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Big data is the present and the future. It holds the potential to make corporations create big revenues, to let scientists solve vital issues and to help governments make informed policy decisions. Yet the massive collection and use of personal data also raises a host of issues, particularly for individuals’ privacy and data protection rights. In Europe, data protection regulation is widely regarded as the answer to these issues. Through regulating data flows and providing individuals control over their personal data, some of big data’s possible undesired effects may be prevented. Yet upon closer scrutiny, it is uncertain whether European data protection legislation suffices to address these negative consequences. The practices in big data projects and the threshold for data to fall within the material scope of EU data protection legislation raise doubts about data protection’s applicability to big data.

OBJECTIVE: The prevalence of osteoarthritis (OA) increases, but the impact of the disorder on peoples' functional capacity is not known. Therefore, the objective of this study was to compare self-reported health status and functional capacity of subjects with early OA of hip and/or knee to reference data of healthy working subjects and to assess whether this capacity is sufficient to meet physical job demands. METHODS: Self-reported health status and functional capacity of 93 subjects from the Cohort Hip and Cohort Knee (CHECK) were measured using the Short-Form 36 Health Survey and 6 tests of the Work Well Systems Functional Capacity Evaluation. Results were compared with reference data from 275 healthy workers, using t-tests. To compare the functional capacity with job demands, the proportions of subjects with OA performing lower than the p(5) of reference data were calculated. RESULTS: Compared to healthy workers, the subjects (mean age 56) from CHECK at baseline reported a significantly worse physical health status, whereas the women (n = 78) also reported a worse mental health status. On the FCE female OA subjects performed significantly lower than their healthy working counterparts on all 6 tests. Male OA subjects performed lower than male workers on 3 tests. A substantial proportion of women demonstrated functional capacities that could be considered insufficient to perform jobs with low physical demands. CONCLUSIONS: Functional capacity and self-reported health of subjects with early OA of the hips and knees were worse compared to healthy ageing workers. A substantial proportion of female subjects did not meet physical job demands.

Objective. To examine the work participation of Dutch people with early osteoarthritis (OA) in hips or knees and compare this with data from the American Osteoarthritis Initiative (OAI) cohort. The influence of health status and personal factors on work participation was analyzed. Methods. In the Cohort Hip and Cohort Knee (CHECK) study, 1,002 subjects were included. Baseline questionnaire data from 970 subjects were analyzed. Rate ratios were calculated to compare work participation with the general Dutch population, after correcting (by stratifying) for age, sex, and education. Health status was measured using the Short Form 36 health survey and the Western Ontario and McMaster Universities Osteoarthritis Index. Groups were compared (CHECK versus OAI, workers versus nonworkers) using t-tests. Results. The mean age of the subjects was 56 years and 79% were women. Overall participation was 51%, similar to the general Dutch population and lower than in the OAI (76%). Point prevalence of sick leave because of hip/knee symptoms was 2%, and year prevalence was 12%. Of the subjects, 14% had made work adaptations. Workers reported significantly better health status (corrected for age, sex, and education) than nonworkers. Conclusion. Work participation of Dutch people with early OA is similar to the general population and significantly lower than American subjects. Increasing age, female sex, and lower education level were related to lower participation. Societal factors appear to have had more effect on work participation than health status in this stage of OA. The better health status of workers could not be explained solely by selection bias, but may be a result of work.

Objective:To describe the osteoarthritis study population of CHECK (Cohort Hip and Cohort Knee) in comparison with relevant selections of the study population of the Osteoarthritis Initiative (OAI) based on clinical status and radiographic parameters.Methods:In The Netherlands a prospective 10-year follow-up study was initiated by the Dutch Arthritis Association on participants with early osteoarthritis-related complaints of hip and/or knee: CHECK. In parallel in the USA an observational 4-year follow-up study, the OAI, was started by the National Institutes of Health, on patients with or at risk of symptomatic knee osteoarthritis. For comparison with CHECK, the entire cohort and a subgroup of individuals excluding those with exclusively hip pain were compared with relevant subpopulations of the OAI.Results:At baseline, CHECK included 1002 participants with in general similar characteristics as described for the OAI. However, significantly fewer individuals in CHECK had radiographic knee osteoarthritis at baseline when compared with the OAI (pConclusion:Although both cohorts focus on the early phase of osteoarthritis, they differ significantly with respect to structural (radiographic) and clinical (health status) characteristics, CHECK expectedly representing participants in an even earlier phase of disease.

OBJECTIVE: To determine whether MRI features are associated with development of radiographic knee OA and can be used as a predictive tool in early knee OA. METHODS: In 148 participants of the Cohort Hip and Cohort Knee study (mean age 56 years, 78% women), with a Kellgren Lawrence (KL) score ⩽1, we obtained semi-quantitatively scored knee MRI scans and radiographs at baseline. After 5 years, we determined the development of radiographic knee OA (KL ⩾2). We calculated odds ratios (ORs), with 95% CIs adjusted for age, sex and BMI, to identify MRI features associated with OA development. With these MRI features, we constructed an internally validated prediction model, for which we measured the area under the receiver operating characteristics curve, sensitivity and specificity. RESULTS: Radiographic OA developed in 28% of the participants after 5 years. Statistically significant associations were: cartilage defects OR = 1.7 (95% CI: 1.1, 2.6), osteophytes OR = 3.1 (1.7, 5.7), bone marrow lesions OR = 2.0 (1.2, 3.4), effusion OR = 2.1 (1.2, 3.5) and meniscal pathology OR = 2.8 (1.3, 6.3). With the combined MRI features in a prediction model, the sensitivity was 66%, the specificity 67% and the optimism-corrected area under the receiver operating characteristics curve 0.685. CONCLUSION: In early knee OA, MRI depicts significantly associated pathology in cartilage, bone and menisci, whereas the radiograph fails to detect these changes. Although MRI has potential for identifying patients at risk for developing radiographic knee OA, it cannot be used as an absolute diagnostic tool in early knee OA due to its low discriminative ability.

Objectives: A previous 24- week randomised trial demonstrated that sulfasalazine ( SSZ) treatment was superior to placebo ( PLAC) in suppressing disease activity in patients with oligo- and polyarticular onset juvenile idiopathic arthritis ( JIA). The current study determines the long- term outcome of the trial participants and evaluates whether the benefits of SSZ allocation are sustained over time.Methods: Between 2001 and 2003, 32 SSZ and 29 PLAC patients ( 90% of all patients) were prospectively examined clinically and by chart review, median 9 years ( range 7 to 10) after trial inclusion. In the follow- up assessment, variables of the American College of Rheumatology Pediatric 30 ( ACR Pedi 30) criteria were collected. The assessor was blinded to trial treatment allocation.Results: After the trial, patients had been routinely followed in rheumatology referral centres, and treated at the discretion of the attending physician. Almost all patients continued or started disease- modifying antirheumatic drugs ( DMARDs) ( SSZ 91%, PLAC 93%; SSZ treatment in about 80%). DMARD treatment appeared less intensive in the SSZ group as evidenced by a significantly shorter duration of SSZ use ( median 2.5 vs 5.2 years; p = 0.02) and a trend towards less use of methotrexate and other DMARDs. More than onethird of the patients reported long periods of non- compliance with DMARD treatment in both groups. At follow- up, 74% of the patients had active joints, and 30% showed active polyarthritis. Almost all outcome scores were better for SSZ compared with PLAC patients. Differences ( often exceeding 50%) were significant for the number of active joints, patients' overall well- being, number of patients with episodes of clinical remission off medication ( CROM) and duration of these episodes, patients in CROM and ACR Pedi 30 response at follow- up. Additional exploratory analyses performed to detect potential confounders related to patient characteristics or follow- up treatment showed that DMARD treatment compliance was positively correlated with an ACR Pedi 30 response ( odds ratio 3.8, 95% confidence interval ( CI) 1.1 to 13.4; p = 0.03). Adjusted for compliance, an SSZ patient was 4.2 times as likely as a PLAC patient to be an ACR Pedi 30 responder at follow- up ( 95% CI 1.3 to 14.3; p = 0.02).Conclusions: This follow- up study shows that effective suppression of disease activity by SSZ treatment early in active disease in JIA patients has beneficial effects that persist for many years. Given these results, compliance with DMARD treatment deserves serious attention.

Bone loss and fracture are not infrequent when patients with rheumatic disorders are given glucocorticoid treatment. Glucocorticoids inhibit bone formation and also increase the rate of bone resorption. In addition, they decrease intestinal calcium absorption and promote renal calcium excretion. Alendronate has proved effective in preventing and treating glucocorticoid-induced osteoporosis. Calcitriol reportedly is as effective as alendronate in preventing glucocorticoid-induced osteoporosis in patients having a renal or cardiac transplant. This double-placebo, double-blind trial enrolled 201 patients with rheumatic disease who received glucocorticoids in a daily dose equivalent to at least 7.5 mg of prednisone. They were assigned to receive 1Q mg alendronate or 1 μg of alfacalcidol, or respective placebo capsules, once a day, and bone mineral density (BMD) was monitored in the lumbar spine after 18 months of treatment. Patients given glucocorticoid therapy for longer than 12 weeks before the study were excluded. Mean daily and cumulative doses of glucocorticoid were very similar in the two groups. BMD in the lumbar spine increased 2.1% in patients given alendronate (95% confidence interval [CI], 1.1% to 3.1%) and decreased 1.9% in those given alfacalcidol (95% CI, 3.1 to 0.7%). After 18 months the mean difference in change in BMD was 4.0% (95% CI, 2.4 to 5.5%). A similar BMD pattern was evident in the femoral neck and total hip. Three alendronate-treated patients and eight of those given alfacalcidol had a new vertebral deformity, and three of the latter patients developed symptomatic vertebral fractures. The hazard ratio for new deformity in the alendronate group compared with alfacalcidol recipients was 0.4 (95% CI, 0.1 to 1.4). One patient given alendronate had an esophageal ulcer. Hypercalcemia was less frequent in the alendronate group. None of the participants developed thyroid or renal dysfunction during the study. The investigators conclude that alendronate is more effective than alfacalcidol for preventing glucocorticoid-induced bone loss in patients with rheumatic disorders.

Objective. To assess the efficacy, tolerability, and safety of sulfasalazine (SSZ) in the treatment of juvenile chronic arthritis (JCA). Methods. We conducted a 24-week randomized, placebo-controlled, double- blind, multicenter study of patients with active JCA of both oligoarticular and polyarticular onset. Patients were treated with a dosage of 50 mg/kg/day of SSZ (maximum 2,000 mg/day) or placebo. The efficacy variables were joint scores, physician's, parents', and patient's overall assessments, and laboratory parameters of inflammation. Results. Of the 69 patients enrolled, 52 (75%) completed the trial. Six patients (18%) withdrew from the placebo group, and 11 (31%) withdrew from the SSZ group (P = 0.18). In the intention- to-treat analysis of end point efficacy, between-group differences were significant for the overall articular severity score (P = 0.02), all global assessments (P = 0.01), and the laboratory parameters (P < 0.001). Adverse events occurred more frequently in the SSZ group and were the main reason for withdrawal (P < 0.001), but in all instances, these events were transient or reversible upon cessation of treatment. Conclusion. The results of this first placebo-controlled study show that SSZ is effective and safe in the treatment of children with oligoarticular- and polyarticular-onset JCA, although it was not well tolerated in one-third of the patients.

Fumaric acid esters (FAE) are used as an effective and safe oral treatment for plaque psoriasis in adult patients, but little is known about their efficacy and safety in children with psoriasis.To assess the effectiveness and safety of FAE in the treatment of paediatric psoriasis.This is a retrospective analysis of 14 paediatric patients with psoriasis (age18 years) treated with FAE between 2004 and 2012 at several Dutch university and regional clinics. Patients were identified through databases or registries.The median age at the start of FAE treatment was 15 years (range 8-17 years). The median duration of FAE treatment was 10 months (range 1-80 months), and the median maintenance dosage per day was 360 mg dimethylfumarate (range 240-600 mg). Five patients (36%) achieved a complete clearance of their psoriasis, one patient (7%) had a good improvement, three patients (21%) had a partial response and five patients (36%) were nonresponders. FAE treatment was well tolerated, but two patients (14%) discontinued FAE, one with severe diarrhoea and one with flushes. Five patients (36%) had transient, slightly abnormal laboratory values of liver-function tests or leucocytes that did not necessitate FAE dosage reduction or treatment discontinuation. No serious adverse events occurred.In this retrospective case series FAE seemed to be an effective and safe treatment for children with psoriasis. FAE may be an attractive therapeutic alternative to the currently used systemic immunosuppressive agents for paediatric patients with psoriasis. Further studies are needed to evaluate the suitability of FAE in paediatric psoriasis.

Dit is een casuistische verkenning waarin de beleving en hulpvraag van jongeren van wie de vader seropositief is of AIDS heeft, in beeld wordt gebracht. Vier jongeren uit drie gezinnen waarvan de vader HIV–geinfecteerd is, zijn geinterviewd. Alle vaders zijn homoseksueel. De jongeren lijken zich te kunnen handhaven in deze situatie, alhoewel die bijzondere eisen aan hen stelt. Zij zijn sterk betrokken bij het ziekteproces. Zelf geven zij aan geen problemen te ervaren waarvoor directe hulpverlening nodig is. Het betreft hier een eerste verkenning. De resultaten zijn niet generaliseerbaar. Voor de hulpverlening is meer kennis van deze groep kinderen nodig. Het blijkt onvoldoende bekend wie van de volwassen HIV/AIDS–patienten kinderen heeft. Het is belangrijk om de groep seronegatieve kinderen van seropositieve ouders met hun problemen te identificeren.

Juvenile psoriasis has a negative effect on the quality of life (QoL). The influence of treatments on QoL of these children has never been investigated before in a prospective observational study.To assess the Children's Dermatology Life Quality Index (CDLQI) in a cohort of patients with juvenile psoriasis and to evaluate the influence of treatments in daily clinical practice on CDLQI.We conducted a prospective observational study of children with psoriasis from a registry containing daily clinical practice data. Before and after treatment, QoL was assessed by the CDLQI and disease severity was documented by the Psoriasis Area and Severity Index (PASI). Three clusters of treatments were analysed: topical, dithranol and systemic therapy.In total, 125 patients were enrolled in the registry. Cross-sectionally, a mean ± SD CDLQI score of 7·5 ± 5·0 and a mean ± SD PASI of 7·0 ± 5·8 were recorded. Itching and problems with treatment had the highest impact on the children's QoL. Longitudinally, 85 patients were analysed with a total of 137 treatment episodes. All treatments contributed to a significant decline in total CDLQI score, with the largest decrease seen in dithranol and systemic treatments. A significant correlation was found between ΔCDLQI and ΔPASI for all treatment modalities. The highest positive impact of treatments was found in a decline of itch and sleep disturbance.In this first prospective observational study on CDLQI in juvenile psoriasis, a positive influence of treatments in daily clinical practice on QoL was demonstrated.

Recent genome-wide association studies have identified several genetic risk factors for psoriasis, but data on their association with age at onset are lacking.To compare the association between known risk alleles and psoriasis in well-defined cohorts with paediatric- and adult-onset psoriasis.Based on previous studies we selected seven genes and loci associated with psoriasis. Patients with paediatric-onset (18 years) and adult-onset psoriasis (≥ 18 years) and controls were genotyped. Genotype frequencies were compared between controls (n = 450) and all cases (n = 217), and between controls and cases stratified for confirmed age at onset (paediatric onset n = 80, adult onset n = 85).Paediatric-onset psoriasis showed a significant association with single nucleotide polymorphisms in the ERAP1 (P = 0.042) and IL23R loci (P = 0.042), LCE3C_LCE3B-del (P = 0.003) and HLA-C*06 (P = 1.72 × 10(-19)) when compared with the control group. A significant association of these four genes was also demonstrated when all psoriasis cases were compared with controls. In adult-onset psoriasis a significant association was found for HLA-C*06 (P = 5.11 × 10(-6)) and for LCE3C_LCE3B-del (P = 0.042). No associations were found for the IFIH1, IL12B and TRAF3IP2 loci.Notwithstanding the small cohort sizes, we demonstrated an association with established and recently discovered genetic risk factors in paediatric-onset psoriasis including genes involved in epidermal barrier function and adaptive immunity. Our data suggest that heritable factors may play a more important role in paediatric-onset psoriasis than in adult-onset psoriasis.

Combined heat and power (CHP) concepts for small-scale distributed power generation offer significant potential for saving energy and reducing CO2 emissions. Microturbines are an interesting candidate for small CHP systems with advantages in terms of performance, size, noise, and costs. MTT is developing a 3 kW recuperated microturbine for micro CHP applications for large households and for truck combined APU-heating systems. To minimize costs, off-the-shelf automotive turbocharger technology has been used for the turbomachinery. During recent years, turbocharger turbomachinery performance and efficiencies have significantly increased, even for very small sizes. At the same time, efficient high-speed motor-generators have become available at relatively low prices. The development of a concept demonstrator started in May 2008. This program phase included a cycle analysis and component selection study around off-the-shelf turbomachinery, design of a custom combustor, recuperator and generator, and a test program. In this paper, results of the cycle definition, conceptual design and component matching study are presented. Next, the development of a detailed performance model is described and performance prediction results are given. Also, results of the test program and test analysis work are presented. Finally, from the conclusion of the demonstrator phase an outlook is given on the prototype design and performance, which will be the next phase of the development program.

Combined heat and power (CHP) concepts for small scale distributed power generation offer significant potential for saving energy and reducing CO2 emissions. Micro turbines are an interesting candidate for small CHP systems with advantages in terms of performance, size, noise and costs. MTT is developing a 3kW recuperated micro turbine for micro CHP applications for large households and for truck combined APU-heating systems. To minimize costs, off-the-shelf automotive turbocharger technology has been used for the turbomachinery. During recent years, turbocharger turbomachinery performance and efficiencies have significantly increased, even for very small sizes. At the same time, efficient high-speed motor-generators have become available at relatively low prices. The development of a concept demonstrator started in May 2008. This program phase included a cycle analysis and component selection study around off-the-shelf turbomachinery, design of a custom combustor, recuperator and generator and a test program. In this paper, results of the cycle definition, conceptual design and component matching study are presented. Next, the development of a detailed performance model is described and performance prediction results are given. Also, results of the test program and test analysis work are presented. Finally, from the conclusion of the demonstrator phase an outlook is given on the prototype design and performance, which will be the next phase of the development program.Copyright © 2010 by ASME

To compare the effects of alendronate and alfacalcidol in the prevention ofglucocorticoid-related osteoporosis in patients with a rheumatic disease.Randomised, double-blind, double-placebo clinical trial (www. clinicaltrials.gov; number: NCT00138983).A total of 201 patients with rheumatic disease who were starting glucocorticoid treatment at a daily dose that was equivalent to at least 7.5 mg of prednisone were randomised to alendronate (10 mg) and a placebo capsule ofalfacalcidol daily (n = 100) or alfacalcidol (1 microg) and a placebo tablet ofalendronate daily (n = 101) for 18 months. Primary outcome was change in lumbar spine bone mineral density at 18 months. The main secondary outcome was the incidence of morphometrically confirmed vertebral deformities.Overall, 163 patients completed the study. The bone mineral density of the lumbar spine increased by 2.1% (95% CI: 1.1-3.1) in the alendronate group and decreased by 1.9% (95% CI: -3.I--0.7) in the alfacalcidol group. At 18 months the mean difference in change in bone mineral density between the two groups was 4.0% (95% CI: 2.4-5-5). Three patients in the alendronate group had a new vertebral deformity, compared with 8 patients in the alfacalcidol group, including 5 symptomatic vertebral fractures in 3 patients; the hazard ratio was 0.4 (95% CI: 0.1-1.4).Alendronate was more effective than alfacalcidol in preventing glucocorticoid-induced bone loss during this 18-month trial in patients with rheumatic diseases who were starting glucocorticoid treatment.

To explore the relation of changes in measures of bone turnover and changes in bone mineral density (BMD) of the lumbar spine and total hip over 18 months in a double-blinded, randomized trial, comparing the effect of alfacalcidol (101 patients) versus alendronate (100 patients) on BMD in patients who recently started treatment with glucocorticoids for various rheumatic diseases.Associations between changes in serum procollagen type I C-propeptide (P1CP), fasting urine N-terminal telopeptide of type I collagen (NTx), serum calcium, parathyroid hormone (PTH), osteocalcin, and change from baseline in BMD over 18 months were explored with regression and correlation analyses.In both treatment groups, there was a statistically significant decrease in NTx. In the alfacalcidol group there was also a significant increase in P1CP and osteocalcin, in contrast to the alendronate group, but BMD in the alfacalcidol decreased versus an increase in the alendronate group (p0.001). In neither treatment group were changes in biochemical measures correlated with the change in BMD, with the exception of a negative correlation in the alendronate group between changes in total hip BMD and NTx. Use of alendronate resulted in an increased PTH in 27 patients, but the increase in BMD of these patients was not statistically significantly different compared to patients taking alendronate with normal PTH levels.Changes in BMD were not associated with changes in bone measures, with the exception of NTx in the alendronate group. For the patient taking glucocorticoids in clinical practice, the value of serial assessment of bone markers is low; changes in markers are no substitute for changes in BMD. ClinicalTrials.gov number: NCT00138983.

SKF’s primary tool for gas turbine engine performance analysis is GSP (Gas turbine Simulation Program), a component based modeling environment that is developed at National Aerospace Laboratory NLR and Delft University of Technology, The Netherlands. One of the applications is gas path analysis (GPA) using GSP’s generic adaptive modeling capability. With GSP, gas path analysis has been applied to different aero engines at several maintenance facilities. Additional functionalities have been developed to analyze multiple engine operating points and combine results of different adaptive modeling configurations automatically, resulting in more accurate and reliable GPA results. A ‘multi-point calibration’ method for the reference model was developed providing a significant improvement of GPA accuracy and stability. Also, a method was developed using ‘multiple analysis cycles’ on different condition indicator subsets, which successfully generated values for all condition parameters in cases with fewer measurement parameters than condition indicators and where measurement data are unreliable. The method has been successfully demonstrated on the GEM42 turbo shaft engine. A number of case studies have shown GPA results corresponding to available maintenance notes and inspection data. The extension of the GSP GPA tool with a database system provides a useful tool for analyzing engine history and comparison of analyzed component conditions throughout the fleet. When a large amount of analysis data is stored in the database, statistic analyses, trending and data mining can be performed. Also maintenance work scope effect on engine performance can be predicted. In this paper, the newly developed GSP gas path analysis functionalities are described and experiences and results with the GEM42 engine operational environment are presented.Copyright © 2006 by ASME

Contains fulltext : 49196.pdf (Publisher’s version ) (Closed access) OBJECTIVE: To evaluate the sensitivity to change of a newly developed radiologic assessment tool, the Dijkstra score, and to develop a numeric composite score and progressor classification scheme to apply in juvenile idiopathic arthritis (JIA) trials. METHODS: A placebo-controlled trial of sulfasalazine (SSZ) in patients with oligoarticular- and polyarticular-onset JIA yielded the data for this study. Data were obtained from 418 sets of radiographs of the clinically involved and contralateral joints (at study entry and at 6 months' followup) from 66 JIA patients. The Dijkstra score assesses the presence or absence of swelling, osteopenia, joint space narrowing, growth abnormalities, subchondral bone cysts, erosions, and malalignment. These signs were combined in the Dijkstra composite score, to assess inflammation (DI), growth (DG), and damage (DD). Progression was defined as an increase in either the DG or the DD score. Scores were evaluated among all radiographs, a standard set of films (hand, foot, and knee), and per patient. All scores were used to explore differences between the 2 treatment groups. RESULTS: Over time, 58% of joints remained normal, 23% remained abnormal but stable, 14% showed an increase in signs, and 5% showed a decrease in signs. Of the 66 JIA patients, 12% had normal radiographic findings throughout followup, 27% showed abnormalities at some sites without change, and 61% showed change in at least 1 site. Changes in the DI, DG, and DD scores varied considerably per type of joint and occurred most frequently in joints of the standard set. DI and DG scores changed most often in the knees, while DD scores changed primarily in the hands and feet. The disease course in 8% of joints was classified as progressive. Films of SSZ-treated patients, versus the placebo group, showed less deterioration by the DD scores (P = 0.04), and the disease course was more often classified as nonprogressive in the SSZ group (P = 0.037). When progressors were defined as those who had at least one radiograph showing progression, significantly more placebo-treated patients were considered progressors (P = 0.046). CONCLUSION: In this trial data set, the Dijkstra composite score and the resulting progressor classification system are comprehensive and feasible tools that are sensitive to change and discriminate between clinical situations. They should now be tested by other investigators and in other data sets.

For gas turbine engine performance analysis, a variety of simulation tools is available. In order to minimize model development and software maintenance costs, generic gas turbine system simulation tools are required for new modeling tasks. Many modeling aspects remain engine specific however and still require large implementation efforts. One of those aspects is adaptive modeling. Therefore, an adaptive modeling functionality has been developed that can be implemented in a generic component based gas turbine environment. A single component in a system modeling environment is able to turn any new or existing model into an adaptive model without extra coding. The concept has been demonstrated in the GSP gas turbine modeling environment. An object-oriented architecture allows automatic addition of the necessary equations for the adaptation to measurement values. Using the adaptive modeling component, the user can pre-configure the adaptive model and quickly optimize gas path diagnostics capability using experimentation with field data. The resulting adaptive model can be used by maintenance engineers for diagnostics. In this paper the integration of the adaptive modeling function into a system modeling environment is described. Results of a case study on a large turbofan engine application are presented.Copyright © 2004 by ASME

Objective. To describe radiologic features of patients with juvenile idiopathic arthritis (JIA) in a standardized manner, to test the reliability and feasibility of this description, and to correlate these features with clinical signs as a first step in the development of a standardized assessment method.Methods. The placebo-controlled study of sulfasalazine in patients with oligoarticular, extended oligoarticular, and polyarticular JIA performed by the Dutch Juvenile Idiopathic Arthritis Study Group yielded the data for this study. All trial entry radio-graphs (clinically involved joints and contralateral joints) were scored (in consensus by a skeletal radiologist and pediatric rheumatologist) for the presence of swelling, osteopenia, joint space narrowing, growth abnormalities, subchondral bone cysts, erosions, and mal-alignment.Results. Data on 67 of 69 patients were analyzed. The mean age was 9.1 years (range 2.5-17.6 years), and the median disease duration was 24 months (range 5-176 months). Thirteen percent of the patients were IgM rheumatoid factor (IgM-RF) positive, and 16% were HLA-B27 positive. All 68 clinically evaluated joints were included in the maximum of 19 radiographed joints (or ,joint groups) per patient. The mean number of radiographed joints per patient was 7 (range 2-15); knees, hands, ankles, and feet were most frequently affected. Fifty-eight patients (87%) had radiologic abnormalities in at least one joint (soft-tissue swelling in 63% of patients, growth disturbances in 48%, joint space narrowing in 28%, and erosions in 15%). In total, half of the radiographs of the clinically involved joints showed radiologic abnormalities, including two-thirds of the radiographs of the clinically affected hands and knees. Univariate analysis revealed a good correlation between the overall articular (clinical) severity and the presence of radiologic abnormalities (odds ratio [OR] 1.38, P Conclusion. Our model of describing and scoring radiologic abnormalities of radiographed joints in JIA was feasible, mostly reproducible, correlated well with the overall articular severity score, and added substantial new information not available on clinical examination.

This article describes the effects of sulfasalazine (SSZ) treatment on serum immunoglobulin (Ig) levels in 6 children with oligoarticular- or polyarticular onset juvenile chronic arthritis (JCA). None of the children who developed dysimmunoglobulinemia during treatment showed clinical symptoms of this adverse event, in particular none developed severe infections. All patients regained normal immunoglobulin levels after discontinuing SSZ treatment. One patient with a partial IgA deficiency at the start of SSZ treatment showed a slow increase in the IgA level during treatment. During follow-up (4-6 years), one patient spontaneously developed a dysimmunoglobulinemia and one patient developed diabetes mellitus. Based on these case reports and review of the literature we advocate monitoring of serum immunoglobulin levels while on SSZ treatment.

To register forms of sexual behaviour in 5-6-year-old Dutch children in connection to background features and the way parents interact with their child.Descriptive.Naarden, Bussum and Hilversumse Meent, the Netherlands.During the school year 1992/93 all Dutch parents of 5-6-year-old children attending the Medical Service were invited to complete a questionnaire comprising background information, questions on the parents' attitude towards and reaction to sexual behaviour of children and a questionnaire on sexual behaviour of the child (SBC).Data were collected on 362 children, 190 boys and 172 girls, mean age 5.6 years (response: 62%). Respondents with secondary education appeared to be somewhat overrepresented. Touching of own genitals' and 'playing doctor' were reported with the highest frequencies (95 and 63% respectively of boys, 85 and 86% of girls). 'Adult-like sexual behaviour' was observed but to a much lesser extent (7 and 8% respectively). Only the degree of family nudity was related to the total SBC score. The higher-educated parents displayed a more permissive attitude towards sexuality than the lower-educated.Children showed sexual behaviour notably directed at self-gratification and satisfaction of curiosity. Typically adult-like' sexual behaviour was observed in young children only rarely. Caution is therefore indicated before concluding on the basis of incidentally observed atypical sexual behaviour that a child is sexually abused.

Tuberous sclerosis is a rare disease and pulmonarymanifestations are extremely rare. Like pulmonary lymph-angioleiomyomatosis, tuberous sclerosis ofthe lung affectsonly women of childbearing age and carries a very poorprognosis. Theradiological andpathological features ofthetwo conditions in the lung are indistinguishable and someauthorsthereforeconsiderlymphangioleiomyomatosistobeaformefruste oftuberoussclerosis,1 althoughothersbelievethat the relationship is unclear.2In pulmonary lymphangioleiomyomatosis bilateraloophorectomy and high dose progesterone have sometimesbeen