The magnetics diagnostic set for ITER

The current status of the ITER magnetics diagnostic is summarised. The various risks are discussed on the basis of when they could be present during ITER operation and appropriate mitigation is reviewed. An implementation risk or common mode operation failure risk to the magnetic diagnostic can only be mitigated by diversity. All identified risks are being handled with ongoing research and development to minimise these risks. A reasonable level of design and manufacturing diversity has been therefore introduced in the procurement and installation plans, in order to optimise overall reliability avoiding dispersion of resources.     

Determination of anthocyanin content in C.V Monastrell grapes during ripening period using several procedures

The accumulation of anthocyanins in grape skin and their evolution during ripening were evaluated in Monastrell grapes grown at four different sites growing season. Of the different techniques used for this purpose, we used one chromatographic [high‐performance liquid chromatography (HPLC)] and three spectrophotometric (Saint‐Cricq, Fragoso‐García and Lamadon) methods. The grapes from two of the sites (Agüeros and Rubializas) presented much higher values of anthocyanin (1279 and 1207 mg kg^?1 respectively) compounds than the grapes from the other two sites. Of the different methods used, HPLC proved to be an invaluable tool for identifying and quantifying individual anthocyanins in a sample, while the Fragoso‐García technique was the best of the spectrophotometric methods for laboratories that do not have access to HPLC equipment. Application of principal component determination (PCA) to our experimental data resulted satisfactory classification of wines in terms of their geographical origin. High correlation coefficients were observed among the different spectrophotometric techniques (0.88 between Fragoso‐García and Saint Cricq; and 0.92 between Lamadon and Saint Cricq) and lower coefficients between HPLC and the spectrophotometric techniques.     

Determinant spreading associated with demyelination in a nonhuman primate model of multiple sclerosis

Definition of the immune process that causes demyelination in multiple sclerosis is essential to determine the feasibility of Ag-directed immunotherapy. Using the nonhuman primate, Callithrix jacchus jacchus (common marmoset), we show that immunization with myelin basic protein and proteolipid protein determinants results in clinical disease with significant demyelination. Demyelination was associated with spreading to myelin oligodendrocyte glycoprotein (MOG) determinants that generated anti-MOG serum Abs and Ig deposition in central nervous system white matter lesions. These data associate intermolecular "determinant spreading" with clinical autoimmune disease in primates and raise important issues for the pathogenesis and treatment of multiple sclerosis.     

Growth rate characteristics of acoustic neuromas associated with neurofibromatosis type 2

Neurofibromatosis type 2 (NF2) is a dominantly inherited disorder characterized by the occurrence of bilateral acoustic neuromas (ANs and other central nervous system tumors. Magnetic resonance images and audiologic data on 22 patients with NF2 who underwent multiple studies at the National Institutes of Health between 1983 and 1993 were reviewed to determine the growth characteristics of ANs in these patients. The average growth rate of ANs in NF2 patients was 0.30 cm3 per year and was significantly higher in older patients (0.75 cm3 per year) than in younger ones (0.12 cm3 per year). Larger ANs were more commonly found in patients with concomitant spinal tumors or meningiomas. NF2 patients with spinal tumors but not meningiomas demonstrated faster growth rates than patients without additional tumor burden. The data from this study suggest that older patients or patients with associated spinal tumors have faster growing ANs and therefore should be followed closely and treated aggressively.     

Germ-line mutations in the neurofibromatosis 2 gene: correlations with disease severity and retinal abnormalities

Neurofibromatosis 2 (NF2) features bilateral vestibular schwannomas, other benign neural tumors, and cataracts. Patients in some families develop many tumors at an early age and have rapid clinical progression, whereas in other families, patients may not have symptoms until much later and vestibular schwannomas may be the only tumors. The NF2 gene has been cloned from chromosome 22q; most identified germ-line mutations result in a truncated protein and severe NF2. To look for additional mutations and clinical correlations, we used SSCP analysis to screen DNA from 32 unrelated patients. We identified 20 different mutations in 21 patients (66%): 10 nonsense mutations, 2 frameshifts, 7 splice-site mutations, and 1 large in-frame deletion. Clinical information on 47 patients from the 21 families included ages at onset and at diagnosis, numbers of meningiomas, spinal and skin tumors, and presence of cataracts and retinal abnormalities. We compared clinical findings in patients with nonsense or frameshift mutations to those with splice-site mutations. When each patient was considered as an independent random event, the two groups differed (P < or = .05) for nearly every variable. Patients with nonsense or frameshift mutations were younger at onset and at diagnosis and had a higher frequency and mean number of tumors, supporting the correlation between nonsense and frameshift mutations and severe NF2. When each family was considered as an independent random event, statistically significant differences between the two groups were observed only for mean ages at onset and at diagnosis. A larger data set is needed to resolve these discrepancies. We observed retinal hamartomas and/or epiretinal membranes in nine patients from five families with four different nonsense mutations. This finding, which may represent a new genotype-phenotype correlation, merits further study.     

Chronic lymphatic leukemia. Peroral cladribine as primary treatment

Ten patients with newly diagnosed B-chronic lymphocytic leukaemia were treated with cladribin orally for five days every four weeks with a median of four series. This is the first reported clinical study where a purine analogue is administered orally. The tumour reducing effect was fast. Eight out of 10 patients responded with a partial or clinical complete remission. Two of these were in molecular biological complete remission. With an observation time of 22 months we have seen no serious side effects so far. A randomized study (including a long term follow up) between chlorambucil, fludarabin and cladribin is needed to clarify the future role of cladribin in B-CLL treatment.     

Psychogenic stress: theory, experiment, practice

Experiments on animals (rodents, beasts, primates) and observation in man have ascertained that psychogenic stress is the most important defensive state of living organisms, which is constantly involved in order to maintain their interaction with external psychogenic factors. Three types of stress should be distinguished, these include normostress, hypostress, and hyperstress. Normostress has definite limits within which optimal responses, which are typical for an individual are realized. Hyperstress develops as a defensive response to extremely strong (extraordinary) stimuli. Hypostress can be observed in the retarded development of self-regulation mechanisms. All three types of stress are provided by a fundamental property of living organisms--the self-regulation mechanism, which realizes the keeping back of normostress constancy, or return of hyper- and hypostress to the normostress status. And only if an extreme deficit of self-regulation mechanisms takes place, hyper(hypo)stress could become a condition for the development of pathology.     

Stability of fumonisins in thermally processed corn products

Little is known about the stability of fumonisins in corn-based foods during heating. This study investigated the effects of canning, baking, and roasting (dry heating) processes on the stability of fumonisins in artificially contaminated and naturally contaminated corn-based foods. All samples were analyzed for fumonisin levels by both a commercial enzyme-linked immunosorbent assay (ELISA) and a high-performance liquid chromatographic (HPLC) method. Canned whole-kernel corn showed a significant (P < or = 0.05) decrease in fumonisins by both ELISA (15%) and HPLC (11%) analyses. Canned cream-style corn and baked corn bread showed significant (P < or = 0.05) decreases in fumonisin levels at an average rate of 9% and 48%, respectively, as analyzed by ELISA. Corn-muffin mix artificially contaminated with 5 micrograms of fumonisin B1 (FB1) per g and naturally contaminated corn-muffin mix showed no significant (P < or = 0.05) losses of fumonisins upon baking. Roasting cornmeal samples artificially contaminated with 5 micrograms of FB1 per g and naturally contaminated cornmeal samples at 218 degrees C for 15 min resulted in almost complete loss of fumonisins.