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EPO treatment rapidly corrects anemia in patients with end-stage renal failure treated with hemodialysis, as long as sufficient iron is available. Absolute and relative (to demand) iron deficiency blunts the erythropoietic response and parenteral iron is frequently required during the course of therapy to restore EPO efficacy. Since the optimum time course of iron administration to restore EPO response in the short term is unknown, we compared three protocols of i.v. iron dextran administration in apparent functionally iron-deficient HD patients on oral iron therapy (hemoglobin10.0 g/dl plus ferritin100 micrograms/l and/or transferrin saturation20%). Intravenous iron (Imferon; Fisons Pty Ltd.) was given either as a single 600 mg dose (n = 15, Group I) or in divided doses of 100 mg administered on 6 successive dialyses (n = 14, Group II) or weekly for 6 weeks (n = 14, Group III). Response was monitored for 8 weeks. No adverse effects were observed. Collectively, mean hemoglobin increased (p0.01) by 0.4-0.5 g/dl plateauing at 4 weeks (between group comparison, p = 0.92). Mean ferritin concentrations changed with time (p0.01), peaking at 2 weeks in Groups I and II and at 4 weeks in Group III. Mean transferrin saturation levels also increased during the study (p0.001). The between group comparisons for the trends in iron indices were significant (p0.01 and 0.05 respectively). As there were no clinically significant differences in hemoglobin response at 4 weeks, single dose iron infusion would seem to be the most expedient in the short term, however frequent small doses are similarly effective.

The DELPHI Vertex Detector has been upgraded for LEP200 with 2 layers of pixel detectors and 2 layers of ministrips as endcaps, thus covering the polar angular range between 10° and 21° and allowing for standalone pattern recognition. During the 1995/96 shutdown the first 95 modules were installed and the installation was completed in May 97 with all 152 modules. Production yields and failures during the assembly will be presented. The detector operated at a threshold of around 9000 electrons resulting in a number of noisy pixels at the 10 −3 level. After masking these hot pixels in the readout, the remaining number of random hits per event is at the 10 −6 level. The observed resolution of about 100 μm is close to the expectation for binary readout. The track reconstruction efficiency increased by more than 100% in the central part of the forward tracker.

Rett's syndrome progresses in 4 stages: the first signs of the disorder appear after a period of 6 to 7 months, during which development is considered to be normal. This asymptomatic period is apparently an essential criterion of the diagnosis, but some parents have reported some prodromes. In stage II of the disease (before 3 years), signs common with autism dominate the clinical picture and the diagnosis of the latter was often formulated. Our working hypothesis is that the pedopsychiatric analysis of home movies of young girls with Rett's syndrome, taken by the parents before the age of 2, may be able to show early clinical signs. The present study involved examining home movies of children subsequently diagnosed as having Rett's syndrome (n = 9) in comparison to those of autistic (n = 9) and normal (n = 9) children, using semiological evaluation tools (IBSE, BFE). The persons scoring were not advised of the diagnosis. The observations were thus situated before the disorders and/or at the time of their appearance. The study confirms the asymptomatic interval between birth and the first signs of the disease, it defines the mode of onset and shows the disturbance of certain functions such as intent and imitation, more pronounced in Rett's syndrome children between 12 and 18 months. At this age, it also enables Rett's and autistic children to be differentiated on the basis of the different involvement of the "cognition" function and unusual posture, more pronounced in these girls. It does not, however, differentiate Rett's from autism between 6 and 12 months and it is thus not surprising that at this stage the diagnosis of rett's syndrome or autism may be a source of confusion.

In 1996 the DELPHI experiment at LEP has upgraded its silicon tracker. In the forward region, pixel detectors were installed. The pixel commissioning and the first results are reported.

Tuberous sclerosis is often associated with developmental and behavioural disorders including typical or partial autistic syndrome. However, it may be difficult to recognize tuberous sclerosis behind an infantile autism during the early stages of the disease. Therefore, tuberous sclerosis must be regularly looked for on the basis of its major and minor criteria in any cases of infantile autism. The child psychiatrist is preferentially involved in the management of the various aspects of this association, ie, behavioural or character disorders, difficulties in social relationships and communication, mental retardation, feeding disorders, and psychological consequences for the families. The support provided may be complemented by that offered by the Association for Bourneville's tuberous sclerosis.

Natural history of infantile autism goes from its first description to current classifications. Most authors agree upon the perfect character of Kanner's description in 1943. But its situation in the nosography has much developed. The parution of 4th edition of Diagnostic and Statistical Manual of mental disorders (DSM IV) bring us to analyse this evolution and the place of autistic disorders in the pervasive developmental disorders, with this of associated pathologies. The comparison of current classifications (DSM IV, ICD 10, CFTMEA) allows us to do correspondence between each diagnostic category in psychosis or developmental disorders of these classifications. It exists a real concordance between DSM IV and ICD 10. The french classification of child and adolescent mental disorders (CFTMEA) proposes original categories.

To improve tracking in the very forward direction for running at LEP200, the angular acceptance of the DELPHI Vertex detector has been extended from 45° to 11° with respect to the beam axis. Pixel detector crowns cover the region between 25° and 13°. Due to very tight space and material thickness constraints it was necessary to develop new techniques (integrated busses in the detector substrate, high density layout on Kapton, etc.). About 1000 cm 2 of pixels are already installed and working in DELPHI. Techniques, tests and production of these detectors will be described, as well as the main problems encountered during this work.

The status of the pixel detector for the DELPHI Silicon Tracker is presented. The main characteristics of the detector, the readout chip and the assembly are summarized.

In working out a realistic long-term scenario for the operational conditions for the innermost silicon layers, it is usually assumed that the upper safe margin for the reverse-bias voltage is around 200 V. Lifetime of the detectors, and consequently total cost of the tracker are directly related to this value. Using pixel test structures, manufactured by CSEM in the same technology as DELPHI pixel detectors, we performed a study of maximum tolerable bias voltages on nonirradiated detectors, and on detectors which have received different fluences of pions, protons and neutrons. A special test was also done with detectors, continuously irradiated on a testbench with a Sr source, at a rate about 13 krad/h.

Twin studies and familial aggregation studies indicate that genetic factors could play a role in infantile autism. In an earlier study, we identified a possible positive association between autism and a c-Harvey-ras (HRAS) oncogene marker at the 3' end of the coding region. In an attempt to confirm this finding, we studied a larger population, well-characterized clinically and genetically. We report a positive association between autism and two HRAS markers, the 3' marker used in the initial study and an additional marker in exon 1.

A large number of investigation techniques are used to establish the relationships between the clinical and biological data which are necessary for physiopathological analysis in the field of developmental disorders. It therefore seemed necessary to develop a quantified grouping system, based on developmental assessments, which could allow closer matching between clinical evaluations and biological numerical data. Two hundred and two subjects presenting developmental disorders (autistic disorder, pervasive developmental disorder not otherwise specified and mental retardation) were examined. For each child, a quantification of autistic behaviour, intellectual impairment, neurological signs and language and communication disorders was performed. A cluster analysis of these quantified data elicited four subgroups according to the scores obtained in these four different areas. We showed the value of this approach by applying it to one of the studies of monoamines routinely examined in childhood autism--dopamine and HVA, its main urinary derivative. Moreover, this method revealed a subgroup within the total population which was independent of nosographic classification and which had a particular clinical and biochemical profile. Other applications could follow, for example in the fields of neurophysiology, cerebral imaging, molecular biology and genetics.

Epidemiological data and family studies in autism show that there is a genetic susceptibility factor in the aetiology of this syndrome. We carried out an association study in infantile autism. Two markers of the homeogene EN2 involved in cerebellar development were tested in a population of 100 autistic children and in a population of 100 control children. With the MP4 probe showing a PvuII polymorphism, significant differences in the allele frequencies between the two populations were found (chi 2 = 7.99, df = 1, p < 0.01). With the MP5 probe showing an SstI polymorphism, no difference appeared (chi 2 = 1.17, not significant). Several clinical examinations allowed us to characterise the autistic children. Most of them had high scores for autistic behaviour and language disorders but low scores for neurological syndromes. Two children had a significant family history and six children had confirmed syndromes or diseases of genetic origin. Discriminant analysis between clinical and molecular data did not give significant results. These preliminary results must be supported by further analyses of this gene and by studies of its potential involvement in the pathophysiology of the autistic syndrome.

The design of a new silicon tracker detector for the forward region in the DELPHI experiment is presented. It consists of two layers of macropixel and two layers of ministrip detectors in both the forward directions. The motivations and the requirements for this detector will be shown together with test beam results.

The aim of this study was to explore two markers of the Insuline (INS) gene and one marker of the Insuline-Like Growth Factor 2 (IGF2) gene by Polymerase Chain Reaction (PCR) in larger samples of autistic and control children

Many mentally retarded autistic children can understand the concept of object permanence, but, in comparison to developmental-age matched normal children, the behavioral strategies they employ in carrying out the Casati-Lezine Object Permanence Test are deficient and lead to failure. These deficiencies appear unrelated to interference of stereotypic or other bizarre behavior in task performance. Similar problem-solving deficiencies can be found in mentally retarded children who are not autistic, suggesting that the deficiencies themselves are less related to the social-communication deficits of autistic children, but more to the general problem-solving difficulties found in children with a lower developmental quotient. Nevertheless, the qualitative analysis of results shows a tendency in autistic children, despite their better developmental level, to use less coordinated and regular sequences to solve the task than normal or mentally retarded children.

A new approach to monolithic pixel detectors, based on silicon on insulator (SOI) wafers with high resistivity substrate, is being pursued by the CERN RD19 collaboration. The fabrication methods and the results of the electrical evaluation of the SOI-MOSFET devices and of the detector structures fabricated in the bulk are reported. The leakage current of the high-resistivity PIN-diodes is kept of the order of 5 to 10 nA/cm/sup 2/. The SOI preparation processes employed-SIMOX (separation by implantation of oxygen) and ZMR (zone melting recrystallization)-produce working electronic circuits, and appear to be compatible with the fabrication of detectors of suitable quality. >

A new type of light detector, the Proximity Focused Hybrid Photodiode Detector, has been constructed and its properties have been studied. The main features of the new device are a gain of up to a few thousand varying linearly with the applied high voltage, a large dynamic range, a low sensitivity to magnetic field, a power consumption close to zero and a multipixel capability.

Specific features of the OMEGA-ION pixel detector are the adjustable delay with external trigger capability and the detector leakage current compensation. A row of pixels can be used for testing the electrical performance of the amplifier and comparator circuits. Detailed results of these electrical tests are presented. A telescope made of three OMEGA-ION hybrid silicon pixel detectors has been successfully tested in the heavy-ion experiment WA94. Each plane consisted of a single detector with 1006 active pixels (500 μm×75 μm), each one being bump-bonded to the readout chip, and arranged in 16 columns and 63 rows respectively. With a sensitive area as small as 8000 × 4725 μm 2 several million events with at least one track originating from the sulphur-sulphur interaction have been recorded in a few hours. Results on target reconstruction, tracking accuracy and efficiency are presented.

The analysis of 11 home movies taken by parents before the recognition of autistic or atypical disorders of their own child has confirmed the major value of this method for describing early pathological signs. Symptomatology analysis has revealed anomalies of eye contact, a deficiency and variability of emotional expression, a defect of attention and initiation of communication, as well as motor abnormalities. The comparison of the frequency of abnormal behaviour, assessed with a rating scale among three groups of children (autistic, pervasive developmental disorders and normal) revealed behavioural differences as a function of early age and diagnosis, which concern not only social and communicative behaviours, but those of emotion and attention as well. The limits and interest of this methodological approach are discussed and the possibilities of subsequently using these documents in a more complete method, such as blind examination and scoring by uninformed investigators, are suggested.

An asynchronous version of a binary pixel readabout circuit has been implemented in an array with 16 columns at 500 mu m pitch and 63 rows at 75 mu m pitch. This readabout chip has been bonded with solder bumps to a silicon detector with matching pixel elements. Event information in a pixel can be strobed into a local memory by a trigger signal and subsequently read out. Without a strobe the information is continuously cleared. The complete hybrid detector has been successfully tested with ionizing particles from a radioactive source. Three such devices have been put in the CERN heavy-ion experiment WA94 in the Omega spectrometer, where they recorded particle tracks from high-multiplicity /sup 32/S interactions. Preliminary data indicate a noise of approximately 60 e/sup -/ and a threshold spread of approximately 500 e/sup -/. The timing characteristics are adequate for a fixed-target experiment. >

In the framework of the CERN detector R&D effort in view of future high luminosity colliders, several pixel detector prototypes, both hybrid and monolithic, are being developed. A hybrid matrix of 16 × 64 pixels with a new readout cell is described here, and it will be first used for a test in a heavy-ion experiment. Preliminary results from the application of SOI processes on high resistivity silicon are also presented.

By operating the parallel-plate imaging chamber in a nonproportional regime it was possible to increase the light yield from the gas avalanches by a factor in excess of 10 4 over the proportional operation. At this higher gain, the avalanches can easily be seen with the naked eye in a moderately lit room and recorded with CCD cameras without any image intensification. The device is continuously operating and is free of sparking. The main limitations are the existence of a detection threshold that is dependent on the primary charge density, and the large amount of current flowing in the high-gain gap, leading to ageing.

The Behavioral Summarized Evaluation (BSE), is a 20-item paper- and-pencil rating scale specifically designed for the measurement of behavioral parameters which could be related to biological data in autistic children involved in educational programs, neurophysiological studies, and therapeutic trials. The development of the scale, the validity, and reliability studies are presented in this paper. The results suggest that the BSE is an acceptable tool for the assessment of autistic behaviors, easy to handle, and accessible to both professionals and paraprofessionals of the medico-educative staff. It is a useful addition to the bioclinical researcher's evaluation battery for bioclinical and therapeutic studies. However, more work is suggested to further investigate the psychometric properties of this behavior assessment instrument.

Clinical studies of early symptoms are among the most significant advances achieved during recent years in the field of autism. The first descriptions of early symptoms were retrospective and lacked precision. Autistic children are now being seen increasingly early and new insight is being gained into the initial manifestations and differences in clinical patterns. Similarly, differential diagnosis problems (borderline forms) are changing as clinicians evaluate children at younger ages. Although a definitive diagnosis is neither possible nor even desirable before the age of 18 to 24 months, detailed clinical data should be collected early. This data forms the basis for differential diagnosis and will be needed later for differentiating various clinical patterns of developmental disorders, establishing a prognosis, and monitoring therapeutic effects. In prospective longitudinal studies of autistic children, comparison of recent observations with detailed early data is essential. Early initial evaluation includes videotape recordings, assessment of cognitive functions and communication skills, and use of a scale for autistic symptoms. Using the results of evaluations of very young children, a specific semiology (communication disorders) can be developed. Several symptoms probably denote neurophysiologic and/or perceptive disorders. Primary symptoms are now being better distinguished from secondary anomalies (behavior disorders) that may be avoidable or treatable.

After reiterating the reliability objectives of such equipment in a special link such as EMOS and the respective allowances made for transmit and receive components, and a overview of the consolidated reliability data obtained on these components, the emphasis will be put on the resulting selection procedures that have been chosen after an optimisation and validation phase. The practical application of these procedures in supplies for the EMOS link shall be described in particular detail.

On a longtemps considere que l'autisme de l'enfant, caracterise par un isolement, des difficultes de communication et des reactions bizarres a l'environnement, pouvait etre du a des erreurs conscientes ou inconscientes de la mere. En fait, des enquetes epidemiologiques, des recherches neurobiologiques portant notamment sur l'association de syndromes autistiques a des maladies connues (genetiques et/ou metaboliques), et des etudes cliniques sur les signes tres precoces suggerent que l'autisme serait lie a un trouble du developpement du systeme nerveux. Les moyens actuels d'exploration (imagerie cerebrale, etude du metabolisme des neurotransmetteurs, etudes genetiques) ont permis de mettre en evidence des anomalies tres diverses. Ces resultats preliminaires n'ont pour le moment pas de consequence therapeutique ; en revanche, les donnees portant sur la physiologie cerebrale permettent d'orienter les « therapeutiques d'echange et de developpement», psychotherapies educatives qui contribuent a l'amelioration de ces enfants.

We have made a quantitative systematic study of the light emitted through the electron avalanche process in low-pressure gases. The mixtures under investigation were argon/(CH4 or C2H6)/(TMAE or TEA). For a given avalanche charge gain, we measured comparable light yields for mixtures containing TEA and TMAE; typical values are of 0.1–1 photons per electrons in the avalanche. We found a linear increase of the light intensity with the amplification gap width. Light production by electrons drifting in these gas mixtures without charge multiplication was also demonstrated.

A novel two-dimensional readout scheme for gas detectors is presented that uses small metal pads with 2.54-mm pitch as an anode. The pads are read out via 128-channel VLSI low-noise preamplifier/multiplexer chips. These chips are mounted on 2.8-cm*2.8-cm modules, which are directly plugged onto the detector backplane, delay-chained with jumpers, and read out sequentially. The readout has been successfully tested with a low-pressure, two-step, TMAE-filled, ultraviolet-sensitive ring imaging Cerenkov counter. A single-electron efficiency of >90% was observed at moderate chamber gains ( >

Rhizobium meliloti Be 151, an effective strain of Rhizobium for Medicago sativa, was found to be a facultative halotolerant bacterium. The presence of NaCl at concentrations as high as 75 mM did not inhibit the bacterial growth. The strain tolerated salt concentrations up to 600 mM although a 40% inhibition in growth was observed at 400 mM NaCl. Addition of 10 mM glycine betaine helped the strain to overcome the diminution in growth in the presence of sodium chloride. The extent of N-methylation of glycine is important in producing the anti-stress effect, since the N-trimethylated compound was more effective than the N-dimethylated which again was superior to the N-monomethylated derivative of glycine. On the contrary, glycine itself acted as a potent inhibitor of R. meliloti growth. Some kind of protection against the deleterious effects of saline conditions was also noted with some alicyclic betaines such as β-homobetaine and γ-butyrobetaine as well as with cyclic betaines like stachydrin and homostachydrin. Glycine betaine and stachydrin which exhibited the most pronounced anti-stress effect were utilized by the bacteria as substrates in the absence of any conventional carbon source. The significance of these findings in relation to the Medicago sativa-Rhizobium meliloti symbiosis is discussed.

Photons emitted by avalanches in gases can be detected with an image intensifier coupled to a solid-state camera. Some vapors enhance the emission at wavelengths close to the visible. Progress made in using this technique to image charged particles and Cherenkov photons is described. Results are presented for various gas mixtures containing TEA and TMAE. >

A low-pressure multistep, TMAE-filled, UV-sensitive detector has been developed for Cerenkov ring imaging for ultrarelativistic heavy-ion experiments (HELIOS-CERN). A description is given of the detector structure and its basic properties, and the experimental setup and the detector performance in tests with high-energy electrons are presented. Three modes of readout are used: three-wire coordinates and flash analog-to-digital converters; pad readout; and optical recording of the avalanches. Single photons can be localized with an accuracy of sigma approximately=2 mm, mainly due to single-photoelectron diffusion and to chromatic dispersion in the radiator gas. The experimental N/sub 0/ values obtained by the three methods are close to those expected, taking into account the various losses due to the test conditions. >

An analysis of the activation and reactivity properties of various types of FeTi sample under H2 pressure was undertaken together with structural investigations using neutron diffraction. The role of oxide phases in the activation process is discussed. The structures of Fe0.9Mn0.1Ti deuterides were studied in situ in both absorption and desorption cycles. The studies revealed slight differences compared with previously reported results for pure FeTi deuterides. In agreement with earlier findings, the orthorhombic space group P2cm (or Pmc21) was retained for the β phase and the monoclinic space group P2 m was retained for the 7 phase.

SUMMARYAn epidemiological study and the simultaneous evaluation of anti-Hav antibody using radio immunoassay (RIA) and immunoadherence hemagglutination assay (IAHA) was performed during three hepatitis A epidemics in the Tours area (France). Fifty-seven sera from 35 subjects with viral hepatitis type A and 16 sera from nine children who did not develop any clinical signs of hepatitis were studied. The more explosive epidemic occurred in an institution for mentally retarded children (attack rate 68%). The two major outbreaks observed were due to the introduction in the institutions of individuals infected with hepatitis A virus. Two out of three of the index cases had a seafood dinner three to four weeks before onset of jaundice. Sera taken one week after jaundice were always found to be anti-HAV positive by both RIA and IAHA, and sera taken more than three days before the appearance of jaundice were negative by both methods. Sera taken at the peak of the transaminase elevation were anti-HAV positive by RIA but only one out of two were positive by Iaha. The anti-HAV titre by RIA increased from the time of the appearance of jaundice and the highest titres, over 1/20000 were seen only after several months. Observations of subjects in close contact with patients who seroconverted without any manifestation of hepatitis, confirmed the existence of clinically mute infections.

UV photons from a Cherenkov radiator are multiplied in a multistep avalanche chamber operating in a gated mode at low gas pressure (40 Torr). The gas mixture is C 2 H 6 -argon ( 80 20 ) and TMAE at 34°C. Visible light emitted from single photoelectron avalanches is detected by a CCD camera coupled to an image intensifier system. The detector was tested with 5 GeV c electrons, using a CH 4 radiator gas at 1 atm. Cherenkov rings essentially free of particle background and of secondary photon feedback were obtained in this mode of operation with a mean number n ≅ 11.5 ( N 0 ≅ 76 cm −1 ). We present this new method and discuss its performance.

The modified Behavior Observation Scale adapted from Freeman et al. was used to compare normal, retarded, and autistic children with very low developmental ages and to determine the types of behavior that could differentiate these three diagnostic categories of children. Examination of the data revealed that there was much more overlap between autistic and retarded children than between autistic and normal children. However, a behavioral pattern of autism could be delineated and very retarded autistic children could be distinguished from the nonautistic retarded children. The autistic behavioral pattern included subclusters of symptoms that might be interpreted as disturbances of sensory modulation and motility.

The expression of the current fluctuations due to minority carriers trapping within short diodes is established. Experiments carried out in pnp integrated bipolar transistors are consistent with this model.