Bibliography July 2006

1. AL HAGGAR M, RAGAB M, AWAD Met AL BAZ R: Soluble adhesion molecules in juvenile idiopathic arthritis: Relation to activity and clinical subtype|, 2006
   Organism:The present research aimed to evaluate serum Adhesion Molecules (AMs) in patients with Juvenile Idiopathic Arthritis (JIA) to correlate their values with disease activity in different clinical subtypes. Serum levels of some soluble AMs (E-selectin, sICAMSUB1 and sVCAMSUB1) were assayed by ELISA in 37 patients with JIA both during activity and after remission. Other activity parameters like sedimentation rate and leukocytic counts were tested as well. Twenty healthy children of matched age and sex were taken as control. Serum E-selectin was found significantly higher in JIA compared to control (in all subtypes across all disease stages), with signifieant drop after remission, yet not reached the normal values. These changes were more evident in systemic JIA compared to other subtypes. Serum ICAMSUB1 and VCAMSUB1 showed the same changes in relation to control and to the disease activity. We can conclude that systemic JIA is associated with higher levels of soluble AMs thus explaining the perpetual inflammatory process and hence the remissions and exacerbations which are usually associated with higher morbidity in systemic JIA than in the other subtypes. We recommend following JIA patients until laboratory remission (normalization of serum AMs) to correlate AMs levels to clinical course aiming to put forward a therapeutic plan

2. AL MAYOUF SM, MADI SM, ALMANE Ket AL JUMMAH S: Comparison of clinical and laboratory variables in familial versus sporadic systemic onset juvenile idiopathic arthritis, J.Rheumatol., Vol. 33(3), 597-600., 2006
   Organism:Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia mayouf@kfshrcedusaFAU - Al-Mayouf, Sulaiman M
   Abstract:OBJECTIVE: To compare patients with familial versus sporadic juvenile idiopathic arthritis (JIA) with respect to clinical and laboratory variables. METHODS: The familial JIA group comprised 11 affected siblings belonging to 4 families, while the comparative group comprised 22 patients selected by systematic sampling from JIA patients presenting to our pediatric rheumatology clinic; the first patient was chosen randomly and the subsequent patients chosen at intervals of 3. The 2 groups were compared with respect to demographic information, age at onset of disease, disease activity, disease damage, and laboratory variables. RESULTS: The 2 groups were comparable with respect to age, sex, and onset type of disease. All patients from the familial group were from a southern province of Saudi Arabia (p = 0.001). The familial group had an earlier age at onset of disease (p = 0.039), the mean number of actively inflamed joints was higher (p = 0.009), and functional capacity as measured by Childhood HAQ was worse (p = 0.048), compared with the sporadic group. Other variables showed no significant differences. CONCLUSION: The comparison of patients with familial versus sporadic JIA revealed a significant difference in origin of patients and age at onset of disease. These differences may be helpful in identifying the predisposing genes in familial patients with JIA

3. AL MAYOUF SM, MADI SM, ALMANE Ket AL JUMMAH S: Comparison of clinical and laboratory variables in familial versus sporadic systemic onset juvenile idiopathic arthritis, Rinsho Ganka, Vol. 33, 597-600., 2006
   Organism:King Faisal Specialist Hosp and Res Ctr, Dept Pediat, MBC 58,POB 3354, Riyadh 11211, Saudi Arabia Saudi Arabia
   Abstract:Objective. To compare patients with familial versus sporadic juvenile idiopathic arthritis (JIA) with respect to clinical and laboratory variables.Methods. The familial JIA group comprised 11 affected siblings belonging to 4 families, while the comparative group comprised 22 patients selected by systematic sampling from JIA patients presenting to our pediatric rheumatology clinic; the first patient was chosen randomly and the subsequent patients chosen at intervals of 3. The 2 groups were compared with respect to demographic information, age at onset of disease, disease activity, disease damag e, and laboratory variables.Results. The 2 groups were comparable with respect to age, sex, and onset type of disease. All patients from the familial group were from a southern province of Saudi Arabia (p = 0.001). The familial group had an earlier age at onset of disease (p = 0.039), the mean number of actively inflamed joints was higher (p = 0.009), and functional capacity as measured by Childhood HAQ was worse (p = 0.048), compared with the sporadic group. Other variables showed no significant differences.Conclusion. The comparison of patients with familial versus sporadic JIA revealed a significant difference in origin of patients and age at onset of disease. These differences may be helpful in identifying the predisposing genes in familial patients with JIA

4. APRIL KT, FELDMAN DE, PLATT RWet DUFFY CM: Comparison between children with juvenile idiopathic arthritis and their parents concerning perceived treatment adherence, Arthritis Rheum., Vol. 55(4), 558-563., 2006
   Organism:Universite de Montreal, Montreal, Quebec, Canada
   Abstract:OBJECTIVE: Adherence to treatment in juvenile idiopathic arthritis (JIA) may be associated with better outcomes. Clinicians must be aware of possible divergence between parents and children regarding adherence, in order to gain a better understanding of adherence and factors associated with it. The objective was to determine the level of agreement between children with JIA and their parents concerning perception of the child's adherence to the treatment regimen (for both medications and exercises). METHODS: Fifty patients and their parents, who attended the JIA clinic at the Montreal Children's Hospital, completed the Child Adherence Report Questionnaire and the Parent Adherence Report Questionnaire. Paired t-tests were used to compare parents' and children's scores for adherence questions and agreements were analyzed by intraclass correlation coefficients (ICCs). RESULTS: Parents reported that their children showed more negative reactions to taking medication and doing exercises, more helpfulness from the medication, and more difficulty to carry out the exercise program than their children reported. ICCs (95% confidence interval) for medications and exercises were, respectively, 0.32 (0.04, 0.56) and 0.77 (0.61, 0.87) for overall adherence, 0.33 (0.05, 0.57) and 0.39 (0.09, 0.62) for perceived difficulty to following treatment, and 0.37 (0.09, 0.60) and 0.45 (0.17, 0.67) for how often children had negative reactions following treatment. Levels of agreement for perceived helpfulness of treatments were quite low. CONCLUSION: Agreement between parents and children concerning adherence was at best moderate, and generally better for the exercise program than for prescribed medications

5. AVOUAC J, GOSSEC Let DOUGADOS M: Diagnostic and predictive value of anti-cyclic citrullinated protein antibodies in rheumatoid arthritis: a systematic literature review, Ann.Rheum.Dis., Vol. 65(7), 845-851., 2006
   Organism:Rene Descartes University, Medicine Faculty, and APHP Cochin Hospital, Rhumatologie B Department, Paris, FranceFAU - Avouac, J
   Abstract:OBJECTIVE: To evaluate the two generations of anti-cyclic citrullinated protein (CCP) antibodies as a diagnostic marker of rheumatoid arthritis (RA) and as a predictor of future development of RA in healthy subjects and in patients with early undifferentiated arthritis. METHODS: A systematic analysis of the literature published between 1999 and February 2006 was conducted. Data were collected on the sensitivity and specificity of the two generations of anti-CCP antibodies for diagnosing RA and predicting future development of the disease. RESULTS: Among 107 studies initially identified, 68 had interpretable data and were analysed. Diagnostic properties were assessed in 58 studies: mean (SD) sensitivity was 53 (10)% (range 41-68) and 68 (15)% (range 39-94) for anti-CCP1 and anti-CCP2, respectively; mean (SD) specificity was 96 (3)% (range 90-99) and 95 (5)% (range 81-100) for anti-CCP1 and anti-CCP2, respectively. Predictive properties were assessed in 14 studies; odds ratio (95% confidence interval) of anti-CCP1 and anti-CCP2 for the future development of RA were 20 (14 to 31) and 25 (18 to 35), respectively, among patients with early undifferentiated arthritis and 64.5 (8.5 to 489) and 28 (8 to 95), respectively, among healthy subjects. CONCLUSION: Sensitivity of the second generation of anti-CCP is close to that of rheumatoid factor, with a higher specificity, for distinguishing RA from other rheumatic diseases. Moreover, anti-CCP antibodies appear to be highly predictive of the future development of RA in both healthy subjects and patients with undifferentiated arthritis

6. BEALS RKet SAUSER DD: Nontraumatic disorders of the clavicle, J.Am.Acad.Orthop.Surg., Vol. 14(4), 205-214., 2006
   Organism:Department of Orthopaedics and Rehabilitation, Oregon Health Sciences University, Portland, OR 97239, USAFAU - Beals, Rodney K
   Abstract:Other than those resulting from trauma and arthritis, disorders of the clavicle are uncommon. Some nontraumatic disorders are found only in infancy and childhood, such as birth fracture, infantile cortical hyperostosis, congenital pseudarthrosis, cleidocranial dysplasia, and short clavicle syndrome. Other nontraumatic disorders occur in both children and adults; these include anterior subluxation of the sternoclavicular joint, Friedrich's disease, hypertrophic osteitis, chronic multifocal periosteitis and arthropathy, and osteomyelitis. Some nontraumatic clavicular disorders are found only in adults, such as distal osteolysis. Because the description and nomenclature of these disorders arise from several medical disciplines, they often are confusing. Until clear, distinguishing features are described, it is advisable to combine some of the entities. This is especially true of the nonsuppurative inflammatory disorders of the clavicle, which appear to fall under the heading of spondyloarthropathy. Treatment varies by disorder and may include symptomatic and expectant management, drug therapy, and nonsurgical or surgical treatment

7. BEJIA I, LAATAR A, BEN SALEM K, TOUZI M, BERAOUI Net ZAKRAOUI L: [Validation of the Tunisian version of the Health Assessment Questionnaire (HAQ) in rheumatoid arthritis patients], Tunis Med., Vol. 84(3), 155-160., 2006
   Organism:Service de rhumatologie EPS Monastir, Hopital Mongi Slim La MarsaFAU - Bejia, Ismail
   Abstract:METHODS: One hundred twenty two rheumatoid arthritis patients were consecutively included in the study. Test-retest reliability was assessed in 61 patients based on the intra-class correlation coefficient. RESULTS: for the 122 patients (104 female and 18 male) the median age was 47 years (18-70). The mean age of the patients who filled in the questionnaire at test and retest times was 45 years (18-70). Test- retest reliability of the HAQ was 0.84. Internal consistency was 0.94. There was a good correlation between the HAQ and the Lee index (r = 0.75, p <10(-4)), the HAQ and the RAQoL (rs = 0.96, p <10(-4)). In a logistic regression model Lee index, RAQoL and age account for the variance of the HAQ. CONCLUSION: The Tunisian version of the HAQ preserves the metrological properties of the original version and can be used for measuring and following functional abilities of Tunisian rheumatoid arthritis patients

8. BINSTADT BA, LEVINE JC, NIGROVIC PA, GAUVREAU K, DEDEOGLU F, FUHLBRIGGE RC, WEINDLING SN, NEWBURGER JWet SUNDEL RP: Coronary artery dilation among patients presenting with systemic-onset juvenile idiopathic arthritis|, 2005
   Organism:Objective. To evaluate coronary artery diameters among patients presenting with systemic-onset juvenile idiopathic arthritis (SoJIA). Methods. Fifty cases of SoJIA were reviewed. At the time of initial presentation with fever, 12 patients had echocardiograms that included a complete evaluation of the coronary arteries. A single reviewer measured the diameters of the left main, proximal left anterior descending, and proximal right coronary arteries. Body surface area-adjusted z scores were calculated with respect to a normative population. Results. Coronary artery dilation (z score: >2) was obs erved for 5 of the 12 patients with SoJIA who had echocardiograms performed at the time of presentation with fever. No patient developed a coronary artery aneurysm, and all of the coronary artery z scores normalized within 4 months. Only 2 of the 5 patients with coronary artery z scores of >2 fulfilled the clinical criteria for Kawasaki disease, the most commonly recognized cause of coronary artery dilation among children. Conclusions. Children presenting with SoJIA may have coronary artery dilation similar to that observed for children with Kawasaki disease. These data suggest that the presence of coronary artery dilation on initial echocardiograms for patients with fever does not exclude the diagnosis of SoJIA. Copyright (c) 2005 by the American Academy of Pediatrics

9. BRUNNER HI, MUELLER M, RUTHERFORD C, PASSO MH, WITTE D, GROM A, MISHRA Jet DEVARAJAN P: Urinary neutrophil gelatinase-associated lipocalin as a biomarker of nephritis in childhood-onset systemic lupus erythematosus, Arthritis Rheum., Vol. 54(8), 2577-2584., 2006
   Organism:Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
   Abstract:OBJECTIVE: Renal involvement in systemic lupus erythematosus (SLE) is associated with poor prognosis. Currently available renal biomarkers are relatively insensitive and nonspecific for diagnosing SLE nephritis. Previous research suggests that neutrophil gelatinase-associated lipocalin (NGAL) is a high-quality renal biomarker of acute kidney injury, while its usefulness in SLE is unclear. We undertook this study to determine the relationship between urinary NGAL excretion and SLE disease activity or damage, with a focus on nephritis. METHODS: A cohort of 35 patients diagnosed as having SLE prior to age 16 years (childhood-onset SLE) was assessed for disease activity (using the Systemic Lupus Erythematosus Disease Activity Index 2000 update) and damage (using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology SLE Damage Index) in a double-blind, cross-sectional study. Information on current markers of renal function and disease was obtained and compared with NGAL levels (ng/mg of urinary creatinine) measured by enzyme-linked immunosorbent assay. Eight children with juvenile idiopathic arthritis (JIA) served as controls. RESULTS: NGAL levels did not differ with the age, weight, height, sex, or race of the patients. Patients with childhood-onset SLE had significantly higher NGAL levels than did those with JIA (P < 0.0001). NGAL levels were strongly to moderately correlated with renal disease activity and renal damage (Spearman's r >/= 0.47, P < 0.0001 for both comparisons), but not with extrarenal disease activity or extrarenal damage. NGAL levels of >0.6 ng/mg urinary creatinine were 90% sensitive and 100% specific for identifying childhood-onset SLE patients with biopsy-proven nephritis. CONCLUSION: Urinary NGAL is a promising potential biomarker of childhood-onset SLE nephritis. The results of the current study require validation in a larger cohort to more accurately delineate urinary NGAL excretion in relation to the diverse SLE phenotypes

10. BUTBUL-AVIEL Y, KOREN A, HALEVY Ret SAKRAN W: Procalcitonin as a diagnostic aid in osteomyelitis and septic arthritis, Pediatr.Emerg.Care., Vol. 21(12), 828-832., 2005
    Organism:Pediatric Department B, Ha'Emek Medical Center, Afula, IsraelFAU - Butbul-Aviel, Yonatan
    Abstract:OBJECTIVES: Plasma procalcitonin (PCT) increases rapidly during bacterial infections but remains low in viral infections and other inflammatory processes. High plasma PCT typically occurs in children with bacterial meningitis, severe bacterial infections, particularly in cases of septic shock or bacteremia, and in renal parenchymal damage. The aim of this study was to test the usefulness of plasma PCT analysis in the diagnosis of osteomyelitis, septic arthritis, and other skeletal inflammatory diseases in pediatric patients admitted because of fever and limping. METHODS: White blood cell count, erythrocyte sedimentation rate, C-reactive protein, and PCT levels were measured in children admitted to the pediatric department with fever, limping, and suspected osteomyelitis or septic arthritis. PCT levels were measured by an immunochromatography assay, based on monoclonal and polyclonal antibodies against katacalcin. RESULTS: Forty-four children were evaluated: 12 (27.3%) were diagnosed with osteomyelitis, 11 (25%) had septic arthritis, 5 children (11.4%) were diagnosed as a soft tissue infection, and transient synovitis or reactive arthritis was diagnosed in another 6 children (13.6%). Four children (9.1%) were diagnosed as having juvenile rheumatoid arthritis, and 6 (13.6%) with different diseases. PCT value was elevated in 7 patients (58.3%) with osteomyelitis, and only 3 children (27.2%) with the diagnosis of septic arthritis had a mildly elevated value. Among the children with other diagnosis, there were no positive PCT values (P < 0.001 between skeletal infection and all other diagnosis). CONCLUSIONS: In this study, PCT was found to be a useful marker in the diagnosis of osteomyelitis and not in septic arthritis. A larger group of patients needed to be studied to confirm our findings

11. CARRICK DM, CHULADA P, DONN R, FABRIS M, MCNICHOLL J, WHITWORTH Wet BLACKSHEAR PJ: Genetic variations in ZFP36 and their possible relationship to autoimmune diseases, J.Autoimmun., Vol. 26, 182-196., 2006
    Organism:Westat Corp, 1500 Res Blvd,TB 206, Rockville, MD 20850 USA USA
    Abstract:The ZFP36 gene codes for TTP, a regulator of TNF alpha. In mice, TTP deficiency results in a systemic autoimmune inflammatory syndrome with severe arthritis. We hypothesized that genetic variations in ZFP36 are associated with autoimmune disease in humans. The primary objective of this study was to identify human ZFP36 genetic variants in autoimmune disease cases and controls, determine their frequencies in a general clinic population, and construct haplotypes. We resequenced ZFP36 in 316 individuals with autoimmune diseases and identified 28 polymorphisms and determined the frequency of all the known ZFP36 polymorphisms in 484 participants of the Environmental Polymorphism Registry, a regional registry being conducted by the NIEHS. Based on the sequence-verified ZFP36 genotypes, 34 haplotypes were constructed. As a secondary objective, we examined autoimmune disease cases and controls for potential ZFP36 genetic associations. One novel polymorphism, ZFP36*8, a C to T transition in the protein coding domain, was significantly associated with rheumatoid arthritis (RA) in African-Americans (RR = 1.23, 95% CI: 1.11-1.36). The data presented here suggest a tentative association between ZFP36 and RA. This finding, as well as the ZFP36 polymorphisms and haplotypes identified here, should form the basis for future association studies in autoimmune diseases. Published by Elsevier Ltd

12. CARVOUNIS PE, HERMAN DC, CHA Set BURKE JP: Incidence and outcomes of uveitis in juvenile rheumatoid arthritis, a synthesis of the literature, Graefes Arch.Clin.Exp.Ophthalmol., Vol. 244(3), 281-290., 2006
    Organism:Mayo Clinic College of Medicine, Mayo Clinic, Rochester, MN 55905, USAFAU - Carvounis, Petros E
    Abstract:BACKGROUND: Juvenile rheumatoid arthritis (JRA) is the most common systemic cause of pediatric uveitis in Europe and North America. Uveitis is commonly perceived as a frequent sequela of JRA and JRA-associated uveitis is commonly considered to have a complicated course with frequent adverse visual outcomes. METHODS: We performed a systematic literature search for series of consecutive patients with JRA (as defined by the American College of Rheumatology criteria) reporting on the frequency of uveitis and/or complications of uveitis, published between January 1980 and December 2004. The main outcome measures were: the cumulative incidence of uveitis in JRA, the cumulative incidence of adverse visual outcome and that of complications in JRA-associated uveitis. Additionally, the influence of gender, presence of antinuclear antibody (ANA) and disease onset subtype to the likelihood of developing uveitis were examined. RESULTS: Analysis of pooled data from the 26 eligible series suggested a cumulative incidence of uveitis in JRA of 8.3% [95% confidence intervals (CI), 7.5-9.1%]. The cumulative incidence of uveitis varied according to geographic location, being highest in Scandinavia, then the US, then Asia and lowest in India. JRA-associated uveitis was more common in pauciarticular than polyarticular onset patients [odds ratio (OR) = 3.2, 95% CI, 2.33-4.36] and in ANA-positive than ANA-negative patients (OR = 3.18, 95% CI, 2.22-4.54). Female gender was only a weak risk factor for the development of uveitis in JRA patients (OR = 1.69, 95% CI 1.09-2.62) and was not statistically significant after considering disease onset subtypes. In JRA-associated uveitis the cumulative incidence of cumulative incidence of adverse outcome (visual acuity < 20/40 OU) was 9.2% (95% CI: 4.7-15.8) of cataracts 20.5% (95% CI: 15.5-26.3), of glaucoma 18.9% (95% CI: 14.4-24.2) and of band keratopathy 15.7% (95% CI: 10.9-21.7). CONCLUSION: The cumulative incidence of uveitis in JRA varies according to geographic location, presence of ANA, type of JRA onset and gender. Uveitis, adverse visual outcome, and complications in JRA are less frequent than commonly accepted

13. CELIK A, SAGLAM F, DOLEK D, SIFIL A, SOYLU A, CAVDAR C, TEMIZKAN A, BORA S, GULAY Het CAMSARI T: Outcome of kidney transplantation for renal amyloidosis:a single-center experience, Transplant.Proc., Vol. 38(2), 435-439., 2006
    Organism:Division of Nephrology, Dokuz Eylul University Medical School, Izmir, Turkey alicelik@deuedutrFAU - Celik, A
    Abstract:The aim of this retrospective study was to investigate the results of kidney transplantation in patients with renal amyloidosis. We analyzed the results of renal transplantation in 13 amyloidotic transplant recipients compared with those in a control group of 13 nonamyloidotic patients. While the etiology of amyloidosis was rheumatoid arthritis in one patient, in all of the others it was secondary to familial Mediterranean fever. Acute rejection episodes developed once in six and twice in one patient. The renal function in these patients was improved by antirejection treatment. Chronic rejection did not develop in any patient. However six patients (46%) died due to various complications despite functional grafts. The others are still being followed with well-functioning grafts. Among the control group, acute and chronic rejection were diagnosed in three and two patients, respectively: one patient returned to hemodialysis after 26 months of transplantation, while the others are still alive with functional grafts. There was no death in the control group. The 5- and 10-year actuarial patient survival rates of the amyloidosis and control groups were 52.2%, 26.6%, and 100%, 100%, respectively (P = .002). However, the graft survivals of the amyloidosis versus control groups were 100%, 100%, versus 87.5%, 87.5, respectively (P = .47). In conclusion, we observed a high rate of early mortality among recipients with amyloidosis associated with infectious complications. Moreover, patient survivals were lower among amyloidotic renal recipients

14. CEPUCH Get WORDLICZEK J: Level of satisfaction and feeling of sense of life in adolescents with cancer and rheumatic disease|, 2006
    Organism:Background. Cancer and rheumatic disease and their biopsychosocial consequences may affect feeling of sense in life of young patients. Intensity of emotional states and their changeability often blur borders between physical and psychical pain. It disturbs past lifestyle in evident way and threatens basic values, past social roles, acquired concept of world and individual's own identity. Sense of aim and sense of life become particularly important for a young person entering adulthood which is disturbed by chronic illness and connected with difficult experiences. Material and methods. The study included 124 male and female adolescents, aged 14 to 20, hospitalized because of cancer (group I - 82 patients) and juvenile rhematoid arthritis (group II - 42 patients). Pain intensity level was measured with Visual-Analogue Scale VAS. Emotional and sensory aspect of pain was assessed with Polish version of Melzack's Questionnaire. Satisfaction with life was evaluated with Cantril's ladder, and feeling of sense of life and sense of aim were assessed with the Purpose In Life Test. Results. Adolescents presented high level of satisfaction with their lives both present and predicted. They had sense of aim and sense of life regardless of the group they belonged to. Conclusion. No significant relationships between intensity of pain experience and satisfaction with life were found. This result may be affected with low intensity of experienced pain. Young patients had sense of aim and life. However, values' hierarchy and life priorities were influenced by disease unit and sense of life threat connected with it

15. CORTIS Eet INSALACO A: Macrophage activation syndrome in juvenile idiopathic arthritis|, 2006
    Organism:Macrophage activation syndrome (MAS) is a rare and potentially lethal complication of chronic rheumatic diseases of childhood, in particular of systemic-onset juvenile idiopathic arthritis (s-JIA), resulting from uncontrolled activation and proliferation of T lymphocytes and macrophages. The onset, acute and dramatic, may mimic a flare of the underlying disease or a severe sepsis. Diagnosis is difficult and, until now, no specific criteria have been developed. Laboratory data show pancytopenia, coagulopathy, low ESR and low concentrations of serum albumin, and high levels of ferritin, liver enzymes and triglycerides. Activated macrophages are found in various organs, particularly in bone marrow. Most hypotheses on the mechanism underlying MAS are based on the data obtained in primary haemophagocytic lymphohistiocytosis (HLH), a genetic disease very similar to MAS. Prompt diagnosis is essential because prognosis is highly related to early treatment. The first approach was to use intravenous methylprednisolone pulse therapy; cyclosporin A was proposed in patients resistant to steroids. We describe nine patients affected by haemophagocytosis: seven patients developed MAS and two patients developed HLH. A child with s-JIA developed three episodes of MAS. After the third episode, as there was no improvement with pulses of methylprednisolone and cyclosporine, he was successfully given etanercept. Conclusion: Our data, together with a similar, published observation, suggest that the TNF inhibitor etanercept is potentially useful for obtaining remission in children not responding to steroids and cyclosporin A. (c) 2006 Taylor & Francis

16. CUI TG, HOU FF, NI ZH, CHEN XM, ZHANG FS, ZHU TY, ZHAO XZ, BAO CD, ZHAO MH, WANG GB, QIAN JQ, CAI GY, LI YN, LU FM, MEI CL, ZOU WZet WANG HY: [Treatment of proliferative lupus nephritis with leflunomide and steroid: a prospective multi-center controlled clinical trial], Zhonghua Nei Ke.Za Zhi., Vol. 44(9), 672-676., 2005
    Organism:Renal Division and Institute of Nephrology, First Hospital, Peking University, Beijing 100034, ChinaFAU - Cui, Tai-gen
    Abstract:OBJECTIVE: Leflunomide (LEF) is a selective inhibitor of de novo pyrimidine synthesis, currently used in the treatment of rheumatoid arthritis. To evaluate the efficacy and safety of LEF in the treatment of proliferative lupus nephritis, a prospective multi-center controlled clinical trial was conducted. METHODS: Patients with biopsy-confirmed proliferative lupus nephritis were recruited. Patients of recent onset who had not used any immunosuppressive drug were given either oral LEF (group A) or IV cyclophosphamide (group B); relapsed patients who had received immunosuppressive therapy 3 months before were given LEF (group C). Efficacy and safety were evaluated at 6 months after treatment. RESULTS: Total 51 patients were enrolled, 4 patients withdrew due to adverse events. For those initial treated patients, total response rate were 80% in group A and 75% in group B, complete remission rate were 40% and 25% respectively, not statistically different. Renal parameters (proteinuria, serum albumin and serum creatinine) and systemic lupus erythematosus disease activity index (SLEDAI) improved similarly in both groups. For 14 relapsed patients, total response rate was 60% and complete remission rate was 6.7%. Major adverse events reported in LEF treated patients were infection and alopecia. Herpes zoster was the most often type among infectious events, and one case of severe lung infection was reported. CONCLUSION: LEF combined with steroid was effective in the induction therapy of proliferative lupus nephritis. LEF was generally well-tolerated, its efficacy in maintenance therapy and long-term safety remains to be clarified

17. DANNER S, SORDET C, TERZIC J, DONATO L, VELTEN M, FISCHBACH Met SIBILIA J: Epidemiology of juvenile idiopathic arthritis in Alsace, France, J.Rheumatol., Vol. 33(7), 1377-1381., 2006
    Organism:Department of Pediatry, Laboratory of Epidemiology, and Department of Rheumatology, CHU Hautepierre, Strasbourg, FranceFAU - Danner, Stephanie
    Abstract:OBJECTIVE: To determine the incidence, prevalence, and principal characteristics of the different forms of juvenile idiopathic arthritis (JIA) in the region of Alsace, northeastern France, using the new classification of the International League of Associations for Rheumatology (ILAR). METHODS: In 2002 we performed a retrospective epidemiologic study pertaining to the year 2001. The pediatricians, rheumatologists, ophthalmologists, orthopedic surgeons, and physicians involved in functional reeducation in the Alsace region were interviewed, and all patients were classified according to the new ILAR classification using the criteria revised in Durban in 1997. RESULTS: Among the 361 clinicians contacted, the participation rate was 97.8%. The study identified 67 children followed for JIA in Alsace in 2001, from a total population of 1.8 million inhabitants including 339,095 children under age 16 years. The incidence was calculated to be 3.2 cases/100,000/year and the prevalence 19.8 cases/100,000 children under age 16 years. Among these 67 cases of JIA, the most frequent forms were oligoarthritis (n = 27, 40.3%), polyarthritis without rheumatoid factor (RF; n = 15, 22.4%), and enthesitis related arthritis (n = 12, 17.9%). Other forms, notably systemic arthritis (n = 6, 8.9%) and psoriatic arthritis (n = 3, 4.5%), were more rare and in this study there was no case of polyarthritis with RF. Only 4 patients (6%) were classified in the undifferentiated arthritis group using the new classification. Antinuclear antibodies (ANA; by indirect immunofluorescence, HEp >/= 1/80) were detected in patients with oligoarthritis (81%) and polyarthritis without RF (79%). Uveitis occurred in 41% of children with oligoarthritis and in 14% of those with polyarthritis without RF. CONCLUSION: Our results are comparable to those of other studies carried out in Caucasian populations with regard to incidence and prevalence. This work also highlights the frequent presence of ANA and uveitis in patients with oligoarthritis or polyarthritis without RF

18. EL GAMAL YM, HESHMAT NM, HOSSNY EM, AFIFI HM, EL SHIBINY AM et HASAN ZE: Diagnostic and prognostic value of soluble P-selectin and D-dimer in juvenile rheumatoid arthritis, S21, 03-03-2001

19. GOODMAN SB, OH K, IMRIE S, HWANG Ket SHEGOG M: Revision total hip arthroplasty in juvenile chronic arthritis - 17 revisions in 11 patients followed for 4-12 years, Acta Orthopaedica, Vol. 77, 242-250., 2006
    Organism:Stanford Univ, Sch Med, Dept Orthopaed Surg, Stanford, CA 94305 USA USA
    Abstract:Background Revision total hip arthroplasty (THA) in patients with juvenile chronic arthritis (JCA) is complicated by the young age of the patient, poor bone stock and small physical proportions. We report the complications and outcome of a prospective series of 17 revision THAs in Charnley class C JCA patients.Methods 15 acetabular components and 10 femoral components were revised. 13 cementless cups, 2 reconstruction/roof rings and cemented cups, and 4 cemented and 6 cementless femoral stems were implanted. 2 proximal femoral allografts and 1 strut allograft were used. Age at revision was 32 (21-53) years. Follow-up averaged 7 (4-12) years.Results 2 patients with ce mented femoral stems developed loosening, osteolysis and fracture. Both were successfully revised to long-stem cementless implants with strut/proximal femoral allografts. 1 loose, worn cementless cup with osteolysis was revised. 1 patient with a peri-operative infection and late acetabular fracture had a loose, non-revised cementless cup. 1 case of sciatic nerve palsy occurred after revision using a reconstruction ring. 1 late infection necessitated resection arthroplasty. Harris hip scores improved from 53 (34-85) to 76 (47-96).Interpretation Revision THA in JCA has a substantial complication rate, even in experienced hands. The problem of obtaining long-term stable fixation, osteolysis, and replenishment of lost bone stock are major difficulties

20. GREGORIO A, GAMBINI C, GERLONI V, PARAFIORITI A, SORMANI MP, GREGORIO S, DE MARCO G, ROSSI F, MARTINI Aet GATTORNO M: Lymphoid neogenesis in juvenile idiopathic arthritis correlates with ANA positivity and plasma cells infiltration, Rheumatology (Oxford)., Vol. ., 2006
    Organism:2nd Division of Pediatrics 'G Gaslini' Institute, Genoa, Italy; Department of Pediatrics, University of Genoa, Genoa, Italy
    Abstract:Objective. The aim of the study was to evaluate the pattern of the lymphoid organization in the synovial tissue of patients affected with juvenile idiopathic arthritis (JIA). Methods. A total of 40 JIA patients who underwent synoviectomy or synovial biopsies were enrolled. The mean age at surgery was 15.1 yrs (range 6-30 yrs) and the mean disease duration was 6.7 yrs (range 3 months to 22.2 yrs). Tissue specimens were grouped according to the following criteria: (i) diffuse perivascular infiltrate without lymphoid organization, (ii) T cell-B cell aggregates with or without germinal centre reaction. Results. Synovial tissues from 12 JIA patients did not show any sign of lymphoid organization, whereas 28 patients displayed a variable number of T-B cell aggregates. Typical features consistent with a germinal centre reaction were present in two JIA patients only. Lymphoid organization in JIA patients did not correlate with the duration and severity of the disease or with the degree of synovial inflammation, but did positively correlate with the presence of anti-nuclear antibodies. Moreover, a diffuse lymphocyte infiltration was significantly related to the presence of an acute phase of inflammation and the presence of lymphoid aggregates correlated with the degree of plasma cells infiltration. Conclusions. Lymphoid neogenesis in JIA represents a phase in the immunopathological process that characterize the development of inflammatory synovitis. It is not related to disease activity or severity, but appears to be more frequent in patients with circulating anti-nuclear antibodies

21. GUTIERREZ-SUAREZ R, PISTORIO A, CESPEDES CA, NORAMBUENA X, FLATO B, RUMBA I, HARJACEK M, NIELSEN S, SUSIC G, MIHAYLOVA D, HUEMER C, MELO-GOMES J, ANDERSSON-GARE B, BALOGH Z, DE CUNTO C, VESELY R, PAGAVA K, ROMICKA AM, BURGOS-VARGAS R, MARTINI Aet RUPERTO N: Health-related quality of life of patients with juvenile idiopathic arthritis coming from 3 different geographic areas. The PRINTO multinational quality of life cohort study, Rheumatology (Oxford)., Vol. ., 2006
    Organism:IRCCS G Gaslini, Pediatria II, Reumatologia, PRINTO, Genova, Italy
    Abstract:Objectives. To compare health-related quality of life (HRQL) and to identify clinical determinants for poor HRQL of patients with juvenile idiopathic arthritis (JIA) coming from three geographic areas. Methods. The HRQL was assessed through the Child Health Questionnaire (CHQ). A total of 30 countries were included grouped in three geographic areas: 16 countries in Western Europe; 10 in Eastern Europe; and four in Latin America. Potential determinants of poor HRQL included demographic data, physician's and parent's global assessments, measures of joint inflammation, disability as measured by Childhood Health Assessment Questionnaire (CHAQ) and erythrocyte sedimentation rate. Poor HRQL was defined as a CHQ physical summary score (PhS) or psychosocial summary score (PsS) <2 s.d. from that of healthy children. Results. A total of 3167 patients with JIA, younger than 18 yrs, were included in this study. The most affected health concepts (<2 s.d. from healthy children) that differentiate the three geographic areas include physical functioning, bodily pain/discomfort, global health, general health perception, change in health with respect to the previous year, self-esteem and family cohesion. Determinants for poor HRQL were similar across geographic areas with physical well-being mostly affected by the level of disability while the psychosocial well-being by the intensity of pain. Conclusion. We found that patients with JIA have a significant impairment of their HRQL compared with healthy peers, particularly in the physical domain. Disability and pain are the most important determinants of physical and psychosocial well-being irrespective of the geographic area of origin

22. HAWROT AC, METRY DW, THEOS AJet LEVY ML: Etanercept for psoriasis in the pediatric population: experience in nine patients, Pediatr.Dermatol., Vol. 23(1), 67-71., 2006
    Organism:Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USAFAU - Hawrot, Aimee C
    Abstract:Psoriasis commonly affects children and adolescents, and the need for safe, effective therapy is a special consideration in the pediatric population. In recent years, the use of targeted immunomodulatory biologic agents has been increasingly studied for the treatment of psoriasis. Of these, etanercept, a tumor necrosis factor-alpha antagonist, has been approved for the treatment of psoriasis and psoriatic arthritis in adults, and while it is approved for use in juvenile rheumatoid arthritis, formal studies are needed to demonstrate its safety and efficacy for childhood psoriasis. We present our preliminary experience of treating nine pediatric patients with generalized, recalcitrant psoriasis with etanercept therapy

23. HOMMEL KA, CHANEY JM, WAGNER JLet JARVIS JN: Learned helplessness in children and adolescents with juvenile rheumatic disease, J.Psychosom.Res., Vol. 60(1), 73-81., 2006
    Organism:The Children's Hospital of Philadelphia, PA 19104-4399, United States hommel@emailchopeduFAU - Hommel, Kevin A
    Abstract:OBJECTIVE: To examine a learned helplessness conceptualization of psychological sequela in children and adolescents with juvenile rheumatic diseases (JRD) via an experimental procedure utilizing behavior-outcome contingent and noncontingent feedback. METHODS: Thirty-eight children and adolescents with JRD completed measures of transient affect, self-efficacy for functional ability, and causal attributions prior to and immediately following a computerized learned helplessness induction procedure. RESULTS: Children across contingent and noncontingent feedback conditions experienced decreased positive affect with a slightly more pronounced decline in the noncontingent condition. Noncontingent feedback resulted in poorer internalization of success for problem solving, while contingent feedback resulted in greater internalization of success for problem solving. Additionally, posttreatment control self-efficacy was significantly greater for children in the contingent condition that initially endorsed higher levels of internal task attributions. CONCLUSIONS: Children with JRD who experience behavior-outcome contingency in their environment may develop increased perceptions of control over functional ability. Furthermore, environmental noncontingency may result in poorer internalization of success, whereas contingent reinforcement may enhance cognitive appraisal mechanisms (i.e., causal attributions) associated with favorable disease outcome. Despite a modest decline in positive affect for children in the noncontingent condition, mood dysfunction is not entirely explicable within the context of noncontingent reinforcement

24. HSIEH YS, YANG SF, LUE KHet LU KH: Clinical correlation with the PA/plasmin system in septic arthritis of the knee, Clin.Orthop.Relat Res., Vol. 447, 172-178., 2006
    Organism:Institute of Biochemistry, Chung Shan Medical University Hospital, Taichung, TaiwanFAU - Hsieh, Yih-Shou
    Abstract:Substantially more gelatinases appear in effusions of septic arthritis than in effusions of aseptic arthritis. We hypothesized there is greater plasminogen activator/plasmin activity in effusions of septic arthritis than aseptic arthritis. We examined the antigenic values of urokinase-type plasminogen activator and plasminogen activator inhibitor Type-1, cell counts, and levels of matrix metalloproteinase-2 and metalloproteinase-9 in 135 knee effusions from 80 patients with septic arthritis, rheumatoid arthritis, gouty arthritis, and osteoarthritis. Urokinase-type plasminogen activator and plasminogen activator inhibitor Type-1 antigenic values in effusions of septic arthritis were greater than those in effusions of aseptic arthritis. The increases of urokinase-type plasminogen activator and plasminogen activator inhibitor Type-1 antigenic values in effusions were associated with increased levels of prometalloproteinase-9 and the appearance of activated metalloproteinase-2. Antigenic values of urokinase-type plasminogen activator also correlated with the appearance of activated metalloproteinase-9. High plasminogen activator/plasmin activity, prometalloproteinase-9 levels, and the presence of activated metalloproteinase-2 and metalloproteinase-9 in effusions from replaced knees should increase suspicion of infection regardless of neutrophil counts. Joint aspiration reduces bacteria counts, endotoxins, proinflammatory cytokines, and matrix metalloproteinase. It also decreases the plasminogen activator/plasmin activity in effusions that may play a part in extracellular matrix destruction.Level of Evidence: Diagnostic study, Level III (study of nonconsecutive patients without consistently applied reference "gold" standard). See the Guidelines for Authors for a complete description of levels of evidence

25. IESAKA K, KUBIAK EN, BONG MR, SU ETet DI CESARE PE: Orthopedic surgical management of hip and knee involvement in patients with juvenile rheumatoid arthritis, Am.J.Orthop., Vol. 35(2), 67-73., 2006
    Organism:Musculoskeletal Research Center, NYU-Hospital for Joint Diseases, New York, NY, USAFAU - Iesaka, Kazuho
    Abstract:Juvenile rheumatoid arthritis is the most common arthritic disease of childhood and a leading cause of childhood disability, affecting an estimated 300,000 US children and adolescents aged < or =16 years. Approximately 10% to 30% of patients experience functional deficits resulting from both the articular and systemic manifestations of their disease, including leg length inequality and deformity, that are often more crippling than joint destruction. Surgical intervention to treat bone and soft-tissue deformity, leg length inequality, and joint destruction is indicated when medical therapy has failed. Synovectomy, soft-tissue release, osteotomy, and epiphysiodesis are used to treat deformity and early joint destruction. Arthroplasty remains the primary therapy for joint destruction, although it is fraught with complications specific to this young patient population

26. JUNNILA JLet CARTWRIGHT VW: Chronic musculoskeletal pain in children: part I. Initial evaluation, Am.Fam.Physician., Vol. 74(1), 115-122., 2006
    Organism:Army Medical Department Center and School, San Antonio, Texas, USA JenniferJunnila@usarmymilFAU - Junnila, Jennifer L
    Abstract:Musculoskeletal pain can be difficult for children to characterize. Primary care physicians must determine whether the pain may be caused by a systemic disease. Change in activity, constitutional symptoms such as fevers and fatigue, or abnormal examination findings without obvious etiology should raise suspicion for rheumatic disease. A complete physical examination should be performed to look for extra-articular signs of rheumatic disease, focusing on but not limited to the affected areas. A logical and consistent approach to diagnosis is recommended, with judicious use of laboratory and radiologic testing. Complete blood count and erythrocyte sedimentation rate measurement are useful if rheumatic disease is suspected. Other rheumatologic tests (e.g., antinuclear antibody) have a low pretest probability in the primary care setting and must be interpreted cautiously. Plain radiography can exclude fractures or malignancy; computed tomography and magnetic resonance imaging are more sensitive in detecting joint inflammation. Family physicians should refer children to a subspecialist when the diagnosis is in question or subspecialty treatment is required. Part II of this series discusses rheumatic diseases that present primarily with musculoskeletal pain in children, including juvenile arthritis, the spondyloarthropathies, acute rheumatic fever, Henoch-Schonlein purpura, and systemic lupus erythematosus

27. KRUITHOF E, VAN dB, V, DE RYCKE L, VANDOOREN B, JOOS R, CANETE JD, TAK PP, BOOTS AM, VEYS EMet BAETEN D: Distinct synovial immunopathologic characteristics of juvenile-onset spondylarthritis and other forms of juvenile idiopathic arthritis, Arthritis Rheum., Vol. 54(8), 2594-2604., 2006
    Organism:Ghent University Hospital, Ghent, Belgium
    Abstract:OBJECTIVE: To characterize the synovial immunopathologic features of juvenile-onset spondylarthritis (SpA) in relation to adult SpA and other forms of juvenile idiopathic arthritis (JIA). METHODS: Synovial biopsy samples were obtained from 10 patients with juvenile-onset SpA, 23 with adult SpA, 19 with rheumatoid arthritis (RA), 8 with juvenile polyarthritis, and 12 with juvenile oligoarthritis. Synovial immunopathologic features were studied by extensive histologic and immunohistochemical analyses. RESULTS: Synovitis in juvenile SpA was characterized by marked lining layer hyperplasia, clear hypervascularity, and pronounced inflammatory cell infiltration with lymphocytes and macrophages, independent of disease duration or time of sampling. The immunopathologic features of juvenile SpA resembled those of adult SpA in terms of hypervascularity and absence of RA-specific intracellular citrullinated proteins and HLA-DR4/human cartilage glycoprotein 39(263-275) complexes, but differed markedly by a stronger lining layer hyperplasia and lower numbers of CD163+ macrophages. Accordingly, class prediction analysis failed to classify juvenile SpA synovitis in the SpA group. Comparison of juvenile SpA with other JIA subtypes showed a broad overlap, with the exception of slightly lower vascularity in juvenile polyarthritis and higher inflammatory cell infiltration in juvenile oligoarthritis. Unsupervised clustering analysis identified a subgroup of samples characterized by high plasma cell infiltration, which corresponded with active, longstanding JIA, mostly of the oligoarthritis subtype. CONCLUSION: Despite some similarities with adult SpA, the findings with regard to lining layer hyperplasia and CD163+ macrophage infiltration are indicative of important differences in the synovial immunopathologic features of juvenile-onset SpA. The partial overlap with other JIA subtypes emphasizes the need for further biologic characterization of JIA in order to define pathophysiologic, rather than phenotypic, subgroups

28. KUMAR A: Experience with anti-tumor necrosis factor-alpha therapy in India|, 2006
    Organism:Aims: To review the Indian experience with anti-tumor necrosis factor (TNF)-alpha therapy. Methods: 'PubMed' and 'IndMED' were searched for Indian studies on anti-TNF-alpha therapy. Data were compiled and analysed. Results: Data on infliximab from 176 patients from five different series were collated. One hundred and forty-seven had ankylosing spondylitis (AS), nine had polyarticular juvenile idiopathic arthritis (JIA), 12 had rheumatoid arthritis (RA), six had undifferentiated spondyloarthropathy, one had inflammatory bowel disease-related spondyloarthritis and one had psoriatic arthritis. Th us, 155/176. (88%) had spondyloarthropathy (SpA). No screening for latent tuberculosis was done in any of the studies. One series comprising 108 cases of AS, used 3 mg/kg infliximab infusions (instead of 5 mg/kg) at 8-weekly intervals with omission of the 2-week and 6-week doses. All others with SpA (n = 47) followed the standard protocol: 171/176 patients had a significant improvement. Reactivation tuberculosis developed in 5/47 (10.6%) SpA patients treated with standard doses of infliximab. This amounted to 56 times increased risk compared to baseline (0.187%). None of the 129 patients treated with 3 mg/kg infusions of infliximab developed reactivation tuberculosis (AS -108, RA -12, JIA -9). The lone study on etanercept showed good efficacy in 40 patients with RA. However, seven serious adverse events occurred. Conclusions: Infliximab showed expected efficacy in SpA, RA and JIA. Reactivation tuberculosis developed in 10.6% of the SpA group treated with standard regimen. Patients treated with lower doses of infliximab which included a large subgroup of SpA patients and those with RA or JIA did not develop tuberculosis. (c) 2006 Asia Pacific League of Associations for Rheumatology and Blackwell Publishing Asia Pty Ltd

29. LEN CA, TERRERI MT, PUCCINI RF, WECHSLER R, SILVA EK, OLIVEIRA LM, BARBOSA CM, PEDROSO GCet HILARIO MO: Development of a tool for early referral of children and adolescents with signs and symptoms suggestive of chronic arthropathy to pediatric rheumatology centers, Arthritis Rheum., Vol. 55(3), 373-377., 2006
    Organism:Escola Paulista de Medicina, Universidade Federal de Sao Paulo, Avenida Brigadeiro Faria Lima 1826 cj 205, Sao Paulo, SP CEP 01451-001, Brazil lencl@terracombrFAU - Len, Claudio Arnaldo
    Abstract:OBJECTIVE: To develop an easy, time-efficient tool to identify children and adolescents with signs and symptoms suggestive of chronic arthropathies, and to evaluate its interobserver reproducibility and reliability. METHODS: The instrument used standardized techniques as required for the development of health-related instruments, targeting parents of apparently healthy children and adolescents ages 1-16 years. A multidisciplinary team was involved in the design of the instrument. RESULTS: Each health professional generated 10-15 questions addressing musculoskeletal complaints that they considered to be the most relevant. A total of 60 questions were listed. During the reduction step, each health professional scored questions from 1 to 4 according to the question's relevance. The tool comprised 12 questions and was administered to the parents of 3 groups: patients with juvenile idiopathic arthritis (JIA; n = 48), children with diffuse musculoskeletal pain (n = 39), and a healthy control group (n = 42). The JIA group achieved the highest scores, followed by the diffuse musculoskeletal pain group and the control group. Nine (18.7%) of 48 patients with JIA and 2 (5.1%) of 39 children with musculoskeletal pain had a score of 5. The interobserver reproducibility was confirmed. All 12 questions were included in the final version of the instrument. We determined that children and adolescents with a score > or =5 should be referred for a rheumatologic evaluation (cluster analysis and logistic regression). CONCLUSION: Our questionnaire seems to be a useful tool for the early detection of musculoskeletal problems in children that may need a referral for a rheumatologic evaluation

30. MANUYAKORN W, KAMCHAISATIAN W, BENJAPONPITAK S, WATTANASIRICHAIGOON D et DIREKWATTANACHAI C: Novel mutation of BTK gene in an XLA patient with late manifestation of chronic arthritis, encephalopathy and pseudomonas, sepsis, S292, 03-03-2001

31. NIEDERLOVA J, KODETOVA D, KRYS cet VILIM V: Immunolocalization of 3-nitrotyrosine in human synovium|, 2006
    Organism:3-nitrotyrosine (3NT) is regarded as some kind of a "footprint" of NO generation. Immunohistochemical analysis was performed on 9 specimens of human synovium (3 rheumatoid arthritis, 4 osteoarthritis (OA), 1 juvenile idiopathic arthritis, and 1 inflamed Baker's cyst derived from a joint of a patient with villonodular synovitis). Serial sections were stained with monoclonal antibody 60-E3 to 3NT, and with antibodies to CD31, CD68, and CD14 reacting to endothelial cells, macrophages and monocytes, respectively. The majority of specimens demonstrated a pattern of strong 3NT staining localized to endothelial cells accompanied by weak immunostaining of tissues closely sur rounding the 3NT-positive endothelia (7 of the 9 specimens, including all of the specimens derived from osteoarthritic joints). We observed strong 3NT immunostaining of synovial stroma in only 2 specimens of inflamed synovia, those collected from the Baker's cyst secondary to villonodular synovitis and a knee joint with rheumatoid arthritis. We conclude that in human synovial tissue, tyrosine is primarily nitrated in endothelial cells. 3NT-positive staining of endothelial cells in OA synovia suggests that activation of endothelial cells occurs even in apparently non-inflammatory OA synovial tissue

32. NORRBY U, NORDHOLM L, ANDERSSON-GARE Bet FASTH A: Health-related quality of life in children diagnosed with asthma, diabetes, juvenile chronic arthritis or short stature, Acta Paediatr., Vol. 95(4), 450-456., 2006
    Organism:Department of Paediatrics, Goteborg University, Goteborg, Sweden ullanorrby@pediatguseFAU - Norrby, Ulla
    Abstract:AIM: 1) To assess the reliability and validity of the Swedish version of the Child Health Questionnaire (CHQ), 2) to determine the correlation between children's and parents' responses to the CHQ, and 3) to describe and compare responses to the CHQ of four diagnostic groups. METHODS: A total of 199 Swedish children aged 9-16 with diagnoses of asthma (n = 53), diabetes (n = 48), short stature (n = 51) and juvenile chronic arthritis (JCA, n = 47) and their parents answered the CHQ and relevant validation instruments at a clinic check-up. Coefficient alphas were determined for all dimensions of the instrument, and all but four had acceptable to very good reliability (0.75-0.94). RESULTS: Concerning construct validity, the CHQ correlated significantly with appropriate dimensions of the validation instruments. In general, there were significant correlations between the children's and parents' responses. Comparisons between the diagnostic groups showed several significant differences. The short stature group had the highest quality of life and the JCA group the lowest. There were no sex differences, but children who had not reached puberty scored better on the dimensions of mental health and self-esteem. CONCLUSION: The Swedish version of the CHQ is a reliable and valid instrument. Furthermore, it is recommended to ask children themselves about their health-related quality of life

33. OJMYR-JOELSSON M, NISELL M, FRENCKNER B, RYDELIUS PAet CHRISTENSSON K: High and intermediate imperforate anus: psychosocial consequences among school-aged children, J.Pediatr.Surg., Vol. 41(7), 1272-1278., 2006
    Organism:Division of Pediatric Surgery, Q2:03, Astrid Lindgren Children's Hospital, Karolinska University Hospital, SE-171 76 Stockholm, Sweden mariaojmyr-joelsson@karolinskaseFAU - Ojmyr-Joelsson, Maria
    Abstract:BACKGROUND/PURPOSE: Imperforate anus is an unusual malformation, which, even after surgical intervention, usually entails constipation and fecal incontinence. This study aimed to evaluate ongoing psychosocial effects of this birth defect in school-aged children. METHODS: Twenty-five children born with high and intermediate imperforate anus participated in the study, along with their parents and classroom teachers. One group of healthy children and 1 group of children with juvenile chronic arthritis, along with their parents, served as controls. Children and parents individually answered a questionnaire devised for this study. Parents filled out the Child Behavior Checklist and the children's teacher filled out the Teacher's Report Form. RESULTS: According to test results, children with imperforate anus were happy and optimistic. They liked school better and reported better relationships with schoolmates than the other children. The index group reported statistically significantly more frequent constipation. According to parental responses, the imperforate-anus children suffered from fecal incontinence and odor, as well as constipation (P < .001). Index-group parents reported on the Child Behavior Checklist that their children had more emotional and behavioral problems. On the Teacher's Report Form, teachers reported few problems for the same children. CONCLUSIONS: Patients with imperforate anus did not experience psychosocial impairment despite significant functional problems

34. OZYUREK AR, GURSES D, ULGER Z, LEVENT E, BAKILER ARet BERDELI A: Allelic frequency of the MCP-1 promoter -2518 polymorphism in the Turkish population and in Turkish patients with juvenile rheumatoid arthritis, Clin.Rheumatol., Vol. ., 2006
    Organism:Department of Pediatrics, Ege University Faculty of Medicine, Izmir, Turkey
    Abstract:Although genetic and environmental factors contribute to the pathogenesis of juvenile rheumathoid arthritis (JRA), the etiology and pathogenesis remain controversial. The objective of this study was to investigate genotypic and allelic frequencies of monocyte chemoattractant protein-1 (MCP-1) gene -2518 (G/A) polymorphism in the healthy Turkish population and patients with JRA. Genomic DNA was collected from 66 JRA patients and 150 healthy individuals. To evaluate the association of the -2518 (G/A) MCP-1 gene polymorphism with the outcome of JRA, we analyzed the types of JRA and the score on the childhood health assessment questionnaire (C-HAQ score). In the healthy Turkish population, the frequencies of A and G alleles were 71 and 29%, respectively. No significant difference was observed between the JRA patients and healthy subjects in the distribution allelic and genotypic frequencies of the -2518 (G/A) MCP-1 gene polymorphism (p>0.05). However, the AG genotype was found to be higher and the AA genotype was found to be lower in the patients with systemic type JRA compared to those with the other types of JRA (p=0.019). When the JRA patients were evaluated according to the C-HAQ score, we found that the -2518 (G/A) MCP-1 gene polymorphism did not relate the prognosis (p>0.05). AG genotype was found to be higher in the systemic type of JRA. The results indicate that MCP-1 gene polymorphism might slightly associate with patients with systemic JRA. Further studies are needed to elucidate the role of this polymorphism in the pathogenesis of JRA in various populations because this polymorphism has a functional significance and an ethnic difference

35. PATTEN SB, WILLIAMS JVet WANG J: Mental disorders in a population sample with musculoskeletal disorders, BMC.Musculoskelet.Disord., Vol. 7, 37, 2006
    Organism:Department of Community Health Sciences, University of Calgary, 3330 Hospital Drive NW, Calgary, AB, T2N 4N1, Canada patten@ucalgarycaFAU - Patten, Scott B
    Abstract:BACKGROUND: Studies using clinical and volunteer samples have reported an elevated prevalence of mood disorders in association with rheumatoid arthritis and osteoarthritis. Clinical studies using anxiety rating scales have reported inconsistent results, but studies using diagnostic instruments have reported that anxiety disorders may be even more strongly associated with arthritis than is depression. One study reported an association between lifetime substance use disorders and arthritis. METHODS: Data from iteration 1.2 of the Canadian Community Health Survey (CCHS) were used. This was a large-scale national Canadian health survey which administered the World Mental Health Composite International Diagnostic Interview to a sample of 36,984 subjects randomly selected from the national population. In the CCHS 1.2, subjects were asked whether they had been diagnosed by a health professional with arthritis or rheumatism. RESULTS: Subjects reporting arthritis or rheumatism had an elevated prevalence of mood, anxiety and substance use disorders. The strength of association resembled that seen in an omnibus category reporting any chronic condition, but was weaker than that seen with back pain or fibromyalgia. The effect of arthritis or rheumatism interacted with age, such that the odds ratios became smaller with increasing age. Mood and anxiety disorders, along with arthritis or rheumatism made an independent contribution to disability. CONCLUSION: Arthritis is associated with psychiatric morbidity in the general population, and this morbidity is seen across a variety of mental disorders. The strength of association is consistent with that seen in persons with other self-reported medical conditions

36. PUOLAKKA K, KAUTIAINEN H, PEKURINEN M, MOTTONEN T, HANNONEN P, KORPELA M, HAKALA M, ARKELA-KAUTIAINEN M, LUUKKAINEN Ret LEIRISALO-REPO M: Monetary value of lost productivity over a five year follow up in early rheumatoid arthritis estimated on the basis of official register data on patients' sickness absence and gross income: experience from the FIN-RACo trial, Ann.Rheum.Dis., Vol. 65(7), 899-904., 2006
    Organism:Department of Medicine, Lappeenranta Central Hospital, Valto Kakelan katu 1, Lappeenranta FIN-53130, and Department of Medicine, Turku University Central Hospital, Finland karipuolakka@fimnetfiFAU - Puolakka, K
    Abstract:OBJECTIVE: To explore the monetary value of rheumatoid arthritis related loss of productivity in patients with early active disease. METHODS: In a prospective cohort substudy of the FIN-RACo Trial, 162 patients with recent onset rheumatoid arthritis, aged 18 to 65 years and available to the workforce, were followed up for five years. Loss of work productivity in euros 2002 was estimated by data on absence for sickness and on income (human capital approach) from official databases. Treatment responses were evaluated by area under the curve (AUC) of the ACR-N measure and by increase in number of erosions in radiographs of hands and feet. The health assessment questionnaire (HAQ) at six months was linked to the International Classification of Functioning, Disability and Health (ICF). RESULTS: In all, 120 (75%) patients, women more often (82%) than men (61%) (p=0.002), lost work days. The mean lost productivity per patient-year was euro7217 (95% confidence interval (CI), 5561 to 9148): for women, euro6477 (4858 to 8536) and for men, euro8443 (5389 to 12,898). There was an inverse correlation with improvement: euro1101 (323 to 2156) and euro14 952 (10,662 to 19,852) for the highest and lowest quartiles of AUC of ARC-N, respectively. Lost productivity was associated with increase in the number of erosions and with disability in "changing and maintaining body position" subcategory of the ICF. CONCLUSIONS: Despite remission targeted treatment with disease modifying antirheumatic drugs, early rheumatoid arthritis results in substantial loss of productivity. A good improvement in the disease reduces the loss markedly

37. QUARTA L, CORRADO A, MELILLO Net CANTATORE FP: Juvenile idiopathic arthritis: an update on clinical and therapeutic approaches, Ann.Ital.Med.Int., Vol. 20(4), 211-217., 2005
    Organism:Clinica Reumatologica M Carrozzo, Universiti degli Studi di FoggiaFAU - Quarta, Laura
    Abstract:Juvenile idiopathic arthritis represents a heterogeneous group of autoimmune diseases. It arises before 16 years of age and lasts more than 6 months. We can distinguish many arthritis sub-types. A serious problem in juvenile idiopathic arthritis is skeletal growth retardation, osteopenia and greater risk of developing fractures. Juvenile idiopathic arthritis diagnosis is an exclusion diagnosis. Many conditions can simulate it. First-choice drugs in juvenile idiopathic arthritis treatment are nonsteroidal anti-inflammatory drugs, analgesic and antipyretic drugs. The second-choice drugs are "slow-acting" antirheumatic drugs, like methotrexate. The use of glucocorticoids is strongly influenced by their side effects, in particular the inhibition of statural growth and the premature appearance of osteoporosis. Recent findings on the central role of tumor necrosis factor-alpha, in particular damage pathogenesis in the course of juvenile idiopathic arthritis, have permitted the development of new therapeutic strategies (infliximab, etanercept), aimed at blocking this cytokine

38. ROBERT V, ESZTO P, PERROTEZ JL, GALZIN Met POUSSEL JF: [Treatment by plasmapheresis of a thrombotic thrombocytopenic purpura associated to a Still's disease: a case report], Ann.Fr.Anesth.Reanim., Vol. 25(5), 532-534., 2006
    Organism:Service de Reanimation Polyvalente, Hopital ND-de-Bonsecours, CHR de Metz-Thionville, 1, place Philippe-de-Vigneulles, BP 81065, 57038 Metz cedex 01, FranceFAU - Robert, V
    Abstract:We report the seventh published case of thrombotic thrombocytopenic purpura (TTP) associated to a Still's disease. We confirmed the secondary character of TTP using the measurement of ADAMTS 13 protease. Because of clinical and biological improvement, daily plasmapheresis was stopped after eight days of ICU treatment. Unfortunately, early (24 hours) relapse occurred resulting in daily plasmapheresis resumption for 11 more days. Main therapeutic goals and aggressive treatment duration of PTT associated with a Still's disease remain to be determined

39. ROTH J, SCHEER I, KRAFT S, KEITZER Ret RIEBEL T: Uncommon synovial cysts in children, Eur.J.Pediatr., Vol. 165(3), 178-181., 2006
    Organism:Pediatric Rheumatology, Charite-Universitaetsmedizin, Berlin, Germany johannesroth@charitedeFAU - Roth, Johannes
    Abstract:Popliteal synovial cysts (Baker's cysts) are a common occurrence in children and adults. Synovial cysts in other locations and/or with atypical extension are less common and may be confounded with tumors or other medical conditions. In this article we describe the underlying disease, clinical presentation and clinical course in six children with a sudden onset of paraarticular soft tissue masses or non-specific chronic pain. Ultrasound was the initial imaging method used in all cases; this was supplemented by MRI in three patients. Four children were diagnosed to be suffering from juvenile idiopathic arthritis (JIA), one child from Lyme Arthritis, whereas in one child no underlying disease was identified. Well-demarcated hypoechogenic lesions without signs of perfusion extending from the shoulder (two patients), elbow (one patient), hip (one patient), knee (1) or ankle (one patient) far into the adjacent musculature were detected on ultrasonography. A direct connection to the joint was demonstrated in all cases. All synovial cysts in the five arthritic patients resolved rapidly with medical treatment for arthritis, whereas the cyst persisted in the non-JIA patient. Conclusion: Uncommon synovial cysts occur in particular as a complication of arthritis. Ultrasonography is the initial and follow-up imaging method of choice, which can be supplemented by MRI in unusual cases. Apart from treatment for arthritis, no specific therapeutic interventions were required in the present cases

40. SHI J, KOVACS SJ, WANG Y, LUDDEN TMet BHARGAVA VO: Population pharmacokinetics of the active metabolite of leflunomide in pediatric subjects with polyarticular course juvenile rheumatoid arthritis, J.Pharmacokinet.Pharmacodyn., Vol. 32(3-4), 419-439., 2005
    Organism:Global Biopharmaceutics, Drug Metabolism and Pharmacokinetics, Aventis Pharmaceuticals, Bridgewater, NJ, USA junshi@FRXcomFAU - Shi, Jun
    Abstract:Leflunomide is a pyrimidine synthesis inhibitor used in the treatment of rheumatoid arthritis. Data from two clinical studies were used to establish a population pharmacokinetic (PPK) model for the active metabolite (M1) of leflunomide in patients with juvenile rheumatoid arthritis (JRA) and determine appropriate pediatric doses. Seventy-three subjects 3-17 years of age provided 674 M1 concentrations. The PPK model was derived from nonlinear mixed-effects modeling and qualified by cross-study evaluation and predictive check. A one-compartment model with first-order input described M1 PPK well. Body weight (WT) correlated weakly with oral clearance (CL/F = 0.020.[WT/40](0.430)) and strongly with volume of distribution (V/F = 5.8.[WT/40](0.769)). Steady-state concentrations (C(ss)) of M1 in JRA were compared for a variety of leflunomide dose regimens using Monte-Carlo simulation. To achieve comparable C(ss) values in pediatric patients with JRA to that in adult patients, doses of leflunomide should be adjusted modestly: 10 mg/d for 10-20 kg, 15 mg/d for 20-40 kg, and 20 mg/d for > 40 kg

41. SIMARD N, BOIRE G, BRUM-FERNANDES Aet ST PIERRE Y: Heterogeneity in the contribution of MMPs in the overall net proteolytic activity in synovial fluids of patients with different forms of arthritis, Arthritis Res.Ther., Vol. %19;8(4), R125, 2006
    Organism:ABSTRACT: Despite decades of research, only a very limited number of MMP inhibitors have been successful clinically in arthritis. One of the central problems associated with this failure is our inability to monitor the local activity of proteases in joints since integrity of the extracellular matrix results from an equilibrium between non-convalent,1:1 stoichiometric binding of protease inhibitors to the catalytic site of the activated forms of the enzymes. In the present work, we have measured by flow cytometry the net proteolytic activity (NPA) in synovial fluids collected from 95 patients with OA or other forms of inflammatory arthritis, including rheumatoid arthritis, spondylarthropathies, and chronic juvenile arthritis. We found that synovial fluids of patients with IA had significantly higher levels of proteolytic activity than that of OA patients. Moreover, the overall activity correlated positively with the number of infiltrated leukocytes and the serum level of CRP. No such correlations were found in OA patients. Members of the MMP family contributed significantly to the proteolytic activity found in arthritic synovium. Small molecular weight MMP inhibitors were indeed effective for inhibiting MMP activity in synovial fluids, but their effectiveness varied greatly among patients. Interestingly, the contribution of MMPs decreased in patients with very high proteolytic activity, at least due to molar excess of TIMP-1 and increased contribution of other proteolytic enzymes. These results emphasize the diversity of the MMPs involved in arthritis and, from a clinical point of view, this approach represents an interesting alternative for testing the potential of new protease inhibitors for the treatment of arthritis

42. SOUZA L, MACHADO SH, BREDEMEIER M, BRENOL JCet XAVIER RM: Effect of inflammatory activity and glucocorticoid [corrected] use on nutritional variables in patients with juvenile idiopathic arthritis, J.Rheumatol., Vol. 33(3), 601-608., 2006
    Organism:Division of Rheumatology, Hospital de Clinicas de Porto Alegre, Faculdade de Medicina, Universidade Federal do Rio Grande do Sul, RS, BrazilFAU - Souza, Leticia
    Abstract:OBJECTIVE: To assess nutritional status in patients with juvenile idiopathic arthritis (JIA) and the influence of inflammatory activity and glucocorticoid use. METHODS: One hundred sixteen patients were evaluated. Disease subtype and disease activity were defined by the attending physician, and the cumulative glucocorticoid dose was recorded from chart review. Percentiles of body mass index (BMI) and triceps skinfold (TSF) and the Z score for height were determined: low weight and low adiposity were diagnosed when BMI and TSF were below the 5th percentile. Short stature was defined by a Z score of height for age < -2. Serum concentration of insulin-like growth factor-I (IGF-I) was measured by radioimmunoassay. RESULTS: The prevalences of low weight, low adiposity, and short stature were 16.4%, 20.7%, and 10.4%, respectively. Low IGF-I serum level was found in 14 patients (12.1%). The factors negatively associated with the Z score of height in multivariable regression analysis were disease duration (partial correlation coefficient -0.370, 95% confidence interval: -0.527 to -0.188; p < 0.001), erythrocyte sedimentation rate (ESR) (-0.357, -0.516 to -0.174; p < 0.001), and polyarticular or systemic disease subtype (-0.290, -0.459 to -0.100; p = 0.003), while there was no significant correlation with the cumulative dose of glucocorticoids (0.086, -0.111 to 0.277; p = 0.391). None of these variables was significantly correlated with the percentiles of BMI and TSF, albeit confidence intervals for these correlation coefficients were relatively large. Patients with a systemic or polyarticular disease subtype tended to present lower percentiles of BMI (p = 0.051). CONCLUSION: Nutritional status is frequently compromised in patients with JIA. Duration and disease subtype and the ESR are factors independently associated with short stature. The cumulative dose of glucocorticoids was not independently associated with short stature or with other nutritional variables, although a relevant negative effect of glucocorticoid dose on BMI and TSF cannot be entirely excluded

43. SOUZA L, MACHADO SH, BREDEMEIER M, BRENOL JOAO CTet XAVIER RM: Effect of inflammatory activity and glucorticoid use on nutritional variables in patients with juvenile idiopathic arthritis, Rinsho Ganka, Vol. 33, 601-608., 2006
    Organism:Univ Fed Rio Grande Sul, Serv Reumatol, Hosp Clin Porto Alegre, Fac Med,Div Rheumatol, Rua Ramiro Barcelos 2350,Sala 645, BR-90035003 Porto Alegre, RS, Brazil Brazil
    Abstract:Objective. To assess nutritional status in patients with juvenile idiopathic arthritis (JIA) and the influence of inflammatory activity and glucocorticoid use.Methods. One hundred sixteen patients were evaluated. Disease subtype and disease activity were defined by the attending physician, and the cumulative glucocorticoid dose was recorded from chart review. Percentiles of body mass index (BMI) and triceps skinfold (TSF) and the Z score for height were determined: low weight and low adiposity were diagnosed when BMI and TSF were below the 5th percentile. Short stature was defined by a Z score of height for age < -2. Serum concentration of insulin-like growth factor-I (IGF-I) was measured by radioimmunoassay.Results. The prevalences of low weight, low adiposity, and short stature were 16.4%, 20.7%, and 10.4%. respectively. Low IGF-I serum level was found in 14 patients (12.1 %). The factors negatively associated with the Z score of height in multivariable regression analysis were disease duration (partial correlation coefficient -0.370, 95% confidence interval: -0.527 to -0.188; p < 0.001), erythrocyte sedimentation rate (ESR) (-0.357, -0.516 to -0.174; p < 0.001), and polyarticular or systemic disease subtype (-0.290, -0.459 to -0.100; p = 0.003), while there was no significant correlation with the cumulative dose of glucocorticoids (0.086, -0.111 to 0.277; p = 0.391). None of these variables was sign ificantly correlated with the percentiles of BMI and TSF, albeit confidence intervals for these correlation coefficients were relatively large. Patients with a systemic or polyarticular disease subtype tended to present lower percentiles of BMI (p = 0.051).Conclusion. Nutritional status is frequently compromised in patients with JIA. Duration and disease subtype and the ESR are factors independently associated with short stature. The cumulative d ose of glucocorticoids was not independently associated with short stature or with other nutritional variables, although a relevant negative effect of glucocorticoid dose on BMI and TSF cannot be entirely excluded

44. STANG PE, BRANDENBURG NA, LANE MC, MERIKANGAS KR, VON KORFF MRet KESSLER RC: Mental and physical comorbid conditions and days in role among persons with arthritis, Psychosom.Med., Vol. 68(1), 152-158., 2006
    Organism:Department of Health, West Chester University and Galt Associates West Chester, PA, USA pstang@galt-assoccomFAU - Stang, Paul E
    Abstract:OBJECTIVE: To estimate the prevalence of comorbidity among people with arthritis in the US adult population and to determine the role of comorbidity in accounting for the association of arthritis with days out of role (a measure of inability to work or carry out normal activities). METHODS: Data come from the National Comorbidity Survey Replication (NCS-R), a nationally representative household survey of 9,282 respondents ages 18 and older carried out in 2001 to 2003. Arthritis was assessed by self-report in a chronic-conditions checklist, along with a wide range of other physical conditions. Mental and substance use disorders were ascertained with the World Health Organization Composite International Diagnostic Interview (CIDI). Number of days out of role was assessed for the 30 days before the interview. RESULTS: Arthritis was reported by 27.3% of respondents, 80.9% of whom also reported at least one other physical or mental disorder, including 45.6% with another chronic pain condition, 62.3% with another chronic physical condition, and 24.3% with a 12-month mental disorder. Arthritis was significantly associated with days out of role, but comorbidity explained more than half of this association. No significant interactions were found between arthritis and the other conditions in predicting days out of role. CONCLUSION: Comorbidity is the rule rather than the exception among people with arthritis. Comorbidity accounts for most of the days out of role associated with arthritis. The societal burden of arthritis needs to be understood and managed within the context of these comorbid conditions

45. WAHEED A, HAMEED K, KHAN AM, SYED JAet MIRZA AI: The burden of anxiety and depression among patients with chronic rheumatologic disorders at a tertiary care hospital clinic in Karachi, Pakistan, J.Pak.Med.Assoc., Vol. 56(5), 243-247., 2006
    Organism:Medical College, Aga Khan University, Karachi, PakistanFAU - Waheed, Abdul
    Abstract:OBJECTIVES: To study the burden of anxiety and depression as a comorbid among patients of chronic rheumatological disorders and to investigate possible determinants of depression and anxiety. METHODS: It was a cross-sectional study conducted at the rheumatology clinic of The Aga Khan University Hospital (AKUH) Karachi, Pakistan. With convenient sampling, 111 patients who fulfilled inclusion/exclusion criteria were screened for anxiety and depression with help of Aga Khan University Anxiety and Depression Scale (AKUADS). The data was entered and analyzed by Statistical Package for Social Sciences (Version 10.0). RESULTS: The population consisted mainly of middle aged (mean age 41) females (80.2%). The most common diagnosis was rheumatoid arthritis 57% followed by systemic lupus erythmatosis 17% and systemic sclerosis 9%. The permanent joint deformity was present in 33.3% patients and 36.9% patients were suffering from active disease with pain and inflammation. The frequency of anxiety and depression was 65.8%. Educational qualification, permanent joint deformity, active inflammation and time elapsed since diagnosis had significant association with anxiety and depression. Marital Status, gender, economic activity and monthly family income had no effect on the frequency of anxiety and depression. CONCLUSION: Almost two third of patients with chronic rheumatological disorders, also suffered from a concomitant mood disorder. Systematic evaluation of all patients for mood disorders and psychological distress in rheumatology clinics is highly recommended

46. WANG SJ, GUO X, ZUO H, ZHANG YG, XU P, PING ZG, ZHANG Zet GENG D: Chondrocyte apoptosis and expression of Bcl-2, Bax, Fas, and iNOS in articular cartilage in patients with Kashin-Beck disease, J.Rheumatol., Vol. 33(3), 615-619., 2006
    Organism:Key Laboratory of Environment and Gene Related Diseases, Xi-an Jiaotong University, Ministry of Education, Shaanxi Province, ChinaFAU - Wang, Shi Jie
    Abstract:OBJECTIVE: Kashin-Beck disease (KBD) is a chronic, endemic osteochondropathy principally occurring in children. We investigated apoptotic chondrocyte death and the expression of Bcl-2, Bax, Fas, and inducible nitric oxide synthase (iNOS) in articular cartilage from patients with KBD in order to determine the pathogenesis of chondronecrosis in KBD. METHODS: Samples of articular cartilage were divided into 2 groups: control children (15 samples from 15 cases), and children with KBD (15 samples from 15 cases). KBD patients were diagnosed according to "Pathological Criteria to Diagnose KBD in China." Chondrocyte apoptosis was detected by TUNEL staining, and Bcl-2, Bax, Fas, and iNOS-positive articular chondrocytes were stained by immunohistochemistry. Articular cartilage was classified in 3 zones, and positive findings were counted by light microscopy for cytoplasmic staining by polyclonal antibodies of Bcl-2, Bax, Fas, and iNOS and apoptotic chondrocytes by TUNEL. RESULTS: The percentage of positive apoptotic chondrocytes stained by TUNEL in the middle zone of articular cartilage from the KBD patient group (33.60% +/- 2.71%) was higher than that of controls (1.33% +/- 0.41%; p < 0.01). The percentages of chondrocytes staining for Bcl-2, Bax, Fas, and iNOS in KBD patients were significantly higher than in controls (p < 0.01); the remarkable difference in Bcl-2, Bax, Fas, and iNOS expression among the upper, middle, and deep cartilage zones was also seen in KBD articular cartilage (p < 0.01); and staining for Bcl-2, Bax, Fas, and iNOS in KBD patients was prominent in the upper zone (41.93% +/- 12.26%, 45.60% +/- 15.78%, 53.60% +/- 16.49%, 45.47% +/- 14.02%, respectively) and the middle zone (14.93% +/- 3.50%, 13.87% +/- 4.32%, 23.27% +/- 4.83%, 21.67% +/- 6.82%) of articular cartilage. CONCLUSION: The apoptotic chondrocytes and Bcl-2, Bax, Fas, and iNOS-positive chondrocytes were significantly more numerous in patients with KBD than in controls

47. WEI JCC, LAN J-L, CHEN D-Y, CHOU C-T, CHOU M-Cet TSAY GJ: Experience of antitumor necrosis factor-alpha therapies in Taiwan|, 2006
    Organism:Background: Etanercept (Enbrel), was the first approved biologics in Taiwan for rheumatoid arthritis (RA), juvenile RA (JRA), psoriatic arthritis and ankylosing spondylitis. Approximately 500 patients with RA were under etanercept therapy by April 2006. Aim: We aimed to review the current status of biological agents use in the treatment of rheumatic diseases in Taiwan. Methods: MEDLINE database (January 1966 to February 2006) was searched by MeSH terms, limited to Taiwan by author affiliations. All known principal clinical investigators and pharmaceutical companies in Taiwan were contacted for unpublished information about this issue. Results: Six articles including two clinical trials, two case series and two preclinical studies were found in MEDLINE since January 1966. A 12-week double-blind placebo-controlled study of 58 patients with active adult RA in Taiwan revealed better efficacy than results in Western countries. For JRA, two studies reported good results in polyarticular type JRA but fair response in systemic type. Adalimumab (Humira, Abbott) and Alefacept (Ameviev, Biogen) had completed their local trials in RA and psoriasis but they are not yet available in Taiwan. Widespread usage of biologics has been limited due to restricted insurance coverage policy only to the refractory patients with RA. Some herbs demonstrating TNF-alpha inhibition properties are under exploratory clinical trials. Conclusions: Etanercept showed better efficacy in patients with adult RA in Taiwan than Western countries. Some herbs demonstrated TNF-alpha inhibition and are under laboratory and clinical investigations. (c) 2006 Asia Pacific League of Associations for Rheumatology and Blackwell Publishing Asia Pty Ltd

48. WIERINGA JW, RAMAKER Cet WOLF BHM: Acute rheumatic fever in children, a diagnostic problem|, 2006
    Organism:Three girls of Moroccan descent, aged 9, 10 and 7 years, presented with fever, joint pain and other symptoms. After Streptococcus infection and carditis were confirmed and the Jones criteria for acute rheumatic fever were met, the patients were treated with penicillin and acetylsalicylic acid. All 3 patients recovered. However, the second girl presented 2 months later with cardiac decompensation caused by valve disorders, after which aortic and mitral valvuloplasty was performed. The third girl developed joint pain again after 3 weeks and was diagnosed with juvenile idiopathic arthritis; treatment was adjusted accordingly. The prevalence of rheumatic heart diseases i s 10-20 times higher in developing countries than in industrialised nations. The diagnosis 'acute rheumatic fever' should be considered in children of school age with unexplained fever, also when the Jones criteria have not yet been met. This may apply to migrant children in particular

49. YAO TC, KUO ML, SEE LC, OU LS, LEE WI, CHAN CKet HUANG JL: RANTES and monocyte chemoattractant protein 1 as sensitive markers of disease activity in patients with juvenile rheumatoid arthritis: A six-year longitudinal study, Arthritis Rheum., Vol. 54(8), 2585-2593., 2006
    Organism:Chang Gung Children's Hospital and Chang Gung University, Taoyuan, Taiwan
    Abstract:OBJECTIVE: To longitudinally investigate serum and synovial fluid (SF) levels of RANTES and monocyte chemoattractant protein 1 (MCP-1) as well as in vitro migration of mononuclear cells toward SF in patients with juvenile rheumatoid arthritis (JRA). METHODS: Serum and SF levels of RANTES and MCP-1 were determined by enzyme-linked immunosorbent assay. Chemotaxis was performed using the modified Boyden chamber method. RESULTS: Serum RANTES levels were significantly increased in all onset types of JRA, with the highest levels present in systemic-onset JRA. Serum MCP-1 levels were significantly elevated in patients with systemic-onset JRA and were associated with current systemic features. Although serum levels of RANTES and MCP-1 decreased significantly after treatment, RANTES and MCP-1 levels during disease remission were still significantly higher in JRA patients than in controls. A relationship was found between serum RANTES levels during remission and the duration of clinical remission, with low levels being associated with prolonged clinical remission and high levels with shorter clinical remission. Serum RANTES levels correlated with C-reactive protein concentrations, hemoglobin values, white blood cell (WBC) counts, and platelet counts, whereas serum MCP-1 levels correlated with WBC counts and serum ferritin levels. Levels of RANTES and MCP-1 in SF were elevated as compared with levels in serum. SF chemotactic activity for mononuclear leukocytes was significantly inhibited by either anti-RANTES or anti-MCP-1 antibody. CONCLUSION: RANTES is a key molecule in the pathogenesis of all onset groups of JRA, whereas MCP-1 is particularly important in systemic-onset JRA. Serum levels of these CC chemokines represent more highly sensitive markers of disease activity than conventional markers of inflammation

50. YOON C-Het KIM H-Y: Extended indications for anti-tumor necrosis factor-alpha therapy|, 2006
    Organism:Tumour necrosis factor-alpha is a pleiotropic cytokine which has a broad range of actions in inflammation, infection and immunity. TNF-alpha is supposed to play a crucial role in the pathogenesis of various autoimmune diseases. TNF-alpha blocking agents have been demonstrated to be highly effective in the treatment of rheumatoid arthritis, psoriatic arthritis, ankylosing spondylitis, and juvenile rheumatoid arthritis. TNF-alpha inhibitors also have been tried with other rheumatic diseases and have emerged as promising treatments. We here review the current evidences of effectiveness of the ant i-TNF-alpha therapy in various autoimmune diseases. (c) 2006 Asia Pacific League of Associations for Rheumatology and Blackwell Publishing Asia Pty Ltd

51. YUN AG, FIGGIE M, DORR LDet SCOTT RD: Hip disease in juvenile rheumatoid arthritis, Orthopedics., Vol. 29(3), 233-239., 2006
    Organism:The Arthritis Institute, Centinela Hospital, 501 E Hardy St, Inglewood, CA 90301, USAFAU - Yun, Andrew G
    Abstract:

52. ZERJAVIC NL, JURESA DS, BABIC-NAGLIC D, CURKOVIC B, POTOCKI K, ZUNEC Ret IVANISEVIC G: [HLA-DRB1 gene distribution in Croatian arthritis patients], Reumatizam., Vol. 52(1), 12-16., 2005
    Organism:Klinika za reumatske bolesti i rehabilitaciju Medicinskog fakulteta Sveucilista u Zagrebu, KBC ZagrebFAU - Zerjavic, Nadica Laktasic
    Abstract:Genetic association between particular HLA-DRB1 genes and severity of rheumatoid arthritis (RA) has been documented in various clinical investigations. Susceptible alleles are *0401, *0404, *0405, *0408, *0101, *1001, *1402. According to the Shared epitope hypothesis presence of these alleles were considered as poor prognostic sign. The aim was to investigate HLA-DRB1 distribution in Croatian arthritis patients. Group of 90 patients with non-specific joint arthritis, non-erosive RA and erosive RA were typed for DRB1 alleles by PCR-SSP method. Susceptible alleles were identified in 58 (64.44 %) patients. The most frequent genes were DRB1 *0101 (43.33 %), *0401 (17.77 %), *0404 (10 %). 9 out of 58 DRB1* positive patients had 2 susceptible alleles, and the rest (49 patients) had only one susceptible allele. The patients with non-specific joint arthritis and non-erosive RA will bee closely followed for more destructive disease course in DRB1* positive patients

53. ZHAO HJ, CHEN TX, HAO YQ, ZHOU YFet YING DM: [Overview of clinical occurrence of primary immunodeficiency disorders in children.], Zhonghua Er.Ke.Za Zhi., Vol. 44(6), 403-406., 2006
    Organism:Department of Immunology/Oncology, Shanghai Institute for Pediatric Research, Xinhua Hospital, Shanghai Jiaotong University Medical School, Shanghai 200092, ChinaFAU - Zhao, Hui-Jun
    Abstract:OBJECTIVE: More than one hundred primary immunodeficiency disorders have been discovered so far. But the incidence of these disorders in our country is still not clear, so we analyzed the clinical data of 93 children with primary immunodeficiency disorders seen in our hospital in recent 30 years to understand the occurrence of primary immunodeficiency disorders in children, to promote the clinicians to become familiar with these disorders, to improve the nationwide registry system and to establish the basis for the treatment and prevention in future. METHODS: To analyze the constituent ratio of the 93 children with primary immunodeficiency disorders seen in our hospital from 1974 to 2003, diagnostic and classification criteria were set by taking the proposal by International Union of Immunological Societies (IUIS) PID classification committee in 2003 into account. All the data were analyzed retrospectively. RESULTS: In the 93 children with primary immunodeficiency disorders, antibody deficiencies were the most frequent (39.8%) finding, followed by combined immunodeficiency, combined T- and B-cell disorders (22.6%), and T lymphocytic deficiencies alone (14.0%). Immunodeficiency with other major defects accounted for 12.9%, phagocytic disorders 9.7%, and complement deficiencies 1.1%. Thus, there seemed to be a tendency that the incidence increased with time. The incidence of these disorders has increased significantly as shown by 50 diagnosed cases in children with these disorders since 1996. Sixteen children died, with the highest mortality occurred with combined immunodeficiency. Seven children developed bronchiectasis. Two children suffered from persistent diarrhea while one of the two was complicated with persistent intestinal fistula. One child developed juvenile rheumatoid arthritis, another one with granulocytopenia and iridocyclitis, and the other with allergic purpura. The boys: girls ratio for all disorders was 3:1. The age of onset ranged from 10 days to 37 years of age. CONCLUSIONS: There are vast variety of primary immunodeficiency disorders in our area and antibody deficiency is the most common abnormality. Combined immunodeficiency has early onset age and high mortality rate. With the great improvement of the diagnostic techniques, these disorders have become a group of important disorders and all the clinicians should pay great attention to these disorders