/17 patients) categories. Two children with probable PsA were reclassified in the

13. NAKAI K, YAMAMOTO S, NAKAGAWA Y, OHGURO N, OKADA AA, NAGAE Y, TANO Y: A case of Behc(cedil)et's disease in an 11-year-old boy with a 7-year history of aphthae. Folia Ophthalmologica Japonica 2001, 52:601-604.
    Organism:Dr. K. Nakai, Dept of Ophthalmol, Kansai Rosai Hosp, 3-1-69 Inabaso, Amagasaki 660-8511
    Abstract: We report a case of Behc(cedil)et's disease in an 11-year-old boy. The patient had a history of oral aphthae diagnosed by a pediatrician when the patient was 4 years old. At the age of 7 years he developed genital ulcers, then at the age of 8 years he developed erythema nodosum on both legs and fever. Serum testing at that time was positive for rheumatoid factor. The diagnosis of juvenile rheumatoid arthritis (JRA) was made and the patient underwent systemic corticosteroid therapy. The patient then presented at the age of 9 years with iridocyclitis and hypopyon in the left eye. Despite topical and systemic corticosteroid therapy, light perception was lost in that eye over the course of the next year. At the age of 11 years, the patient presented to Osaka University Hospital with vision in the right eye that was reduced to hand motion. Clinical examination revealed retinal vasculitis, infiltrates, and hemorrhage. Vitrectomy was performed for vitreous sampling, and viral infection was ruled out by polymerase chain reaction testing. Human lymphocyte antigen (HLA) typing was positive for HLA-B-51. On a presumptive diagnosis of Behc(cedil)et's disease, we initiated systemic therapy with cyclosporin while decreasing the dose of corticosteroid medication. After 1 year of this treatment regimen, the patient now has vision in the right eye of 0.9 with marked decrease in posterior segment inflammation. Although Behc(cedil)et's disease generally presents in adults, the diagnosis must be considered in young children with unexplained retinal vasculitis in the setting of systemic manifestations of the disease

14. ROHAYEM J, LEUPOLD W, PAUL KD, GAHR M: Pulmonary fibrosis and other clinical manifestations of small vessel vasculitis in a family with seropositive juvenile rheumatoid arthritis. Pediatr.Pulmonol. 2002, 33:65-70.
    Organism:Department of Pediatrics, University Hospital of Dresden, Dresden, Germany
    Abstract: We report on a family (mother, daughter, and son) suffering from progressive pulmonary fibrosis associated with deforming arthritis, sinusitis, glomerulonephritis, and cutaneous vasculitis. We suggest that these clinical features display the variable expressions of small-vessel vasculitis in juvenile rheumatoid arthritis. Copyright 2002 Wiley-Liss, Inc
    Internet : PM:11747262

15. SAVOLAINEN HA, LEIRISALO-REPO M: Eosinophilia as a side-effect of methotrexate in patients with chronic arthritis. Journal of developmental and behavioral.pediatrics 2001, 20:432-434.
    Organism:A. Savolainen, Rheumatism Foundation Hospital, 18120 Heinola
    Abstract: This report describes isolated reversible eosinophilia without additional subjective symptoms or signs in three patients on methotrexate therapy, two of them with juvenile idiopathic arthritis and one with rheumatoid arthritis

16. SOYLU A, TURKMEN M, KAVUKC c, OZER E, CANDA T: Familial arthropathy with camptodactyly: Reports of two families. Turk.J.Pediatr. 2001, 43:356-361.
    Organism:A. Soylu, Department of Pediatrics, Dokuz Eylul University, Faculty of Medicine, Izmir
    Abstract: Familial association of congenital camptodactyly and arthropathy without evidence of concurrent inflammation has an autosomal recessive pattern of inheritance. We describe four children born to consanguineous parents in two families with congenital camptodactyly and polyarthropathy which were misdiagnosed and treated as juvenile rheumatoid arthritis (JRA) for some time. The siblings in the second family also had fibrosing pleuritis. Histopathological examination of the synovial tissues of the children in the first family revealed synovial hypertrophy and presence of multinucleated giant cells with minimal inflammation and vasculitis. On the other hand, prominent fibrosis with no inflammation was present in the synovial tissue of the elder boy in the second family. Thus, while the children in the first family had the phenotypic characteristics of congenital familial hypertrophic synovitis, the latter siblings probably represent a form of the familial fibrosing serositis

17. UNSAL E, GULAY Z, GUNAL I: The association of HLA-DR4 antigen with juvenile chronic arthritis and slipped capital femoral epiphysis. Arch.Orthop.Trauma.Surg. 2001, 121:571-573.
    Organism:I. Gunal, Department of Orthopaedics, Dokuz Eylul University, School of Medicine, Koruturk Mah. Ruzgar Sok. No: 51/13, 35330 Balc(cedil)ova Izmir
    Abstract: Seventeen children who met the criteria for juvenile chronic arthritis (JCA) were reviewed. Throughout the study, the clinical examination, HLA phenotyping, and radiological assessment of the hips were performed by separate authors who were blinded to other data. At the end of the study, the results were also compared with 25 healthy, age- and sex-matched children. Six of the children with JCA also had radiological signs of slipped capital femoral epiphysis (SCFE; five with minimal slip pattern, one with moderate slip), and five of them had DR4 in their genotypes, in contrast to the remaining 11 patients who did not (p < 0.001). On the other hand, only 2 of 25 children in the control group had DR4 (p < 0.01). The difference was not significant when the patients without SCFE were compared with the control group (p = 1.0). The relative risk of cases with DR4 antigen for SCFE, was 57.5, while it was below 1 for the other antigens. These results suggest that although DR4 is not specific for JCA, it is the common HLA antigen for those who have SCFE, and patients with JCA and HLA-DR4 antigen should be examined for evidence of SCFE, which was not reported before to exist with JCA
    Internet : izge.gunal@deu.edu.tr

18. WEDDERBURN LR, PATEL A, VARSANI H, WOO P: Divergence in the degree of clonal expansions in inflammatory T cell subpopulations mirrors HLA-associated risk alleles in genetically and clinically distinct subtypes of childhood arthritis. International Immunology 2001, 13:1541-1550.
    Organism:L.R. Wedderburn, Rheumatology Unit, Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH
    Abstract: Clinically distinct forms of childhood arthritis are associated with different risk alleles of polymorphic loci within the MHC, which code for the antigen-presenting class I or class II molecules. We have compared the TCR diversity of synovial T cells from children with enthesitis-related (HLA-B27SUP+) arthritis and oligoarticular arthritis (with class II MHC risk allele associations) in parallel with peripheral blood T cells from each child, using a high-resolution heteroduplex TCR analysis. We demonstrate that multiple clonal T cell expansions are present and persistent within the joint in both groups, but that there is disease-specific divergence in the dominant T cell subset containing these expansions. Thus, the largest clonotypes within the inflamed joints of children with class II-associated arthritis are within the CD4SUP+ synovial T cell population, while the dominant clones from children with enthesitis-related arthritis (associated with a class I allele) are within the CD8SUP+ synovial T cell population. These data provide powerful data to support the concept that recognition of MHC-peptide complexes by T cells plays a role in the pathogenesis of juvenile arthritis
    Internet : L.Wedderburn@ich.ucl.ac.uk