Targeted screening of succinic semialdehyde dehydrogenase deficiency (SSADHD) employing an enzymatic assay for γ-hydroxybutyric acid (GHB) in biofluids.

Hypothesis: An enzymatic assay for quantification of γ-hydroxybutyric acid (GHB) in biofluids can be employed for targeted screening of succinic semialdehyde dehydrogenase deficiency (SSADHD) in selected populations.

Rationale: We used a two-tiered study approach, in which the first study (proof of concept) examined 7 urine samples derived from patients with SSADHD and 5 controls, and the second study (feasibility study) examined a broader sample population of patients and controls, including plasma.

Objective: Split samples of urine and plasma (anonymized) were evaluated by enzymatic assay, gas chromatography alone (proof of concept) and gas chromatography-mass spectrometry, and the results compared.

A SHORT HISTORY AND CRITICAL REVIEW OF INDIVIDUAL MONITORING.

Soon after the discovery of X-rays and the radioactive element radium harmful radiation effects occurred, mainly in the medical field. Consequently, the radiologists, a new profession at that time, called for a limitation of radiation exposures. First proposals were to limit the exposure rate to prevent the incidence of skin erythema.

30-y follow-up of a Pu/Am inhalation case.

In 1983, a young man inhaled accidentally a large amount of plutonium and americium. This case was carefully followed until 2013. Since no decorporation measures had been taken, the undisturbed metabolism of Pu and Am can be derived from the data.

Radiation protection culture: a global challenge.

The central motto 'Radiation Protection Culture-A Global Challenge' of the fourth European IRPA Congress is discussed on the basis of the IRPA Guiding Principles Establishing a Radiation Protection Culture and the contributions presented in the plenary sessions of the conference.

Individual monitoring at accelerator centres.

The accurate determination of personal dose equivalent requires the proper use of appropriate radiological quantities and units, knowledge of the dose equivalent response of the personal dosemeters used and detailed information on the fluence as well as dose equivalent spectra at the workplaces. This information can then be used to select the appropriate dosemeters, to set up the optimum calibration conditions and to introduce, in case of need, normalisation factors for application in specific radiation fields. High-energy neutrons contribute significantly to the radiation fields around high-energy particle accelerators.

Performance of a PADC personal neutron dosemeter at simulated and real workplace fields of the nuclear industry.

In the framework of the EVIDOS (Evaluation of Individual Dosimetry in Mixed Neutron and Photon Radiation Fields) project, funded by the EC, measurements with PADC personal neutron dosemeters were carried out at several workplace fields of the nuclear industry and at simulated workplace fields. The measured personal neutron dose equivalents of the PADC personal neutron dosemeter are compared with values that were assessed within the EVIDOS project by other partners. The detection limits for different spectra types are given.

Twenty-year follow-up of a Pu/Am inhalation case.

In 1983 a technician inhaled a mixture of Pu/Am aerosols in an accidental situation in the hotlab of Paul Scherrer Institute (PSI). This case is of interest for long-term follow-up since the technician was relatively young (26 y) at the time of intake, no chelating agent was used to alter retention and excretion and the inhaled activity was rather high (approximately 20 kBq of alpha emitters). The results obtained from periodic lung counts, urinary and faecal excretions as well as from some bone and liver measurements up to the year 2003 are presented.

Integration of external and internal dosimetry in Switzerland.

Individual monitoring regulations in Switzerland are based on the ICRP60 recommendations. The annual limit of 20 mSv for the effective dose applies to the sum of external and internal radiation. External radiation is monitored monthly or quarterly with TLD, DIS or CR-39 dosemeters by 10 approved external dosimetry services and reported as Hp(10) and Hp(0.

State-of-the-art dosimetric methods for internal and external exposures: conclusions of a EURADOS action.

In radiation protection dosimetry the quantity of interest is the effective dose (E); the dose limit to an adult worker applies to the sum of the relevant doses from external exposures and the relevant committed effective doses from intakes of radionuclides, during the same period of time. A EURADOS study was carried out to investigate how the results from personal dosemeters for external radiation, from workplace monitoring and from monitoring of internal exposures can be combined into a consistent system of individual monitoring. The integration of dosimetric methods and data for external and internal radiation require the complete characterisation of the occupational exposure present at the workplace, and the availability of adequate equipment and tools for the assessment of effective dose.

Performance of a personal neutron dosemeter based on direct ion storage at workplace fields in the nuclear industry.

In the framework of the EVIDOS project, funded by the EC, measurements were carried out using dosemeters, based on ionisation chambers with direct ion storage (DIS-N), at several workplace fields, namely, at a fuel processing plant, a boiling and a pressurised water reactor, and near transport and storage casks. The measurements and results obtained with the DIS-N in these workplaces, which are representative for the nuclear industry, are described in this study. Different dosemeter configurations of converter and shielding materials were considered.

Progress report of the CR-39 neutron personal monitoring service at PSI.

At the Paul Scherrer Institute a personal neutron dosimetry system based on chemically etched CR-39 detectors and automatic track counting is in routine use since the beginning of 1998. The quality of the CR-39 detectors has always been a crucial aspect to maintain a trustable personal neutron dosimetry system. This paper summarises the 7 y experience in routine use.

Thermoluminescent detectors applied in individual monitoring of radiation workers in Europe--a review based on the EURADOS questionnaire.

Among the activities of EURADOS Working Group 2 formed by experts from several European countries is the harmonisation of individual monitoring as part of radiation protection of occupationally exposed persons. Here, we provide information about thermoluminescent detectors (TLDs) applied by the European dosimetric services and the dosimetric characteristics of dosemeters in which these detectors are applied. Among 91 services from 29 countries which responded to the EURADOS questionnaire, 61 apply dosemeters with TLDs for the determination of personal dose equivalent H(p)(10) for photons and beta radiation, and 16 services use TLDs for neutron albedo dosemeters.

Aspects of harmonisation of individual monitoring for external radiation in Europe: conclusions of a EURADOS action.

Following the publication of the EU Council Directive 96/29, EURADOS coordinated two working groups (WGs) for promoting the process of harmonisation on individual monitoring of occupationally exposed persons in Europe. An overview of the major findings of the second WG is presented. Information on the technical and quality standards and on the accreditation and approval procedures has been compiled.

Individual monitoring for internal exposures in Europe: conclusions of an EURADOS action.

Once the EC Directive 96/29 has been implemented into national regulation across Europe, the coordination of dosimetry laboratories for the monitoring of occupational exposures becomes the principal aim to achieve. Within this framework the European Radiation Dosimetry Group, EURADOS, carried out an Action on 'Harmonisation of Individual Monitoring' (2000-2004) to promote coordination in the field of individual monitoring of occupational exposures throughout Europe. With reference to internal exposures, the main aims were the completion of a catalogue of internal dosimetry services and an inventory of methods and techniques used for individual monitoring at European internal dosimetry facilities.

Workplace monitoring for exposures to radon and to other natural sources in Europe: integration of monitoring for internal and external exposures.

Part of the action of the EURADOS working group (European Radiation Dosimetry Group) on "Harmonisation of Individual Monitoring in Europe" was to investigate how the results from personal dosemeters for external radiation, from monitoring for internal exposure and from workplace monitoring, can be combined into a complete and consistent system of individual monitoring. To facilitate this work, the "EURADOS questionnaire Q3" relating to radon and other natural sources of radiation in the workplace was distributed to relevant institutes across Europe. A total of 24 countries replied to the questionnaire.

Individual monitoring for internal exposure in Europe and the integration of dosimetric data.

The European Radiation Dosimetry Group, EURADOS, established a working group consisting of experts whose aim is to assist in the process of harmonisation of individual monitoring as part of the protection of occupationally exposed workers. A catalogue of facilities and internal dosimetric techniques related to individual monitoring in Europe has been completed as a result of this EURADOS study. A questionnaire was sent in 2002 to services requesting information on various topics including type of exposures, techniques used for direct and indirect measurements including calibration and sensitivity data and the methods employed for the assessment of internal doses.

A catalogue of dosemeters and dosimetric services within Europe--an update.

The catalogue of dosemeters and dosimetric services within the European Union (EU) Member States and Switzerland that was issued by EURADOS in the year 2000 has been updated and extended with information on dosimetric services in the new EU Member States and Bulgaria, Croatia, Romania, Serbia and Montenegro, and Ukraine. The total number of dosimetric services in these European countries is now estimated to be about 200. The present catalogue is based on information collected from 90 European dosimetric services, among which 34 questionnaires from 32 services were obtained over the years 2001-2004 for the first time.

Calibration and testing of a TLD dosemeter for area monitoring.

The response of a TLD-600/TLD-700 area dosemeter has been characterized in neutron fields around the 590 MeV cyclotron ring at the Paul Scherrer Institute (PSI). The dosemeter is based on a cylindrical paraffin moderator with three of each type of TLD chip at the centre, and is intended to use for area monitoring around accelerator facilities. The dosemeter is calibrated in terms of ambient dose equivalent using a non-moderated 252Cf neutron source.

Present status of the personal neutron dosemeter based on direct ion storage.

In this paper the present status of the Direct Ion Storage Neutron (DIS-N) prototype dosemeter (RADOS) is described. The separation of neutron from photon dose equivalent has been improved by adding tin shieldings. The neutron energy response has been changed by additional plastic covers containing 40% B4C in order to reduce the over-response to thermal neutrons.

Harmonisation (legal, dosimetric, quality aspects) of individual monitoring, and integration of monitoring for external and internal exposures (EURADOS Working Group).

The EURADOS Working Group II on 'Harmonisation of individual monitoring' consists of experts from almost all EU Member States and Newly Associated States (NAS), involved in tasks related to the assessment of doses for internal and external radiation. The final objective is to achieve harmonisation in individual monitoring for occupational exposures. Sub-group 2 activities are focused on investigating how the results from personal dosemeters for external radiation and workplace monitoring and from monitoring for internal exposure can be combined into a complete and consistent system of individual monitoring.

Incorporation measurements in Switzerland: the approved internal dosimetry service at PSI.

The Swiss radiation protection regulation requires approval of external and internal dosimetry laboratories. The Swiss Personal Dosimetry Ordinance specifies the terms of approval, the survey methods and the standard interpretation to assess the committed effective dose. Specific information is given for 25 radionuclides concerning metabolism, methods of measurement, survey interval and interpretation of the results.

A legally approved personal dosemeter for photon and beta radiation based on direct ion storage.

The novel DIS-1 dosemeter developed by RADOS is based on ionisation chambers with so-called Direct Ion Storage (DIS). The dosemeter can measure Hp(10) and Hp(0.07) of photon and Hp(0.

Harmonisation and dosimetric quality assurance in individual monitoring for external radiation.

The current situation amongst Member States is that there are widely differing national requirements for dosimetric services and for dosemeter performance. It is clear that with the free movement of workers within the European Union (EU) and the requirements for individual dosimetry given in Council Directive 96/29 EURATOM, a degree of harmonisation of requirements and procedures of EU Member States would be desirable. A EURADOS action group, made up of members from each of the EU Member States plus Switzerland, was set up with the overall objectives of consolidating within the EU the quality of individual monitoring using personal dosemeters and assisting movement towards harmonised procedures.

A prototype personal neutron dosemeter based on an ion chamber and direct ion storage.

Ionisation chambers are sensitive to both neutrons and photons. In order to produce a neutron dosemeter based on an ion chamber a double-chamber system which allows for differential readings has to be built. The system consists of one chamber with high neutron sensitivity (e.