Ankylosing spondylitis and sarcoidosis — Coincidence or association?

We report a 25-year-old woman presenting with sarcoidosis and bilateral sacroiliitis. Her sarcoidosis related symptoms (malaise, cough and dyspnoea) improved dramatically under treatment with steroids but severe back pain persisted. Only seven similar cases have been described over the last 40 years and the question of a possible association between the two diseases has been raised. However, prevalence data from the literature and the apparent lack of genetic links are better arguments for coincidence than for association

COMPARISON OF THE VALIDITY AND RELIABILITY OF SELF-REPORTED ARTICULAR INDICES

Our objective was to compare the validity and reliability of three formats for self-administered articular indices assessing pain (PAI) or swelling (SAI). Fifty-five patients with rheumatoid arthritis were asked to mark the degree of pain on a list of 16 joints (PAI list), to mark ‘painful joints' on a mannequin presenting 42 joints (PAI diagram), and to mark ‘swollen or tender joints' on a mannequin presenting 38 joints (SAI diagram). The test-retest reliability (intraclass correlation coefficient) ranged from 0.63 (SAI diagram) to 0.67 (PAI diagram) and 0.85 (PAI list). The correlation with clinical parameters was strongest for the PAI list and the SAI diagram. The association of the SAI diagram with clinical parameters increased with omission of the less reliable toe joints and/or weighting for joint size according to Lansbury. As expected, the short and weighted SAI diagram correlated more strongly with the physician-derived swollen joint count (r = 0.49), C-reactive protein (r = 0.49) and erythrocyte sedimentation rate (r = 0.41) than did the PAI list whereas the PAI list correlated more strongly with physician-derived tender joint count (r = 0.43), global pain measured on a numerical rating scale (r = 0.57) and the Health Assessment Questionnaire (r = 0.49) than did the SAI diagram. We concluded that patients' rating of tender and swollen joints on a mannequin diagram and calculation of a 26-joint and weighted articular index produces an excellent estimate of total joint inflammation, which may be useful in clinical, health services and epidemiological research. An articular index calculated from ratings of pain degree of 16 joints or joint groups may provide complementary informatio

Perspectives of genetically engineered microbes for groundwater bioremediation

Biodegradation is the main process for the removal of organic compounds from the environment, but proceeds slowly for many synthetic chemicals of environmental concern. Research on microbial biodegradation pathways revealed that recalcitrance is - among other factors - caused by biochemical blockages resulting in dysfunctional catabolic routes. This has raised interest in the possibility to construct microorganisms with improved catabolic activities by genetic engineering. Although this goal has been pursued for decades, no full-scale applications have emerged. This perspective explores the lagging implementation of genetically engineered microorganisms in practical bioremediation. The major technical and scientific issues are illustrated by comparing two examples, that of 1,2-dichloroethane where successful full-scale application of pump-and-treat biotreatment processes has been achieved, and 1,2,3-trichloropropane, for which protein and genetic engineering yielded effective bacterial cultures that still await application

A methodology for producing reliable software, volume 2

For abstract, see Vol. 1

A methodology for producing reliable software, volume 1

An investigation into the areas having an impact on producing reliable software including automated verification tools, software modeling, testing techniques, structured programming, and management techniques is presented. This final report contains the results of this investigation, analysis of each technique, and the definition of a methodology for producing reliable software

Nitrate remediation by iron redox reactions in soils

U.S. Department of the InteriorU.S. Geological SurveyOpe

Establishing score equivalence of the Functional Independence Measure motor scale and the Barthel Index, utilising the International Classification of Functioning, Disability and Health and Rasch measurement theory

Introduction: Two widely used outcome measures to assess functioning in neurological rehabilitation are the Functional Independence Measure (FIM™) and the Barthel Index. The current study aims to establish the equivalence of the total score of the FIM™ motor scale and the Barthel Index through the application of the International Classification of Functioning, Disability and Health, and Rasch measurement theory. Methods: Secondary analysis of a large sample of patients with stroke, spinal cord injury, and multiple sclerosis, undergoing rehabilitation was conducted. All patients were assessed at the same time on both the FIM™ and the Barthel Index. The International Classification of Functioning, Disability and Health Linking Rules were used to establish conceptual coherency between the 2 scales, and the Rasch measurement model to establish an exchange of the total scores. Results: Using the FIM™ motor scale, items from both scales linked to the International Classification of Functioning, Disability and Health d4 Mobility or d5 Self-care chapters. Their co-calibration satisfied the assumptions of the Rasch model for each of 3 diagnostic groups. A ceiling effect was observed for the Barthel Index when contrasted against the FIM™ motor scale. Conclusion: Having a Rasch interval metric to transform scores between the FIM™ motor scale and Barthel Index is valuable for monitoring functioning, meta-analysis, quality audits and hospital benchmarking

High rate, long-distance quantum key distribution over 250km of ultra low loss fibres

We present a fully automated quantum key distribution prototype running at 625 MHz clock rate. Taking advantage of ultra low loss fibres and low-noise superconducting detectors, we can distribute 6,000 secret bits per second over 100 km and 15 bits per second over 250km

Gene identification for the cblD defect of vitamin B12 metabolism

Background Vitamin B12 (cobalamin) is an essential cofactor in several metabolic pathways. Intracellular conversion of cobalamin to its two coenzymes, adenosylcobalamin in mitochondria and methylcobalamin in the cytoplasm, is necessary for the homeostasis of methylmalonic acid and homocysteine. Nine defects of intracellular cobalamin metabolism have been defined by means of somatic complementation analysis. One of these defects, the cblD defect, can cause isolated methylmalonic aciduria, isolated homocystinuria, or both. Affected persons present with multisystem clinical abnormalities, including developmental, hematologic, neurologic, and metabolic findings. The gene responsible for the cblD defect has not been identified. Methods We studied seven patients with the cblD defect, and skin fibroblasts from each were investigated in cell culture. Microcell-mediated chromosome transfer and refined genetic mapping were used to localize the responsible gene. This gene was transfected into cblD fibroblasts to test for the rescue of adenosylcobalamin and methylcobalamin synthesis. Results The cblD gene was localized to human chromosome 2q23.2, and a candidate gene, designated MMADHC (methylmalonic aciduria, cblD type, and homocystinuria), was identified in this region. Transfection of wild-type MMADHC rescued the cellular phenotype, and the functional importance of mutant alleles was shown by means of transfection with mutant constructs. The predicted MMADHC protein has sequence homology with a bacterial ATP-binding cassette transporter and contains a putative cobalamin binding motif and a putative mitochondrial targeting sequence. Conclusions Mutations in a gene we designated MMADHC are responsible for the cblD defect in vitamin B12 metabolism. Various mutations are associated with each of the three biochemical phenotypes of the disorder

Effects of creatine supplementation on muscle weakness in patients with rheumatoid arthritis

Background and objectives. Patients with rheumatoid arthritis (RA) frequently suffer from muscle weakness. Oral administration of creatine has been shown to improve muscle strength in healthy subjects. The objective of this study was to examine the effect of oral creatine supplementation on muscle weakness, disease activity and activities of daily living in patients with RA. Methods. During a period of 3 weeks, 12 patients with RA were treated with creatine monohydrate (20 g/day for 5 days followed by 2 g/day for 16 days). They were examined on entry and at the end of the study. The patients were investigated clinically, blood and urine samples were obtained, muscle biopsies were performed before and after treatment, muscle strength was determined, and self‐administered patient questionnaires were completed. Results. From all patients we were able to obtain full clinical and questionnaire data, while biopsies were taken from 12 patients at the start and from nine patients at the end of the study. Muscle strength, as determined by the muscle strength index, increased in eight of 12 patients. In contrast, physical functional ability and disease activity did not change significantly. The creatine concentration in serum and skeletal muscle increased significantly, while creatine phosphate and total creatine did not increase in skeletal muscle. The skeletal muscle creatine content was associated with muscle strength at baseline but not after administration of creatine. The changes in muscle strength were not associated with the changes in skeletal muscle creatine or creatine phosphate. Conclusion. Although the skeletal muscle creatine content and muscle strength increased with creatine administration in some patients with RA, a clear clinical benefit could not be demonstrated for this treatment when the patients were considered as one grou