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OBJECTIVE In this article, a statistical-based iterative ring removal (IRR) algorithm that effectively removes ring artifacts generated by defective detector cells is proposed. METHODS The physical state of computed tomography (CT) detector elements can change dynamically owing to their temperature dependence and the varying irradiation caused by focal spot movements. This variation in the properties of cells may cause false pixel values in sinograms, resulting in rings or segments of rings in reconstructed images. In this article, the proposed algorithm is studied on clinical CT. Two patients were scanned using a clinical CT scanner (AnyScan SPECT/CT, Mediso). Artificial rings and band rings were generated on the real sinogram data to examine the algorithm in different cases. The method was performed also on real ring artifacts. RESULTS The IRR can correct both single and band-like ring artifacts with one or more defective pixels. The proposed algorithm can detect the period when pixels contain false signals and only those periods are corrected. The IRR reduces ring artifacts, even in cases where low-contrast rings occur in the reconstructed image. CONCLUSIONS This statistical correction method efficiently detects and corrects false pixel values in the projection data without causing new artifacts in the reconstructed image. The algorithm is less sensitive to its parameters.

Let [Formula: see text] be the group algebra of the modular group [Formula: see text] over a finite field [Formula: see text] of characteristic two. We calculate the order of the ∗-unitary subgroup of the group algebra [Formula: see text] and describe the structure of the ∗-unitary subgroup in the case when [Formula: see text].

Objective. To report the efficacy and safety of TNF-a inhibitors (etanercept and adalimumab) in a cohort of patients with JIA treated in a single paediatric rheumatological centre. Methods. Patients with JIA under the age of 18 years, treated with TNF-a blockers at the Paediatric Rheumatologic Centre of the National Institute of Rheumatology and Physiotherapy (Budapest, Hungary) from 2002, were enrolled in an open, observational study. At baseline, patient and disease characteristics were registered. Disease activity was evaluated (before the start of the treatment and after every 3 months) according to the JIA core set of the ACR paediatric definition of improvement (ACR Pedi). Adverse events (AEs) were documented. Results. In all, 72 patients were evaluated. Mean (S.D.) age at onset was 5.5 (3.8) years, mean disease duration was 7.4 (3.9) years. All disease activity parameters improved significantly in the first 3 months of treatment. After 3 and 12 months of treatment, 88 and 76% of patients, respectively, achieved the criteria of the ACR Pedi 30. AEs were uncommon. After 12 months, >85% of patients continued the therapy. Conclusion. Anti-TNF-a agents (etanercept and adalimumab) are effective, safe and well tolerated in JIA patients. Extension of this study for a longer follow-up period and to the patients with JIA after the age of 18 years (with validated and comparable disease activity parameters) is needed to evaluate the long-term effectiveness and safety of the TNF-a inhibitors.

Hereby, we report the case of a 12-year-old girl developing oligoarthritis and progressing into a polyarticular form. Rheumatoid factor was positive, and juvenile idiopathic arthritis (JIA) was diagnosed. After a poor response to DMARDs, an anti-TNF agent (infliximab) was initiated, but to be discontinued due to an allergic reaction. The same complication was observed with the fully human derivative, adalimumab. At the age of 22, the patient presented septicemia with severe anemia and subsequent development of leukopenia, myocarditis with heart failure, and ANA, aSm, aSS-A, aCL positives, and nephrotic syndrome. These new clinical manifestations fulfilled the classification criteria for the diagnosis of systemic lupus erythematosus. Due to the poor therapeutic responses for both diseases, alternative medical options have to be considered, such as targeted therapy with anti-CD20 or interleukin-6 receptor antagonist monoclonal antibodies. This patient may also be a candidate for autologous hemopoietic stem cell transplantation.

The standardization of the criteria to evaluate improvement in rheumatic diseases has been a goal of numerous research groups. This work led to establishment of definition of response in rheumatoid arthritis (1), juvenile arthritis (2–4), systemic lupus erythematosus (SLE) both in adults (5–7) and children (8–10). The International Myositis Outcome Assessment and Clinical Studies (IMACS) group proposed a core set of outcome variables for inclusion in clinical trials in adult and juvenile inflammatory myopathies and defined the degree of change in each core set variables that is clinically meaningful, as well as guidelines for performing clinical trials (11–14). However, until now these proposals have not yet been formally validated in the context of external prospective pediatric studies or clinical trials. Although children/adolescents and adults with DM share many signs and symptoms of disease, they differ in the clinical features and outcome (15–17), and treatment approaches should consider the peculiarities of juvenile patients as well as their longer life expectancy. Therefore, all outcome measures developed for adults need to be subjected to a critical evidence-based evaluation of their measurement properties in children and adolescents. To help standardize the conduct and reporting of juvenile dermatomyositis (JDM) clinical trials and enhance identification of new therapeutic agents, the Pediatric Rheumatology International Trials Organization (PRINTO) (18), in collaboration with the Pediatric Rheumatology Collaborative Study Group (PRCSG) and with the support of the European Union and the U.S. National Institutes of Health, undertook in year 2000 a multinational effort to develop, and promulgate a core set of outcome variables and a definition of clinical improvement to evaluate response to therapy in patients with JDM and in juvenile SLE. The first two phases of the project, previously published (8;19), led to the development of a prospectively evidence-based validated core set of six variables for the evaluation of response to therapy that is now known as the provisional PRINTO/American College of Rheumatology/European League Against Rheumatism Disease Activity Core Set for the evaluation of response to therapy in JDM (PRINTO/ACR/EULAR JDM core set) (Table 1). Table 1 Final domains and suggested variables included in the final PRINTO/ACR EULAR core set for the evaluation of response to therapy in JDM (adapted from ref. (19)). In this paper we report the results of the third phase of the project, which was aimed at developing a provisional validated definition of improvement to aid in the classification of individual patients in future therapeutic trials and in current clinical practice as either improved or not improved.

The authors present case reports with juvenile idiopathic arthritis being associated with functional constipation but not with inflammatory bowel disease. The cause of megacolon was only cleared in one case in the form of Hirschprung’s disease. The skeletal symptoms could be classified as seronegative spondylathropathy. Surgical treatment resulted in the remission of arthritis in one case and a possible pathogenic explanation is discussed. Common genetic predisposition is a plausible explanation, but fecal stasis leading to increased permeability to macromolecules, increased exposure to microbial and dietary antigens, loss of tolerance to own bacterial flora and host susceptibility to the increased antigenic load may stand in the background of this infrequent and not yet described association between juvenile idiopathic arthritis and functional constipation.

Objective To assess the long-term efficacy and safety of infliximab plus methotrexate in juvenile rheumatoid arthritis (JRA). Methods Patients eligible for the open-label extension (OLE, weeks 52–204) received infliximab 3–6 mg/kg every 8 weeks plus methotrexate. Results Of the 78/122 (64%) children entering the OLE, 42 discontinued infliximab, most commonly due to consent withdrawal (11 patients), lack of efficacy (eight patients) or patient/physician/sponsor requirement (eight patients). Infliximab (mean dose 4.4 mg/kg per infusion) was generally well tolerated. Infusion reactions occurred in 32% (25/78) of patients, with a higher incidence in patients positive for antibodies to infliximab (58%, 15/26). At week 204, the proportions of patients achieving ACR-Pedi-30/50/70/90 response criteria and inactive disease status were 44%, 40%, 33%, 24% and 13%, respectively. Conclusions In the limited population of JRA patients remaining in the study at 4 years, infliximab was safe and effective but associated with a high patient discontinuation rate. Clinical trials registration number NCT00036374.

Background Hemorrhage-related mortality (HRM) associated with pelvic fractures continues to challenge trauma care. This study describes the management and outcome of hemodynamically unstable patients with a pelvic fracture, with emphasis on primary intervention for hemorrhage control and HRM. Methods Blunt trauma patients [Injury Severity Score (ISS) >= 16] with a major pelvic fracture (Abbreviated Injury Score, pelvis >= 3) and hemodynamic instability [admission systolic blood pressure (SBP) = 6 units of packed red blood cells (PRBCs)/24 hours) were included into a 48-month (ending in December 2003) multicenter retrospective study of 11 major trauma centers. Data are presented as the mean +/- SD. Results A total of 217 patients (mean age 41 +/- 19 years, 71% male, ISS 42 +/- 16) were studied. The admission SBP was 96 +/- 37 mmHg and the Glascow Coma Scale (GCS) 11 +/- 5. Patients received 4 +/- 2 liters of fluids including 4 +/- 4 units of PRBCs in the emergency room (ER). In total, 69 (32%) patients died, among whom the HRM was 19%; 29% of the deaths were due to pelvic bleeding. Altogether, 120 of the 217 (55%) patients underwent focused abdominal sonography for trauma (FAST) or diagnostic peritoneal aspiration (DPA) and diagnostic peritoneal lavage (DPL); 60 of the 217 (28%) patients were found to have pelvic binding in the ER. In all, 53 of 109 (49%) patients had no bleeding noted at laparotomy, 26 of 106 (25%) had no abdominal findings, and 15 of 53 (28%) had had no prior abdominal investigation (FAST/DPL/computed tomography). Angiography was positive in 48 of 58 (83%) patients. The HRM was highest in patients with laparotomy as the primary intervention (29%) followed by the angiography group (18%), the combined laparotomy/pelvic fixation group (16%), and the pelvic fixation-only group (10%). Conclusion HRM associated with major pelvic trauma is unacceptably high especially in the laparotomy group. Hence, nontherapeutic laparotomy must be avoided, concentrating instead on arresting pelvic hemorrhage. Standards of care must be implemented and abided by

Az idiopathiás inflammatoricus myopathiák szisztémás autoimmun megbetegedések, amelyek krónikus izomgyulladás által okozott progresszív izomgyengeséggel, továbbá egyes belső szervek, úgymint a cardiovascularis rendszer, a tüdő és az emésztőrendszer elváltozásaival jellemezhetők.Cél:79 juvenilis és felnőtt korú dermatomyositisben szenvedő beteg adatai alapján a betegség kórlefolyásának és a relapsusok gyakoriságának, továbbá a túlélési valószínűségének bemutatása.Módszer:A szerzők retrospektív módon elemezték 44, a Bohan- és Peter-féle diagnosztikus kritériumrendszer alapján 1976 és 2004 között diagnosztizált juvenilis dermatomyositisben szenvedő gyermek adatait. A túlélési valószínűséget a Kaplan–Meier-módszer segítségével számították ki. A juvenilis dermatomyositisben szenvedő gyermekek adatait 35 felnőtt dermatomyositises beteg adataival hasonlították össze.Eredmények:A betegség kórlefolyása az esetek több mint felében monofázisos volt, míg a betegek egyharmada policiklusos formában szenvedett. A relapsus kockázata a remissziót követő első évben volt a legnagyobb. A juvenilis betegek közül senki sem hunyt el, míg a felnőtt korú betegek között négy betegséghez köthető haláleset is előfordult.Összegzés:Nem találtak összefüggést a relapsusmentes túlélés és a betegség kezdeti terápiája között (glükokortikoid önmagában vagy immunszuppresszív szerrel kiegészítve). A betegek jelentős aránya szenvedett policiklusos vagy krónikus lefolyású betegségben. A relapsusok jelentkezésére egy hosszabb betegségmentes intervallum után is számítani lehet, ezért mindenképpen javasolt a betegek hosszabb távú követése, legkevesebb 2 éven keresztül. Bár igen kedvező eredményeket találtak a betegség túlélésével kapcsolatban, további vizsgálatok szükségesek a betegség funkcionális kimenetelét illetően.

Background This study reviews the efficacy of vacuum-assisted wound closure (VAWC) to obtain primary fascial closure of open abdomens after severe trauma. Methods The study population included shock resuscitation patients who had open abdomens treated with VAWC. The VAWC dressing was changed at 2- to 3-day intervals and downsized as fascial closure was completed with interrupted suture. The Trauma Research Database and the medical records were reviewed for pertinent data. Results Over 26 months, 35 patients with open abdomens were managed by VAWC. Six died early, leaving 29 patients who were discharged. Of these, 25 (86%) were successfully closed using VAWC at a mean of 7 +/- 1 days (range, 3-18 days). Of the four patients that failed VAWC, two developed fistulas. No patients developed evisceration, intra-abdominal abscess, or wound infection. Conclusion VAWC achieved early fascial closure in a high percentage of open abdomens, with an acceptable rate of complications.

To identify preliminary core sets of outcome variables for disease activity and damage assessment in juvenile systemic lupus erythematosus (JSLE) and juvenile dermatomyositis (JDM).Two questionnaire surveys were mailed to 267 physicians from 46 different countries asking each member to select and rank the response variables used when assessing clinical response in patients with JSLE or JDM. Next, 40 paediatric rheumatologists from 34 countries met and, using the nominal group technique, selected the domains to be included in the disease activity and damage core sets for JSLE and JDM.A total of 41 response variables for JSLE and 37 response variables for JDM were selected and ranked through the questionnaire surveys. In the consensus conference, domains selected for both JSLE and JDM activity or damage core sets included the physician and parent/patient subjective assessments and a global score tool. Domains specific for JSLE activity were the immunological tests and the kidney function parameters. Concerning JDM, functional ability and muscle strength assessments were indicated for both activity and damage core sets, whereas serum muscle enzymes were included only in the activity core set. A specific paediatric domain called 'growth and development' was introduced in the disease damage core set for both diseases and the evaluation of health-related quality of life was advised in order to capture the influence of the disease on the patient lifestyle.We developed preliminary core sets of measures for disease activity and damage assessment in JSLE and JDM. The prospective validation of the core sets is in progress.

Objectives Juvenile idiopathic arthritis (JIA) is a chronic inflammatory joint disease affecting children. Even if remission is successfully induced, about half of the patients experience a relapse after stopping anti-inflammatory therapy. The present study investigated whether patients with JIA at risk of relapse can be identified by biomarkers even if clinical signs of disease activity are absent. Methods Patients fulfilling the criteria of inactive disease on medication were included at the time when all medication was withdrawn. The phagocyte activation markers S100A12 and myeloid-related proteins 8/14 (MRP8/14) were compared as well as the acute phase reactant high-sensitivity C reactive protein (hsCRP) as predictive biomarkers for the risk of a flare within a time frame of 6 months. Results 35 of 188 enrolled patients experienced a flare within 6 months. Clinical or standard laboratory parameters could not differentiate between patients at risk of relapse and those not at risk. S100A12 and MRP8/14 levels were significantly higher in patients who subsequently developed flares than in patients with stable remission. The best single biomarker for the prediction of flare was S100A12 (HR 2.81). The predictive performance may be improved if a combination with hsCRP is used. Conclusions Subclinical disease activity may result in unstable remission (ie, a status of clinical but not immunological remission). Biomarkers such as S100A12 and MRP8/14 inform about the activation status of innate immunity at the molecular level and thereby identify patients with unstable remission and an increased risk of relapse.

A szerzők jelen munkában négy juvenilis betegük esetét mutatják be, akiknél a krónikus ízületi gyulladást kísérő megacolont észleltek. A két betegség lefolyását és egymással való (feltehetőleg patogenetikai) kapcsolatát ismertetik, felhívva a figyelmet arra, hogy nem véletlen társulásról lehet szó. A mozgásszervi klinikai kép alapján mind a négy eset a szeronegatív spondylarthritisek csoportjába sorolható. A hasi statust illetően fizikális vizsgálattal minden alkalommal a bal oldali iliacalis régióban masszív rezisztenciát, nagy mennyiségű scibalakkal telített megacolont észleltek. A megacolon egy esetben bizonyult valódi Hirschprung-betegségnek, műtéti megoldására egy esetben került sor, amely egyúttal az arthritis remisszióját is eredményezte. A többi betegnél a krónikus obstipáció, megacolon továbbra is megoldásra vár.

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)

Objective. To evaluate the safety and efficacy of infliximab in the treatment of juvenile rheumatoid arthritis (JRA). Methods. This was an international, multicenter, randomized, placebo-controlled, double-blind study. One hundred twenty-two children with persistent polyarticular JRA despite prior methotrexate (MTX) therapy were randomized to receive infliximab or placebo for 14 weeks, after which all children received infliximab through week 44. Patients received MTX plus infliximab 3 mg/kg through week 44, or MTX plus placebo for 14 weeks followed by MTX plus infliximab 6 mg/kg through week 44. Results. Although a higher proportion of patients in the 3 mg/kg infliximab group than in the placebo group had achieved responses according to the American College of Rheumatology (ACR) Pediatric 30 (Pedi 30) criteria for improvement at week 14 (63.8% and 49.2%, respectively), the between-group difference in this primary efficacy end point was not statistically significant (P = 0.12). By week 16, after the crossover from placebo to infliximab 6 mg/kg when all patients were receiving infliximab, an ACR Pedi 30 response was achieved in 73.2% of all patients. By week 52, ACR Pedi 50 and ACR Pedi 70 responses had been reached in 69.6% and 51.8%, respectively, of patients. Infliximab was generally well tolerated, but the safety profile of infliximab 3 mg/kg appeared less favorable than that of infliximab 6 mg/kg, with more frequent occurrences of serious adverse events, infusion reactions, antibodies to infliximab, and newly induced antinuclear antibodies and antibodies to double-stranded DNA observed with the 3 mg/kg dose. Conclusion. While infliximab at 3 mg/kg and 6 mg/kg showed durable efficacy at 1 year, achievement of the primary efficacy end point at 3 months did not differ significantly between infliximab-treated and placebo-treated patients. Safety data indicated that the 6-mg/kg dose may provide a more favorable risk/benefit profile. These results warrant further investigation in children with JRA. © 2007, American College of Rheumatology.

JIA affected sibling pairs present high correlation for ANA and ILAR category G Filocamo, C Malattia, I Foeldvari, V Stanevicha, S Nielsen, T Herlin, C Pruunsild, F Zulian, Z Balogh, F Dressler, I Rumba, MG Alpigiani, E Cortis, F Falcini, R Trauzeddel, G Calcagno, L Lepore, M Alessio, DN Glass, SD Thompson, A Martini, N Ruperto, for the Paediatric Rheumatology International Trials Organisation (PRINTO)

SummaryA single-blind, crossover trial was carried out in 24 patients with definite or classical rheumatoid arthritis to compare the effectiveness and tolerability of piroxicam (20 mg once daily) with that of indoniethacin (25 mg 3-times daily), ibuprofen (400 mg 3-times daily) and placebo. Each drug was given in random order for a period of 1 week. Pain, joint tenderness and morning stiffness decreased and grip strength increased with piroxicam compared to placebo; no statistically significant difference could be found between piroxicam and the two other active agents. A reduction in joint circumference could not be demonstrated with piroxicam or ibuprofen but most patients did not have reducible swelling in their finger joints. Piroxicam was just as effective as the other two drugs overall, but fewer side-effects were noted with piroxicam.