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The OECD Nuclear Energy Agency Data Bank operates as an international centre of reference for its member countries with respect to basic tools, such as computer codes and data, for use in different nuclear applications. The NEA Data Bank, as part of an international network of data centres, maintains large databases containing experimental (EXFOR), bibliographic (CINDA), and evaluated (EVA) nuclear data, and makes them available online via its website or via the JANIS display program. As part of its responsibilities, the Data Bank is also in charge of the compilation, verification, and distribution of the Joint Evaluated Fission and Fusion (JEFF) library. In addition, the Data Bank provides a service to its users by collecting, validating and making available upon request computer codes and associated application data libraries in many different areas relevant to the nuclear field. As part of its knowledge preservation activities, large collections of integral benchmark experiments for data and code validation are also available in areas such as criticality safety (ICSBEP), reactor physics (IRPhE), fuel performance (IFPE), and radiation shielding (SINBAD). Finally, the Data Bank coordinates the development and distribution of a database of recommended chemical thermodynamic data (TDB) for radioactive waste management applications. The Data Bank provides also support to other NEA divisions, a major part of this support is allocated to the NEA Science programme.

The OECD Nuclear Energy Agency (NEA) is organising a co-operation between the major nuclear data evaluation projects in the world. The co-operation involves the ENDF, JEFF, and JENDL projects, and, owing to the collaboration with the International Atomic Energy Agency (IAEA), also the Russian BROND and the Chinese CENDL projects. The Working Party on international nuclear data Evaluation Cooperation (WPEC), comprised of about 20 core members, manages this co-operation and meets annually to discuss progress in each evaluation project and also related experimental activities. The WPEC assesses common needs for nuclear data improvements and these needs are then addressed by initiating joint evaluation efforts. The work is performed in specially established subgroups, consisting of experts from the participating evaluation projects. The outcome of these subgroups is published in reports, issued by the NEA. Current WPEC activities comprise for example a number of studies related to nuclear data uncertainties, including a review of methods for the combined use of integral experiments and covariance data, as well as evaluations of some of the major actinides, such as {sup 235}U and {sup 239}Pu. This paper gives an overview of current and planned activities within the WPEC.

The OECD Nuclear Energy Agency (NEA) Data Bank participates in the production of data and their distribution to users including the collection and validation as well as the distribution of the Joint Evaluated Fission and Fusion (JEFF) library. The JEFF project has evolved from two separate projects, namely the European Fusion File (EFF) and the Joint Evaluated File (JEF), with the release of JEFF-3.1, in May 2005. The NEA also provides tools for the EFF project, such as computer codes for nuclear energy and radiation physics applications. Of special interest for fusion applications are the integral experiments collected in the SINBAD database, with radiation shielding and dosimetry experiments including many fusion neutronics shielding experiments. In this paper an overview will be given of the NEA, and some examples of nuclear data services offered, such as the SINBAD database. JANIS, a display program evaluated and experimental data developed at the NEA will be mentioned briefly. The main emphasis will be given to the EFF project including the contents of the new JEFF-3.1 library, released in May 2005. Examples of recent work are given as well as a discussion on the forth-coming evaluation efforts among the EFF collaborators.

Background Iatrogenic nerve injury caused by heat from dissection instruments is a significant problem in many areas of surgery. The aim of the present study was to compare the risk of nerve injury for three different dissection instruments: monopolar and bipolar electrosurgery (ES) and an ultrasonically activated (US) instrument. Methods The biceps femoris muscle was cut in a standard manner just adjacent to the sciatic nerve using monopolar ES, bipolar ES or US shears. A total of 73 functional experiments were conducted in which the nerve was isolated, divided proximally, and stimulated supramaximally in 37 anaesthetized rats. The electromyographic (EMG) potential was recorded distally before and after each experiment. Nerve dysfunction was defined as more than 10 per cent loss of the evoked EMG potential. Fifty-nine nerves were examined histologically after dissection with the different instruments. The extent of heat damage was determined in four nerves that were divided with ES bipolar scissors and five that were divided with US shears. Results Reduction in the EMG potential was significantly more frequent in the monopolar ES group than in the US group. Morphological examination also showed significantly less nerve damage in the US group. Conclusion US instruments may be safer than ES for dissection close to nerves.

An eight-year-old girl with Aicardi syndrome (AIC) developed signs of increased intracranial pressure. A clinical and radiological investigation revealed a tumor in the posterior fossa, which was resected. The histopathological diagnosis was large-cell medulloblastoma. Eight months later, she died of a local recurrence, despite treatment with chemotherapy and radiotherapy according to a PNET protocol. In addition to the growth of a large-cell medulloblastoma at the location of the primary tumor and the meningeal spread of the tumor, the autopsy revealed major cortical and subcortical malformations of the brain. Various benign (e.g., plexus papillomas) and malignant tumors (angiosarcoma, embryonic carcinoma, and hepatoblastoma) have been reported in connection with Aicardi syndrome. A genetic analysis of AIC suggests that the mutation is localized on the distal part of the short arm of the X chromosome, an area that may be of importance for tumor development. This is the first report of a primary malignant brain tumor -- large-cell medulloblastoma -- in a patient with Aicardi syndrome.

Giant cell arteritis (GCA) is a chronic systemic vasculitis with a marked female predominance and restriction to old age. The disease process distinctly targets large and medium sized arteries, preferentially the aorta and its extracranial branches. Morphological observations indicate that the age and sex distribution of GCA is related to the occurrence of degenerative changes in the arterial wall. GCA is not a truly infectious vasculitis. However, an infection might be a triggering factor. Different centres report an increase in GCA incidence, but annual fluctuations have not been shown to be statistically significant. However, significant seasonal variations have been observed by several groups. The mortality is not increased in adequately treated patients. Although, alternative steroid-sparing agents have been proposed, corticosteroids are still the first treatment choice.

The OECD Nuclear Energy Agency (NEA) is organising a co-operation between the major nuclear data evaluation projects in the world. The co-operation involves the US ENDF project, the Western European JEFF project, the Japanese JENDL project, and, through collaboration with the IAEA, the Russian BROND project, the Chinese CENDL project and the IAEA FENDL project. The Korean data evaluation effort at KAERI presently has observer status within the co-operation.A Working Party comprised of 16 core members meets annually to discuss progress within each evaluation project and to assess common needs for nuclear data improvements. These needs are then addressed by initiating joint evaluation and/or measurement efforts. The work is performed in specially established subgroups, consisting of experts from the different evaluation projects. The results of these subgroups are published in reports issued by the NEA. Fourteen such reports have so far been issued.Present activities cover the following subjects:• Nuclear d...

Objective. To investigate morphometrically the relationship between the degree of inflammatory reaction and arterial cross-sectional dimensions in giant cell arteritis (GCA). Methods. The media and intima of cross-sectioned inflamed arteries from GCA patients were outlined. The media circumference and the media and intima cross-sectional areas were measured. The two segments in every biopsy displaying the least and most widespread media inflammation were compared, using a paired Student's t-test. Moreover, paired comparisons were made of the intima area in inflamed and noninflamed sectors in single cross-sections. Results. The segment in each biopsy showing the most widespread media inflammation had the largest circumference and volume of media and intima, the intima cross-sectional area increased disproportionately, The intima was thickest in inflamed sectors in single cross-sections. Conclusion. The close spatial correlation between inflammatory media infiltration and the marked intimal expansion supports the contention that promoting factors produced by inflammatory cells play a pathogenetic role in GCA.

The aim of the present study was to compare an ultrasonically activated instrument (US), monopolar electrosurgery, and bipolar electrosurgery (ES) with respect to heat production, nerve function, and nerve morphology following in vivo application. less thanbrgreater than less thanbrgreater thanThe biceps femoris muscle of anesthetized rats was cut in a standardized manner longitudinally 1 mm adjacent to the sciatic nerve using US shears, a monopolar ES knife, or a bipolar ES scissors. Activation time and temperature were recorded continuously within 1-4 mm of the activation site ipsilateral and contralateral to the nerve with two thermoelectric microsensors. Temperature rise and time delay of reaching the temperature maximum, as an expression of heat spread within tissue, maximum temperature, and thermal dose (equivalent time of exposure at 43A degrees C) were measured and calculated. A total of 49 functional experiments were conducted. The electromyographic (EMG) potential was recorded distally. Nerve dysfunction was defined as more than 10% loss of the evoked EMG amplitude. Forty-eight nerves were coded and submitted to blind histopathological examination, and morphological damage was graded on a 4-grade scale. less thanbrgreater than less thanbrgreater thanThe maximum temperature elevation and the thermal dose were significantly higher for the bipolar ES compared with the US instrument ( = 0.024, = 0.049), and with much less variation of results for the US instrument. The monopolar ES maximum temperature and thermal dose were lower, but a very large variation occurred, probably as a result of more random electrical spread to the ground electrode and muscle motion artifacts. Functional loss was least common in the US group-without being significant-compared to bipolar and monopolar ES. Moderate and severe morphological damage was significantly less common in the US group than in the monopolar ES group ( = 0.041). We found no statistically significant correlation between the highest temperatures and the degree of morphological damage or functional loss less thanbrgreater than less thanbrgreater thanThe temperature elevation depends strongly on the distance to the activated instrument. The bipolar ES scissors generates a higher maximum temperature and thermal dose with a greater variation in than the US. Functional loss and severe morphological damage were uncommon in all groups. Funding Agencies|FORSS

Hereditary diffuse leukoencephalopathy with spheroids (HDLS) was first identified among a Swedish kindred with 17 cases. The average age of onset was 36 years. Autopsy in four cases revealed the presence of axonal spheroids. The causative gene is unknown.We performed genealogical and longitudinal observations of the original kindred. Forty members were examined, five telephone-interviewed, and one of the original HDLS cases from 1984 was neuropathologically examined. The clinical course was documented. The cerebrospinal fluid (CSF) findings of two recently affected cases were examined, and one of those autopsied.Of those examined, two developed HDLS during our survey and 38 were healthy. Those interviewed by telephone were healthy. One had symptoms suggestive of HDLS in 1984, but autopsy during our survey showed no spheroids. This patient, two relatives healthy at our examination and one without symptoms at telephone interview had HDLS diagnoses in the 1984 report. Thus, four HDLS diagnoses were unconfirmed. The number of identified patients amounts to 15 among 75 individuals in four generations, including two recent cases who demonstrated a subacute multisystem encephalopathy in Case 1 and an insidious course in Case 2. CSF showed signs of neurodegeneration without inflammation, and autopsy verified HDLS in Case 1.Some HDLS cases were misdiagnosed with unspecified psychiatric diagnoses in affected relatives from the original 1984 publication. However, HDLS is an encephalopathy dominated by a frontal lobe syndrome with an inexorably progressive and fatal course, where the different symptomatology in two recent cases confirmed the existence of acute and chronic variants.

We report a case of successful surgical repair of an acute aortic dissection (Stanford Type A) in a severely malnourished 39-year old patient with anorexia nervosa (body mass index [BMI] 11.3 kg/m2) and essential hypertension. The case is of interest since 1) acute aortic dissection in patients with anorexia nervosa has not previously been described; 2) hypertension is extremely rare in patients with eating disorders; and 3) successful aortic repair in a patient with so low BMI has not been reported before. We conclude that extremely low BMI due to anorexia nervosa is not an absolute contraindication for major aortic surgery.

Myocardial capillary exchange capacity was investigated by stereologic and functional techniques in parallel during pressure-overload cardiac hypertrophy and after long-term antihypertensive therapy with the vasodilator felodipine. In 26-week-old female spontaneously hypertensive rats (SHR) blood pressure increased by 25% and left ventricular weight (LVW/BW) increased by 18% compared to Wistar-Kyoto rats (WKY). Myocardial capillary surface and volume densities normalized for organ weight were similar in both ventricles for both strains. Moreover, capillary surface density was higher sub-epicardially (EPI) than in the subendocardium (ENDO) in the left ventricle of SHR. Thirteen weeks of felodipine-therapy (SHR-Felo) normalized blood pressure without affecting LVW/BW although a transition from concentric to eccentric hypertrophy is known to occur. Myocardial capillary surface and volume densities and the left ventricular ENDO-EPI-gradient in surface density were similar to untreated SHR. However, felodipine-treatment increased right ventricular weight and capillary volume density. Functional capillary exchange was estimated in terms of permeability surface area products (PS) for Cr-EDTA and vitamin B12 and normalized for organ weight. PSCr-EDTA, PSB12 and the ratio PSCr-EDTA/PSB12 (an index of capillary permeability) were similar in SHR and WKY. Furthermore, the relation between functional and stereological indices of exchange capacity was investigated in a multiple linear regression analysis. However, no significant correlation between PS and neither capillary surface nor volume density was found. In conclusion, myocardial capillary exchange capacity was well adapted to the pressure overload cardiac hypertrophy present in female SHR. Despite induction of right ventricular hypertrophy, felodipine-treatment did not affect capillary exchange capacity. Furthermore, when functional and stereologic estimates were performed in parallel, the importance of dynamic factors for myocardial capillary exchange capacity (e.g. heterogeneity) was illustrated.

A population-based series of 198 children, aged 0 to 16.9 years, with primary brain tumors, diagnosed from 1970 to 1984, was retrieved from the Swedish Cancer Registry. After review of slides and reclassification of histology according to the American Cancer Society, the average annual incidence rate was estimated to be 34.9 per million, which is a very high incidence compared to other countries. The age distribution was not uniform as age group 0 to 4 included more children than age groups 5 to 9 and 10 to 14. The largest subgroups were astrocytomas (25%) and primitive neuroectodermal tumor (PNET)/medulloblastomas (MB) (21%). Associated diseases were neurofibromatosis and Rubinstein-Taybi syndrome. The overall male to female ratio was 1.08:1, the same as in the population at risk; but for PNET/MB, it was 1.8:1. The 5-year survival for all tumors was 54%, and the 15-year survival, 49%, with great variation between tumor subgroups.

Proc on line; International audience; The status of the Joint Evaluated Fission and Fusion file (JEFF) is described. JEFF-3.1 comprises a significant update of actinide evaluations, materials evaluations that have emerged from various European nuclear data projects, the activation library JEFF-3.1/A, the decay data and fission yield sub-libraries, and fusion-related data files from the EFF project. The revisions were motivated by the availability of new measurements, modelling capabilities and trends from integral experiments. Validations have been performed, mainly for criticality, reactivity temperature coefficients, fuel inventory and shielding of thermal and fast systems. Compared with earlier releases, JEFF-3.1 provides improved performance with respect to a variety of scientific and industrial applications. Following on from the public release of JEFF-3.1, the French nuclear power industry has selected this suite of nuclear applications libraries for inclusion in their production codes.

The OECD Nuclear Energy Agency (NEA) Data Bank is an international centre of reference for its member countries with respect to basic nuclear tools, such as computer codes and nuclear data. The Data Bank is part of an international network of data centres in charge of the compilation and dissemination of basic nuclear data. The NEA nuclear data services include the collection of data, validation and distribution of the Nuclear Data libraries via the NEA website, offering easy access to databases containing bibliographical and experimental information, as well as evaluated libraries, e.g., the Joint Evaluated Fission and Fusion (JEFF) library. The selection and distribution of chemical thermodynamic data for radioactive waste management applications is the subject of the Thermochemical Database Project (TDB), supported by 17 organisations in 12 member countries and co-ordinated by the NEA Data Bank. In support of data evaluation, and generation of project oriented data libraries, relevant computer codes in the field of nuclear models, experimental data processing and evaluated data processing are made available to experts with the support of the member countries. Large collections of benchmark experiments for data and code validation are also available from the NEA in areas such as criticality safety (ICSBEP), radiation shielding (SINBAD), fuel performance (IFPE) and reactor physics (IRPhE). The NEA Working Party on international nuclear data Evaluation Co-operation (WPEC) provides a framework for co-operative activities between the six major evaluation projects (BROND, CENDL, ENDF, FENDL, JEFF and JENDL).

The epidemiology of giant cell arteritis (GCA) may indicate a pathogenetic relationship between GCA and female sex hormone metabolism; GCA is two to four times more common in women compared with men. Our previous analyses gave no support for the hypothesis that the pathogenesis of GCA should be related to somatic mutations in the estrogen receptor alpha (ERalpha) gene. The object of the present study was to investigate the size of the estrogen receptor beta (ERBeta), and the size and nucleotide sequence of the ERBeta gene in temporal arteries in GCA.The ERBeta protein was analyzed by Western blot technique and the ERBeta gene by RT-PCR and direct sequencing of the PCR product.Western blot analysis revealed an ERBeta of normal size. There were no aberrations in size or nucleotide sequence in the ERBeta gene in the GCA patients.The present observations gave no support for the hypothesis that somatic mutations in the ERBeta gene should be involved in the pathogenesis of GCA.

OBJECTIVE: To assess female sex hormone related variables in a group of women with biopsy positive giant cell arteritis and a control group. METHODS: 49 women with biopsy positive giant cell arteritis, aged 50 to 69 years at the time of diagnosis, answered a questionnaire on hormonal and reproductive factors. The same questions were answered by a large population of women from the same geographical area in connection with routine mammograms. The results were tested statistically, using logistic regression analysis of each variable adjusted for age, and a multivariate logistic regression analysis including age and the variables which differed significantly between giant cell arteritis and controls. RESULTS: From the multivariate logistic regression analysis, three independent variables were associated with an increased risk of having giant cell arteritis: smoking and being an ex-smoker (odds ratio (OR) = 6.324 (95% confidence interval (CI), 3.503 to 11.418), p

Microdysgenesis is a microscopic malformation of cortical development characterized by heterotopic neurones and abnormal cortical architecture. It has been described in primary generalized and partial epilepsy. Its significance in epileptogenesis is controversial, partly due to lack of consensus of diagnostic criteria. Different terms have also been used for the malformation. Several quantitative studies have been performed of the histopathological aberrations associated with microdysgenesis. A majority of the studies have revealed an increased number of heterotopic neurones in specimens from epilepsy patients. However, the quantitative values given for abnormal numbers of white matter neurones vary greatly between studies and there is no consensus yet on quantitative criteria for microdysgenesis. There have also been conflicting results from studies correlating microdysgenesis with clinical data. Both favourable and worse outcome after epilepsy surgery have been reported in patients with increased numbers of white matter neurones and microdysgenesis. While some studies have shown earlier seizure onset and increased frequency of mental retardation in patients with microdysgenesis, others have not. Differences in inclusion criteria and definition might contribute to the contradictory results. There is some evidence that microdysgenesis could be important in epileptogenesis, but the mechanisms involved remain unknown and difficult to investigate. A consensus on what histopathological criteria to use for the diagnosis of microdysgenesis is needed to explore this further and enable comparisons between centres. There are advantages and disadvantages both with quantitative stereological and with qualitative assessments. It is necessary to evaluate these in the decision on diagnostic criteria, if possible taking both qualitative and quantitative aspects into account.

The Working Party on International Nuclear Data Cooperation represents a collaborative effort of major nuclear‐reaction data‐evaluation projects. The technical work is presently performed by 3 standing and 6 short‐term subgroups. Among the most recent achievements are the establishment of ModLib (modular nuclear reaction code library), the development of a new version of neutron cross‐section standards, and an effort to improve the 238U+n evaluation. The paper reviews achievements of the Working Party and outlines its future plans.

The Nuclear Energy Agency (NEA) is revising the formats and working methods of its nuclear data request list project. The objective is to shorten the request list and make this activity more responsive to the needs of data measurers, evaluators, and users. The Working Party for International Nuclear Data Evaluation Cooperation (WPEC) is responsible for leading this effort. An ad hoc group of experts met in October 2003 and May 2004 to discuss the issues and to make recommendations to WPEC at its annual meeting. The following recommendations were submitted: i) two distinct request lists should be maintained: general needs and high priority needs; ii) stringent criteria for inclusion of requests in these lists should keep them shorter; iii) a high priority request would need to be well justified and benefits that would accrue from satisfying the request clearly stated; iv) Subgroup C, consisting of one provider of data and one user of data from each regional evaluation project, should oversee the request lists; v) outside consultants should be recruited as needed to help review requests; vi) all requests should be reviewed periodically; vii) Subgroup C should actively solicit new data requests; and viii) the NEA website should be revised to reflect the new procedures. These recommendations have been accepted by WPEC. This paper discusses the new request list criteria, stewardship procedures, and features of the request list website. An example of a request submitted in the new format is provided.The Nuclear Energy Agency (NEA) is revising the formats and working methods of its nuclear data request list project. The objective is to shorten the request list and make this activity more responsive to the needs of data measurers, evaluators, and users. The Working Party for International Nuclear Data Evaluation Cooperation (WPEC) is responsible for leading this effort. An ad hoc group of experts met in October 2003 and May 2004 to discuss the issues and to make recommendations to WPEC at its annual meeting. The following recommendations were submitted: i) two distinct request lists should be maintained: general needs and high priority needs; ii) stringent criteria for inclusion of requests in these lists should keep them shorter; iii) a high priority request would need to be well justified and benefits that would accrue from satisfying the request clearly stated; iv) Subgroup C, consisting of one provider of data and one user of data from each regional evaluation project, should oversee the request li...

To investigate morphometrically the relationship between the degree of inflammatory reaction and arterial cross-sectional dimensions in giant cell arteritis (GCA).The media and intima of cross-sectioned inflamed arteries from GCA patients were outlined. The media circumference and the media and intima cross-sectional areas were measured. The two segments in every biopsy displaying the least and most widespread media inflammation were compared, using a paired Student's t-test. Moreover, paired comparisons were made of the intima area in inflamed and noninflamed sectors in single cross-sections.The segment in each biopsy showing the most widespread media inflammation had the largest circumference and volume of media and intima; the intima cross-sectional area increased disproportionately. The intima was thickest in inflamed sectors in single cross-sections.The close spatial correlation between inflammatory media infiltration and the marked intimal expansion supports the contention that promoting factors produced by inflammatory cells play a pathogenetic role in GCA.

To investigate the age and sex distribution of calcifications of the internal elastic membrane (IEM) in temporal arteries.Calcifications of the IEM were assessed light-microscopically in temporal arteries from 40 women and 21 men, aged 51 or more, who were known not to have giant cell arteritis (GCA). Their relation to age and the difference between women and men were tested statistically.The IEM calcifications differed morphologically from the calcifications in Mönckeberg's mediosclerosis and atherosclerosis. They increased significantly with age and were 2.62 times more common in women than men.Previous morphological studies indicate that the inflammatory process in GCA is initiated by a foreign-body, giant-cell attack on calcifications of the IEM. The present study showed that IEM calcifications in non-GCA controls show an age and sex distribution similar to that of GCA morbidity. The results may indicate that the presence of IEM calcifications in the general population influences the age and sex distribution of GCA. Furthermore, the findings support the hypothesis that the calcifications, although not disease specific, may play a pathogenetic role in the latter.

Giant cell arteritis (GCA) predominantly affects postmenopausal women. Estrogen receptor alpha (ER alpha) accumulates in the cytoplasm of smooth muscle cells, activated mononuclear inflammatory cells and giant cells in the temporal arteries of GCA patients, as well as in smooth muscle cells in arteries from non-GCA controls. The aim of this study was to analyse whether this accumulation is related to structural aberrations in the ER alpha mRNA leading to a change in protein structure.Total RNA was extracted from inflamed temporal artery tissue in two GCA patients and from non-inflamed arteries in two non-GCA controls. Products from the nested RT-PCR of the cDNA were cloned and plasmid inserts of 20 different clones from each case were investigated using nucleotide sequence analysis.A total of eight different types of transcripts lacking parts of the ER alpha mRNA were detected. Seven of these could be explained by alternative splicing. There were no significant differences between the GCA patients and the non-GCA controls in terms of the number of transcript variants.The accumulated cytoplasmic ER alpha in temporal arterial tissue from elderly persons appears mainly to be of wild type. The main structural changes in the ER alpha mRNA may be due to alternative splicing. Somatic mutations of the ER alpha gene appear to be rare and it is therefore unlikely that they are involved in the pathogenesis of GCA.

Giant cell arteritis (GCA) is a chronic inflammatory disorder targeting large and medium-sized arteries, which predominantly affects postmenopausal women. Its high incidence in populations with Scandinavian lineage, some familial accumulation, and the association with the HLA-DR4 haplotype indicate a genetic predisposition. Epidemiological observations, as well as the symptomatology, may indicate an infectious origin, but so far GCA has not been shown to be a truly infectious form of vasculitis. Immunological research indicates an antigen-driven disease with local T-cell and macrophage activation in the vessel wall. Morphologically, the inflammatory process appears to be initiated by a foreign-body giant-cell attack on calcified internal elastic membrane in arteries and on calcified atrophic parts of the aortic media. The ensuing diffuse chronic inflammation leads to vessel dilatation and extensive intimal thickening. The latter, which relates to the production of promoting factors by the inflammatory cells, causes arterial stenosis and ischemic complications. The possible role of female sex hormones in GCA requires further investigation. Mononuclear and giant cells in GCA display the cytoplasmic accumulation of estrogen receptor (ER) alpha. Cytoplasmic ER-alpha is also seen in media smooth-muscle cells in GCA and in non-GCA controls, but nucleotide sequence analysis of the ER-alpha gene revealed no differences between GCA patients and controls. In the future, comprehensive morphological, cell biological and immunological research will be required for a better understanding of the complex etiology and pathogenesis of GCA.

Giant cell arteritis (GCA) is a chronic form of vasculitis which predominantly affects women over 50 years of age. The aim of this study was to analyse the presence of estrogen receptor alpha (ER) in the temporal arteries of patients with GCA.Inflamed temporal artery biopsies from 43 GCA patients were stained with monoclonal antibodies to two different segments of the ER and compared with non-inflamed arteries from age- and sex-matched controls who had not received a clinical diagnosis of GCA. The protein that was extracted from 4 GCA-positive biopsies and 4 non-GCA controls was analysed using the Western blot method with a monoclonal antibody to ER. Reverse transcriptase-polymerase chain reaction (RT-PCR) analysis using primer pairs specific to ER-cDNA was performed on the total RNA from 4 GCA-positive biopsies and 4 non-GCA controls.The inflamed arteries expressed distinct cytoplasmic immunoreactivity to ER in activated mononuclear inflammatory cells and in giant cells. Biopsies from GCA patients and controls displayed cytoplasmic ER positivity in smooth muscle cells. Western blot analysis revealed two bands corresponding to approximately 64 and 54 kDa, respectively, in the inflamed arteries and controls. In the inflamed biopsies and non-GCA controls, RT-PCR analysis revealed a strong band corresponding to approximately 670 bp, as expected, and a weaker band corresponding to approximately 440 bp.In inflamed arteries from GCA patients, smooth muscle cells, activated mononuclear inflammatory cells and giant cells express cytoplasmic ER. Non-inflamed control arteries also express cytoplasmic ER in smooth muscle cells. The accumulation of cytoplasmic ER may suggest the involvement of estrogen not only in GCA but also in normal vascular aging. The results justify further investigations into the pathogenetic roles of estrogen metabolism in GCA.

To compare the morphology of foreign body and Langhans giant cells in the two different inflammatory phases of giant cell arteritis (GCA).Electron microscopy was performed on 6 positive temporal arterial biopsies. Light microscopy and immunocytochemistry for macrophage-associated antigen (KP1) and alpha-smooth muscle actin (alpha-SMA) were performed on 16 positive biopsies.A focal granulomatous reaction with foreign body giant cells was found only in association with the internal elastic membrane (IEM) in atrophic arterial segments, which often displayed calcification of the IEM. Diffuse invasion of lymphocytes and monocytes/macrophages affected non-atrophic as well as atrophic arterial segments. Within such segments Langhans giant cells were found in all layers of the wall. Electron microscopy of biopsies displaying the focal foreign body reaction revealed that large cells devoid of lysosomes but with cytoplasmic densities, tightly packed cytoplasmic filaments and numerous micropinocytotic vesicles formed clusters close to calcified parts of the internal elastic membrane. Furthermore, foreign body giant cells were surrounded by large cells devoid of lysosomes. Lysosomes tended to concentrate in central parts of the foreign body giant cells. In the diffusely inflamed arteries, the Langhans giant cells were surrounded by mononuclear cells rich in lysosomes. The lysosomes in the Langhans giant cells were more evenly distributed than in foreign body giant cells. Immunocytochemistry of biopsies displaying the focal granulomatous reaction revealed an uneven, often central immunoreactivity for the macrophage marker (KP1) in the foreign body giant cells, and immunostaining for alpha-smooth muscle antigen (alpha-SMA) showed their poor delineation from the surrounding vascular smooth muscle cells. The Langhans giant cells in the diffusely inflamed arteries displayed a strong even cytoplasmic immunoreactivity for KP1 and a distinct delineation from the smooth muscle cells in the alpha-SMA staining.Differences in terms of distribution, light microscopy, immunocytochemistry and electron microscopy between the two types of giant cells in GCA indicate a difference in their function as well as their histogenesis.

Temporal arterial biopsies from 27 patients with a clinical diagnosis of pure polymyalgia rheumatica (PMR) were examined using light microscopy on paraffin and plastic sections. The primary routine examination of the paraffin-embedded parts of the biopsies (biopsy length: 12.7 +/- 4.5 mm, sub-segments: 4.9 +/- 1.2 mm) revealed 4 positive cases, whereas the primary examination of the smaller plastic-embedded parts showed inflammation in 6 cases (biopsy length: 2.7 +/- 1.2 mm, sub-segments: 0.7 +/- 0.3 mm). Serial sectioning with a 50 microns interval of arteries which were negative primarily revealed three new positive cases in the paraffin-embedded material (total length: 174.0 mm), whereas sectioning the plastic-embedded material (total length: 52.8 mm) produced one more positive artery. All the new cases displayed a focal inflammatory process in atrophic, calcified arterial segments. The high yield of positive biopsies in the present material (11 of 27; 40.7%) demonstrates the diagnostic value of temporal arterial biopsy in PMR and the importance of a careful histologic examination. The results also indicate the influence of biopsy length on the yield of positive biopsies. The division of fixed temporal arterial biopsies into approximately 1-mm-long sub-segments before the embedding and the further serial sectioning of those negative biopsies which are atrophic and/or calcified is recommended in cases of pure PMR.

A 62-year-old man with a history of coronary insufficiency complained at his scheduled visit to the outpatient clinic of symptoms suggestive of gastritis. His blood hemoglobin concentration, however, was markedly increased. Results of a hematological work-up suggested an erythropoietin-producing tumor. Signs of increased intracranial pressure then led to the finding of a cerebellar tumor, which could explain his vertigo and abdominal symptoms. This cystic capillary hemangioblastoma probably was responsible for the erythropoietin production and also seemed to produce basic fibroblast growth factor. The clinical evaluation of polycythemia as well as erythropoietin biochemistry and clinical application are reviewed.

An increased concentration of gastrin was observed in plasma of male Sprague-Dawley strain rats fed on soybean protein diet for a 9-month period, compared with rats fed on casein diet. Both diets contained 0.5% (w/w) cholesterol. Protein-dependent differences were also observed in the fatty acid pattern of hepatic phospholipids, hepatic delta 6-desaturase activity, and plasma cholesterol. No signs of arteriosclerosis were observed in the aortas. Sixty percent of the hearts showed various degrees of lipid staining in coronary arterial branches of different sizes. Despite a large difference in plasma cholesterol level, there was no quantitative or qualitative difference between groups in the occurrence of coronary lipid staining.

The results of an international code comparison on decay heat are presented and discussed. Participants from more than ten laboratories calculated, using the same input data, decay heat for thirteen cooling times between 1 and 1013 sec. Two irradiation cases were proposed: fission pulse and 3 107 seconds of irradiation of 235U fuel. The results are analysed and compared. This inter-comparison shows that, if the same input data ate used, almost all of the participating codes give very similar results for the decay heat and consequently also for the fission product contribution.

Following the positive experience gained with the first version of the JEF (Joint Evaluated File) library, which was distributed to NEA Data Bank member countries in 1985, a significantly improved version of the library, JEF-2.0 (in ENDF-6 format), was distributed for testing at the end of February 1990. It is planned to compile, in the middle of 1991, following intensive benchmark testing, a version of the JEF-2 library for general use.

A slowly developing, mainly sensory, polyneuropathy was found in a 60-year-old man exposed to n-hexane (10-100 mg/m3) for 30 years in a mountain fuel stockpile. The nerve damage was verified by electromyography and sural nerve biopsy, and it is proposed that also a low-grade, but prolonged, exposure to n-hexane may cause polyneuropathy.

In spite of improvements in obstetric and neonatal care, hypoxic-ischemic brain damage with severe neurologic disability is still a clinical reality. A model in 7-day-old rats has been introduced to study the pathophysiology of perinatal hypoxic-ischemic brain damage. Unilateral brain damage is produced in the cerebral cortex, striatum and hippocampus, i.e. a similar distribution as is often seen in human asphyxiated neonates. In the present investigation the model was evaluated further by comparing three different methods to assess the brain damage: weighing the hemispheres, morphometry and somatosensory evoked potentials. Seven-day-old rats were subjected to unilateral carotid artery ligation followed by 2 h of hypoxia (7.7% O2 at 36 degrees C). After 2 h of hypoxic-ischemia pCO2 and pO2 decreased in mixed arterial/venous blood. The evaluation of the damage 2 weeks after the insult, demonstrated close correlation between morphometry and weighing (r = 0.836, P less than 0.01). The amplitude of evoked potentials correlated to the other parameters (r = 0.814, P less than 0.01 and r = 0.824, P less than 0.01 respectively) and displayed a greater relative attenuation than the other methods but with a more pronounced variability. These results indicate that the degree of brain damage can be assessed by weighing for screening purposes.

A population-based series of 198 children, aged 0 to 16.9 years, with primary brain tumors, diagnosed from 1970 to 1984, was retrieved from the Swedish Cancer Registry. After review of slides and reclassification of histology according to the American Cancer Society, the average annual incidence rate was estimated to be 34.9 per million, which is a very high incidence compared to other countries. The age distribution was not uniform as age group 0 to 4 included more children than age groups 5 to 9 and 10 to 14. The largest subgroups were astrocytomas (25%) and primitive neuroectodermal tumor (PNET)/medulloblastomas (MB) (21%). Associated diseases were neurofibromatosis and Rubinstein-Taybi syndrome. The overall male to female ratio was 1.08:1, the same as in the population at risk; but for PNET/MB, it was 1.8:1. The 5-year survival for all tumors was 54%, and the 15-year survival, 49%, with great variation between tumor subgroups.

The gamma-ray production cross section of oxygen has been measured at incident neutron energies between 7 and 10.5 MeV. The production of the 6.13-, 6.92-, and 7.12-MeV gamma rays by the (n,n'..gamma..) reaction in /sup 16/O and the 3.09-, 3.68-, and 3.85-MeV gamma rays by the (n,..cap alpha gamma..) reaction has been studied. In addition, the production cross section of the 4.44-MeV gamma ray from inelastic neutron scattering on carbon has been measured at one neutron energy, since many earlier measurements of gamma-ray production cross sections have been performed relative to this cross section. Monoenergetic neutrons were produced by the /sup 2/H(d,n)/sup 3/He and /sup 3/H(p,n)/sup 3/He reactions. The gamma radiation was detected by a large NaI(Tl) scintillator using time-of-flight techniques. The neutron flux was measured by means of a proton-recoil telescope using the n-p scattering cross section. The differential gamma-ray production cross sections were measured at 90/sup 0/. In addition, the angular distribution for the 6.13-MeV gamma ray was determined at one neutron energy. The results for oxygen, which show pronounced structure of the cross section for the 6.13-MeV gamma ray over the whole energy region, are in disagreement with current data files, whereas the results for carbon aremore » in agreement with a number of recent investigations of the /sup 12/C(n,n'..gamma..)/sup 12/C and /sup 12/C(n,n')/sup 12/C reactions.« less

Rabbit tibial nerves were subjected to direct, acute graded compression by means of an inflatable compression chamber. The acute and long term effects of 50, 200 and 400 mmHg applied for two hours on nerve function and nerve fibre structure were investigated. A pressure of 50 mmHg applied for two hours induced only minimal or no acute deterioration of maximal conduction velocity and nerve fibre structure. Conduction velocity was gradually reduced during compression at 200-400 mmHg pressure for two hours and in those cases the recovery of nerve conduction after pressure release was incomplete. Ultrastructural analysis revealed pronounced, early nerve fibre damage in these nerves. Three weeks after compression, nerves compressed at 50 mmHg for two hours had normal afferent and motor conduction velocity, although there were morphological signs of slight nerve fibre damage. Nerves compressed at 200 mmHg for two hours exhibited reduction of conduction velocity only at the level of compression, in contrast to the nerves compressed at 400 mmHg for two hours in which conduction velocity was reduced both at the level of compression and distal to the compressed segment. Morphologically, the nerves compressed at 200-400 mmHg for two hours showed varying degrees of demyelination and axonal degeneration three weeks after compression.

Three cases of non-progressive, sensory neuropathy with dysautonomia are presented. Light and electron microscopy on whole sural nerve biopsies revealed an almost total lack of myelinated nerve fibres. The total fibre count was also reduced as was the total number of Schwann cell nuclei. No degenerative phenomena were seen within the nerve fibres. The aberrations are probably caused by a maldevelopment of the neural crest implying a stunted proliferation and growth of sensory and autonomous neurones as well as a reduced proliferation of Schwann cells. Since the morphology and clinical features differ from that in other cases of sensory neuropathy with dysautonomia the three present cases are considered to represent a new type of the disease.

Local anesthetics are designed for application in or close to nerve tissue. In spite of their wide clinical use, surprisingly few investigations deal with the neural toxicity of modern local anesthetics. In this experimental study, the effects were investigated of intrafascicular or topical application of the long-acting local anesthetic bupivacaine on the rabbit sciatic nerve. Axonal degeneration was histologically evaluated and a fluorescence-microscopic technique used to detect lesions in the blood-nerve barrier. Topical application of bupivacaine in clinically recommended concentrations around the nerve caused no detectable nerve injury, while intrafascicular injections caused considerable axonal degeneration and damaged the blood nerve barrier. Axonal degeneration was the same after injection of physiologic saline solution and bupivacaine 0.5%, but it increased with increasing bupivacaine concentration and especially with the addition of adrenaline. On the other hand, the acute effects of intrafascicular injection, as visualized in the barrier experiments, changed little with the addition of adrenaline, indicating that it is the injection trauma itself which is deleterious. It is concluded that intraneural injections should be avoided and that plain bupivacaine solutions should be routinely used.

Arteries from spontaneously hypertensive rats (SHR) and Wistar Kyoto controls (WKY) were investigated. Specified cross-sections were collected from the extra- and intracranial parts of the internal carotid arteries, from the carotid segment situated in the skull bone and from intra- and extracranial segments of the vertebral arteries. The internal radius, media thickness and ratio between media thickness and internal radius (m/r ratio) were calculated for a standardized condition implying a smooth and circular internal elastic membrane. The m/r ratio was significantly increased in all arterial segments of 7-month-old SHR compared to WKY. This was caused mainly by decrease of radius in the vertebral arteries and in the intracranial carotid segment. In the extracranial and intraosseous carotid segments the increase of m/r ratio in SHR was mainly due to an increase of media. Whereas the radius was the same in the intra-osseous and intracranial carotid segments of WKY, the m/r ratio was greater intracranially. The results suggest that the physical properties of supporting tissues may influence the normal arterial development and modify the influence of an increased arterial pressure on the vessel wall.

Spontaneously hypertensive rats (SHR) and stroke-prone spontaneously hypertensive rats (SHRSP) were treated with a combination of a beta 1-blocker (Metoprolol) and a calcium antagonist (Felodipine) from 1 to 4 or from 4 to 6 months of age. Cross sections of plastic embedded basal cerebral arteries were measured with a digitizer. The ratio between media thickness and luminal radius (m/r ratio) could then be calculated for a standardized condition assuming a smooth, circular internal elastic membrane. The treatment caused a significant decrease of the m/r ratio in various basal cerebral arteries of young and adult SHR and SHRSP, i.e. the therapy may prevent as well as reverse hypertensive structural changes. Unilateral superior cervical ganglionectomy or preganglionic denervation did not affect the structure of basal cerebral arteries in young SHRSP or normotensive controls. A slight decrease of m/r ratio was indicated in the smallest pial arteries on the sympathectomized side, but the results did not allow any conclusion as to differences in effect between preganglionic denervation and ganglionectomy.

The effect of metoprolol, a beta 1-blocker, on atherogenesis was evaluated in rabbits fed a diet supplemented with 0.25% cholesterol and 3% coconut oil for 21 weeks. After 7 weeks on the diet, the rabbits were randomly divided into treated (n = 22) and untreated (n = 22) groups. Treated animals received metoprolol subcutaneously by an osmotic pump for 14 weeks, resulting in a plasma level of 774 +/- 69 nM during the investigation. Plasma concentrations of cholesterol, triglycerides, and phospholipids did not differ between the two groups. Nor were there any significant differences between the two groups in plasma concentrations of apolipoprotein A-I, apolipoprotein B, apolipoprotein C-III, and apolipoprotein E measured by electroimmunoassay. At the end of the study, the aortas were cut into three portions and the extent of atherosclerosis was determined by morphometry. The group that had received metoprolol had significantly (p less than 0.015) less atherosclerosis in the aorta (ascending plus arch 37.8 +/- 6.8%, thoracic 32.9 +/- 6.1%, abdominal 19.8 +/- 6.1% of total intimal area; mean +/- SEM) than the controls (ascending plus arch 54.9 +/- 7.1%, thoracic 48.0 +/- 6.2%, abdominal 25.9 +/- 5.5%).

Following an initiative by the NEA Committee on Reactor Physics (NEACRP) at their meeting in September 1980, the NEA Steering Committee approved the setting up of a Joint European-Japanese Programme on Neutron Data Evaluations. A Scientific Coordinating Group was formed to determine the content of the Joint Evaluated File (JEF) and to co-ordinate work in national research organisations associated with the periodic revisions to this library. The NEA Data Bank is taking care of the secretariat functions of the Programme and will also, among other things, assemble and maintain the Joint Evaluated File (JEF). The plans are to release a first version of the file at the end of 1982, followed by a period of re-evaluations and benchmark testing, which will lead to the release of a second version at the end of 1985. The availability of the file will be restricted to the member countries of the NEA Data Bank.