Airborne concentrations and chemical considerations of radioactive ruthenium from an undeclared major nuclear release in 2017.

In October 2017, most European countries reported unique atmospheric detections of aerosol-bound radioruthenium (106Ru). The range of concentrations varied from some tenths of µBq·m-3 to more than 150 mBq·m-3 The widespread detection at such considerable (yet innocuous) levels suggested a considerable release. To compare activity reports of airborne 106Ru with different sampling periods, concentrations were reconstructed based on the most probable plume presence duration at each location. Based on airborne concentration spreading and chemical considerations, it is possible to assume that the release occurred in the Southern Urals region (Russian Federation). The 106Ru age was estimated to be about 2 years. It exhibited highly soluble and less soluble fractions in aqueous media, high radiopurity (lack of concomitant radionuclides), and volatility between 700 and 1,000 °C, thus suggesting a release at an advanced stage in the reprocessing of nuclear fuel. The amount and isotopic characteristics of the radioruthenium release may indicate a context with the production of a large 144Ce source for a neutrino experiment.

Potential Source Apportionment and Meteorological Conditions Involved in Airborne 131I Detections in January/February 2017 in Europe.

Traces of particulate radioactive iodine (131I) were detected in the European atmosphere in January/February 2017. Concentrations of this nuclear fission product were very low, ranging 0.1 to 10 µBq m-3 except at one location in western Russia where they reached up to several mBq m-3. Detections have been reported continuously over an 8-week period by about 30 monitoring stations. We examine possible emission source apportionments and rank them considering their expected contribution in terms of orders of magnitude from typical routine releases: radiopharmaceutical production units > sewage sludge incinerators > nuclear power plants > spontaneous fission of uranium in soil. Inverse modeling simulations indicate that the widespread detections of 131I resulted from the combination of multiple source releases. Among them, those from radiopharmaceutical production units remain the most likely. One of them is located in Western Russia and its estimated source term complies with authorized limits. Other existing sources related to 131I use (medical purposes or sewage sludge incineration) can explain detections on a rather local scale. As an enhancing factor, the prevailing wintertime meteorological situations marked by strong temperature inversions led to poor dispersion conditions that resulted in higher concentrations exceeding usual detection limits in use within the informal Ring of Five (Ro5) monitoring network.

Estonian waterworks treatment plants: clearance of residues, discharge of effluents and efficiency of removal of radium from drinking water.

Considerable levels of radium were detected in a certain fraction of the Estonian drinking water supply network. Some of these waterworks have treatment systems for the removal of (mainly) iron and manganese from drinking water. Three of these waterworks and another one equipped with a radium removal pilot plant were examined, and a specific study was conducted in order to assess the environmental compatibility of effluents and residues produced in the plants. (226)Ra and (228)Ra activity concentrations were analysed in both liquid (backwash water) and solid (sand filter and sediment) materials to evaluate their compliance, from the radiological point of view, with current Estonian legislation and international technical documents that propose reference levels for radium in effluents and residues. Also with regard to water treatment by-products, a preliminary analysis was done of possible consequences of the transposition of the European Basic Safety Standards Draft into Estonian law. Radium removal efficiency was also tested in the same plants. Iron and manganese treatment plants turned out to be scarcely effective, whilst the radium mitigation pilot plant showed a promising performance.

Intercomparison exercises as a tool for improving the quality of radiochemical measurements in drinking waters - Radon-222 determinations.

The results of a European intercomparison on 222Rn in water were analyzed to evaluate the performances of standard and non-standard methods. Then, results obtained with a specific LSC method (ISO 13164-4) based on two-phase liquid scintillation counting which has been employed by a considerable number of participants were examined in detail. This ISO LSC method was proved to be accurate, reliable and its reproducibility has been also sufficient. The intercomparison could be used as a collaborative study and the analysis of its results allowed to estimate the method reproducibility.

Radium isotopes in Estonian groundwater: measurements, analytical correlations, population dose and a proposal for a monitoring strategy.

In some areas of Estonia, groundwater contains a significant number of natural radionuclides, especially radium isotopes, which may cause radiation protection concern depending on the geological structure of the aquifer. Indeed, the parametric value of 0.1 mSv y⁻¹ for the total indicative dose established by European Directive 98/83/EC, adopted as a limit value in Estonian national legislation, is often exceeded. A Twinning Project between Estonia and Italy was carried out within the framework of the Estonian Transition Facility Programme, sponsored by the European Union. Its aims were to assess the radiological situation of Estonian groundwater and related health consequences. The first step was a study of Estonian aqueducts and the population served by them, and a thorough analysis of the radiological database for drinking water, from which the relevant effective doses for the population were obtained. Particular attention was devoted to doses to children and infants. Correlations between the chemical parameters were investigated, in order to suggest the best possible analytical approach. Lastly, a monitoring strategy, i.e. sampling points and sampling frequencies, was proposed.

Identification of two mutations in cis in the SCN1A gene in a family showing genetic epilepsy with febrile seizures plus (GEFS+) and idiopathic generalized epilepsy (IGE).

Mutations in the SCN1A gene causing either loss or gain of function have been frequently found in patients affected by genetic epilepsy with febrile seizures plus (GEFS+) or Dravet syndrome (also named severe myoclonic epilepsy in infancy SMEI). By mutation screening of the SCN1A gene, we identified for the first time a case of two missense mutations in cis (p.[Arg1525Gln;Thr297Ile]) in all affected individuals of an Italian family showing GEFS+ and idiopathic generalized epilepsy (IGE). The p.Arg1525Gln mutation was not previously reported yet and was predicted to be pathological by prediction tools, whereas the p.Thr297Ile was already identified in patients showing SMEI. Functional studies revealed that the Nav1.1 channels harboring both mutations were characterized by a significant shift in the activation curve towards more positive potentials. Our data demonstrate that the p.Arg1525Gln represents a novel mutation in the SCN1A gene altering the channel properties in the co-presence of the p.Thr297Ile.

The evaluation of uncertainty in low-level LSC measurements of water samples.

The uncertainty in measurements of gross alpha and beta activities in water samples by liquid scintillation counting with alpha/beta discrimination has been evaluated considering the problems typical of low-level measurements of environmental samples. The use of a pulse shape analysis device to discriminate alpha and beta events introduces a correlation between some of the input quantities, and it has to be considered. Main contributors to total uncertainty have been assessed by specifically designed experimental tests. Results have been fully examined and discussed.

Clivus osteomyelitis secondary to Enterococcus faecium infection in a 6-year-old girl.

A 6-year-old girl was diagnosed to suffer from clivus osteomyelitis secondary to Enterococcus faecium infection. On the basis of the magnetic resonance image, the abscess was drained via the posterior wall of the pharyngeal tract immediately. Subsequent antibiotic therapy allowed rapid improvement and long-term healing of the osteomyelitic process without any side effect. Osteomyelitis or abscess of the clivus is very rare in adult patients and extremely rare in children. Some etiopathogenetic hypotheses are discussed in this case.

Validation of a method for measuring (226)Ra in drinking waters by LSC.

A simple method for measuring (226)Ra in drinking waters has been validated and validation parameters are provided. It is based on the measurement by LSC of (222)Rn, at equilibrium with (226)Ra, which is absorbed into a water immiscible scintillation cocktail (Ultima Gold F) inside the counting vial. The validated application field ranges between the detection limit (2·10(-3) Bq/kg) to 150 Bq/kg. The method has proven to be reliable, effective and suitable for wide-range measuring campaigns. A summary of results obtained in recent years is also given.

A multicenter study on insulin-like growth factor-I serum levels in children with chronic inflammatory diseases.

OBJECTIVE: To study insulin-like factor-I (IGF-I) levels in children and adolescents with connective tissue diseases (CTDs), compare them with values obtained in normal controls, and correlate them with age, sex, steroid treatment, and inflammatory parameters. METHODS: A multicenter, cross-sectional study was performed in 3 Italian pediatric rheumatology centers. The subjects studied comprised 117 patients with juvenile arthritis (53 systemic, 25 pauciarticular and 17 polyarticular) and other CTDs (22), and 78 children without inflammatory conditions. IGF-I levels were measured by radioimmunoassay after acid-ethanol extraction. RESULTS: Mean IGF-I serum levels were 167.6 ng/ml (+/- 132.5) in patients and 214.4 (+/- 142.8) in controls. A significant correlation was found between IGF-I levels and age in the controls (P = 0.001), but not in the patients. Covariance analysis with age as the covariate showed significantly lower IGF-I levels in the patient group (P = 0.001). No significant correlation was found between IGF-I levels and the total quantity of steroid taken. Multiple regression analysis showed that IGF-I levels were inversely correlated with the ESR (P = 0.0001) and positively correlated with age (P = 0.0002) and sex (P = 0.021) in the patient group. CONCLUSION: IGF-I serum levels are decreased in patients with CTDs; inflammation could play a major role.

The monitoring of tap waters in Milano: planning, methods and results.

The radiometric monitoring of tap waters has been carried out, in Northern Italy, since late the 1980s as part of the activities headed by the National Surveillance Network on environmental radioactivity. Measurements were accomplished by gamma spectrometry over large samples of water that were drained in a single place and concentrated by ion exchange resin treatment. More recently a regular, periodical monitoring was started using liquid scintillation measurements. In this manner, gross alpha and beta activities, uranium, (226)Ra and (222)Rn concentrations are determined at present. Nevertheless, a single-point sampling may be not fully representative of the whole Milano aqueduct, significantly extended and fed by a number of wells. As a control, an extended monitoring of the aqueduct was planned and variations of water radioactivity in different Milano areas have been assessed.

Uncertainty evaluation of radon measurements with LR115 detector and spark counter.

To evaluate the uncertainties for nuclear track detectors used in radon measurements, a full understanding is required of the physical phenomena involved and the behaviour of the instruments utilised in the measuring process. As it concerns the LR115 nuclear track detector, an overall evaluation of uncertainty was given. It was assessed taking into account different contributions and determining their relative weights. Since such detectors are often read by a spark counter device, a model to describe its behaviour was developed and a saturation factor was estimated. Its expression and its associated uncertainty are given. Hence, it has been possible to draw a calibration curve, in which all the uncertainty sources have been considered.

Determination of uranium isotopes in food and environmental samples by different techniques: a comparison.

The uranium concentration in 59 samples of bottled and tap water, mainly from northern Italy, was measured by different techniques. Results obtained by inductively coupled plasma mass spectrometry (ICP-MS), semiconductor alpha spectrometry and low level liquid scintillation counting with alpha/beta discrimination (LSC) have been compared. High resolution gamma spectrometry and semiconductor alpha spectrometry have been used to analyse uranium in a variety of organic and inorganic samples. Isotopic secular equilibrium in the 238U series may be lacking or hidden by auto-absorption phenomena, so caution should be used in evaluating gamma spectrometry data. Alpha spectrometry has also been used to ascertain the possible pollution from depleted uranium in the environment.

[Evaluation of the nutritional status and growth of children with chronic HBsAg-positive hepatitis]. / Valutazione dello stato nutrizionale e dell'accrescimento in bambini con epatite cronica HBsAg positiva.

Chronic hepatitis in children seems to have a better prognosis than in adults, with a discrete trend to spontaneous remission. Actually biopsy and liver enzymes are mandatory for a correct follow up of the disease, while few authors are interested in the assessment of growth and nutritional status. We think that an auxological approach is one of the main points in every chronic disease of the pediatric age, chiefly when some anabolic step might be affected. Our first result in a cross sectional study of growth and nutritional status in a selected group of untreated HBsAg+ chronic hepatitis children are as follows. According to enzyme values in the range of 16-171 UI/1 SGOT all patients are growing very well with a good-discrete nutritional status. Though anthropometric proteic nutritional status (muscle circumference) was in the range of normal distribution, we were able to show a moderate correlation between SGOT and muscle circumference (r = -0,50). Our impression is that some patients with a worse hepatic damage can be exposed at risk of proteic malnutrition which is a negative element in the prognosis of every chronic disease. Anthropometric auxology can detect degrees of proteic malnutrition, and can follow its development. So we recommend routine auxological assessment in all pediatric patients with chronic hepatitis.

["Follow-up milk": general principles and evaluation of the nutritional status of 100 subjects fed a liquid transitional formula]. / Latti di seguito: principi generali e valutazione dello stato nutrizionale in 100 soggetti alimentati con una formula di transizione liquida.

Many authors have pointed that precocious weaning expose infants to serious risks as hypernutrition, obesity, adverse reactions to foods, hypernatremia, dental caries, emotional problems as anorexia or bulimia, so that actually weaning is delayed after 6th month of age. Going on with the "adapted" formula is a relative nonsense because "adapted" milks have low protein and calcium contents so that they are not adequate to cover estimated and advisable intakes of 4-6 month baby unless feeding unusual higher volumes. On the other side "fresh milk" can not be considered a nutritional "chance", owing its low values of EFA, iron, vitamins, getting worse when fresh milk is diluted. So, recently was born a new milk formula "the follow up milk", on covering nutritional requirement for infants after 4 months of age. Someone is still critical about a follow up milk, also if ESPGAN in 1981 has confirmed its value in the infant feeding. Our work dealed on physical and biochemical nutritional assessment of 100 infants fed a new "liquid follow up formula" (Transilat). Nutritional assessment was performed with the following parameters: daily changes in weight according Fomon standards, plasmatic iron, cholesterol, transferrin, calcium, total proteins, hemoglobin concentration; all data are related to literature values for age. Results show that infants fed (Transilat) are growing well; nutritional data from biochemical point of view discovered any form of minimal or sporadic malnutrition. The follow up milk is a good nutritional "chance" after 4th month of age, instead of fresh cow milk; some infant with clinical problem needing a delayed introduction of cow milk can benefit of follow up milk also in older ages.

[Average number of bleeding episodes in hemophilic children of different ages in "on demand" therapy]. / Incidenza media di episodi emorragici nelle varie età del bambino emofilico in terapia "on demand".

We have studied the number of important bleeds in our patients with respect to the age group in order to find criteria for putting the hemophilic child on an effective continuous prophylactic regime as early as possible. The bleeding sites are shown in the Tables. 48 children with severe hemophilia were studied. We observed an increase in bleeds with an increase in age. This increase in bleeds and their recurrence in preferential sites in a high percentage (48%) of these children indicate a higher exposure to trauma in part, and a gradual incipience of an arthro-miopathy as well. To avoid this occurring we propose beginning prophylactic treatment as early as possible.

[Home care for the hemophilic child: experience of 4 training courses for the parents]. / Trattamento domiciliare del bambino emofilico: esperienza di quattro corsi di addestramento per i genitori.

Much has already been written about training courses for home care of the hemophiliac held both in Italy and abroad. The advantages of home care are unquestionable. On one hand, the disorder becomes less hospital-dependent, and on the ofter, trough greater partecipation both the infusor and family become more responsible and competent on dealing with it. Hemophilia is a disease which puts the heaving burden on the pediatrics years. A specialized pediatric center is undoubtedly ideal for providing "global" assistance to the hemophilic child and his family so as to prevent any physical consequence of the disorder. There is a natural setting for training courses for home care for parents where they receive proper instruction with respect to problems that are often solely pediatric. The purpose of the course is not just to teach how to reconstitute and infuse freeze-dried concentrate, a practice which is, in itself, fairly easy, but also to give the parents through explanation, and a chance to meet with other parents to exchange ideas and experience.

[Hemophilia: evaluation of the nutritional status and growth in childhood]. / Emofilia: valutazione dello stato nutrizionale e della crescita in età pediatrica.

Haemophilia is a bleeding disorder characterized by decreased activity of the circulating antihemophilic factor, with different degrees of severity. The prognosis of the disease for an useful normal life is good, providing an adeguate follow-up. We think that the auxological determinants of growth and nutritional status must enter as points of a multicentre follow-up sheet of haemophilia in the pediatric age. We have tried such an approach in 13 patients; preliminary results have shown that all patients are growing well with some degrees of discrete malnutrition (arm circumference less than -1.6 SD). From costitutional point of view, haemophilic boys show a discrete "lean" body pattern (W/H2 less than or equal to O SD(with their trunk longer than legs. Data from other studies are wellcome for confirming our first impressions.

[Idiopathic pulmonary hemosiderosis: report of a case with a favorable response to cyclophophamide therapy]. / Emosiderosi polmonare idiopatica: descrizione di un caso clinico con favorevole risposta alla terapia con ciclofosfamide.

A 13-years old boy who presented a severe degree of iron-deficiency anemia and diffuse parenchimal infiltrates on the chest roentgenogram is reported. The clinical picture and the presence of hemosiderin laden macrophages in bronchial washing suggest Idiopathic Pulmonary Hemosiderosis (I.P.H.): open lung biopsy confirmed the diagnosis. Immunofluorescence studies showed no deposition of IgG, IgA, IgM and B1C in the lung. The most striking abnormality observed at electron microscopy was hemosiderin deposition in the alveolar-capillary basement membrane. One year after cyclophosphamide therapy was both in complete hematologic and pulmonary remission.

[Reference values of urinary delta-aminolevulinic acid in children]. / Valori di riferimento dell'acido delta-aminolevulinico urinario in età pediatrica.

Delta-aminolaevulinic acid is well known to be an intermediate in the synthesis of haeme and small amounts are normally excreted in urine (ALA-U). An increased excretion of this substance occurs in porphyria and in lead poisoning. The urine specimens of 670 children (362 boys and 308 girls), aging from less than one to fifteen years and who had no evidence of abnormal exposure to lead, have been collected in order to find out the range of normal values of ALA-U expressed in mg/l for the different age and sex group. Our data can be usefull, together with zincprotoporphyrin (ZPP), for screening of anemias and for monitoring lead exposure.