Radioactive waste management: review on clearance levels and acceptance criteria legislation, requirements and standards.

In 2011 the joint research project Metrology for Radioactive Waste Management (MetroRWM)(1) of the European Metrology Research Programme (EMRP) started with a total duration of three years. Within this project, new metrological resources for the assessment of radioactive waste, including their calibration with new reference materials traceable to national standards will be developed. This paper gives a review on national, European and international strategies as basis for science-based metrological requirements in clearance and acceptance of radioactive waste.

Results of an international comparison for the determination of radionuclide activity in bilberry material.

Radioactivity found in wild food products has assumed greater importance when assessing the total exposure of the population. For this reason, IRMM has been developing a reference material for the activity concentration of three radionuclides in bilberry samples. In order to characterise this new material, a CCRI(II) supplementary comparison was organised. The difficulties encountered in this comparison are discussed, in particular the efficiency calibration for volume sources of gamma-ray emitters, and comparison reference values for (137)Cs and (40)K are calculated.

Standardization of 89Sr at the National Office of Measures.

Strontium-89 is an important radiotherapy nuclide in bone palliation. The standardization of 59Sr has been performed at the National Office of Measures (OMH) within the frame of the BIPM CCRI(II)-K2.Sr-89 key comparison. The activity concentration of the 89Sr solution was measured by two different methods. The conventional 4pi beta counting technique was used with two proportional counting measurement systems. The 4pi beta-gamma-coincidence efficiency tracing method was used with a 4pi beta-gamma-coincidence measurement system using 24Na as the tracer radionuclide. The beta efficiencies of each of these counting techniques were nearly 100%. The combined uncertainties of the activity concentration determined by these absolute methods are 0.3%. The general agreement of results between the two methods was 0.5%. The measurement equipment, evaluation methods and results of activity concentration measurements with their uncertainty budgets are reported.

[Non-ketotic hyperglycinemia]. / Non-ketoticus hyperglycinaemia.

Three children with non-ketotic hyperglycinaemia (NKH) is reported. Two patients had typical neonatal form of NKH, one patients had atypical form of NKH. The clinical symptoms laboratory findings and therapeutical approach are discussed. One of the patients with typical neonatal form of NKH is died, neuropatological examination revealed corpus callosal agenesis and diffuse hypomyelinisation. The two children treated with N-methyl-D-aspartate-antagonist drugs reached a significantly better clinical condition. The authors reviewed the data of the literature, especially focused on the therapeutical possibilities.

Plasma amino acid concentration under human growth hormone treatment in uremic children.

The plasma amino acid concentrations were investigated before and after 3 and 30 months of human recombinant growth hormone treatment in 7 children with chronic renal failure. The concentrations of amino acids in plasma showed characteristic changes (pretreatment vs. after 3 and 30 months of treatment): Lys 113 +/- 33 vs. 162 +/- 27 and 109 +/- 38 mumol/l, Met 21 +/- 8 vs. 31 +/- 4 and 16 +/- 5, Thr 105 +/- 23 vs. 148 +/- 60 and 118 +/- 30, Ala 455 +/- 109 vs. 536 +/- 93 and 314 +/- 60, Gln 298 +/- 66 vs. 277 +/- 52 and 544 +/- 65, Glu 168 +/- 46 vs. 209 +/- 57 and 96 +/- 24, Gly 345 +/- 137 vs. 479 +/- 169 and 342 +/- 95, Pro 378 +/- 148 vs. 422 +/- 28 and 527 +/- 229, OH-Pro 33 +/- 17 vs. 105 +/- 23 and 97 +/- 35, Se 133 +/- 39 vs. 178 +/- 55 and 131 +/- 12 mumol/l. Long-term treatment with human recombinant growth hormone normalized plasma alanine, glutamine, and glutamic acid levels, increased the OH-Pro concentration, and did not alter the amino acid ratios of Gly/Val, Phe/Tyr, Ser/Gly, and Asn/Asp, but the Gln/Glu ratio approached the normal value.

Red cell sodium-lithium countertransport and blood pressure in children with insulin-dependent diabetes mellitus.

Sodium-lithium countertransport and blood pressure responses, maximal elevated plasma norepinephrine concentrations induced by acute physical work load and the carbohydrate metabolic state were analyzed in 40 children suffering from insulin-dependent diabetes mellitus (IDDM). Patients were selected according to the duration of the disease to get a horizontal insight into the progression of the diabetes. Sixteen healthy children served as controls. Sodium-lithium countertransport (Na-Li CT) was 281 +/- 64 mumol/l red blood cells (RBC) per hour in the control group. Na-Li CT was elevated in all diabetic groups (newly diagnosed: 455 +/- 48; diabetics for 5-7 years: 495 +/- 48; diabetics for 10-13 years: 470 +/- 36). Plasma norepinephrine concentration increased during physical exercise, the elevation was more pronounced in diabetic children being 13.5 +/- 10.4, 10.1 +/- 5.0 and 12.3 +/- 5.4 nmol/l in the three diabetic groups, respectively, which differed significantly from that of controls (7.94 +/- 2.9; P < 0.01). Systolic blood pressure increased significantly during physical exercise in each group. However, maximal elevated systolic blood pressure was higher in children who had diabetes for more than 10 years than in controls (158 +/- 11 vs. 137 +/- 9.7 mmHg; P < 0.001). Na-Li CT correlated positively with the maximal systolic blood pressure measured during physical exercise in those diabetic children who suffered from diabetes for more than 5 years. High activity of Na-Li CT in combination with elevated blood pressure and high plasma concentration of norepinephrine induced by acute physical exercise may represent a risk of renal/vascular complications in patients suffering from IDDM.

Sympathetic-adrenergic activity and acid-base regulation under acute physical stress in type I (insulin-dependent) diabetic children.

To evaluate the efficacy of the acute-physical-stress response, plasma catecholamine and lactate levels, serum electrolytes, fructosamine, blood glucose and acid-base status were measured in insulin-dependent diabetes mellitus (IDDM) children and the data compared to those of healthy controls. Four groups were studied: group 1, healthy controls; group 2, newly diagnosed diabetic patients with an IDDM duration of 2-4 weeks; group 3, with an IDDM duration of 5-7 years; group 4, with an IDDM duration of 10-13 years. According to their fructosamine levels, IDDM children were in a well-controlled metabolic state. The physical stress was induced by 1.5-1.7 W/kg/10 min bicycle ergometer determined by a target pulse rate of 170/min. IDDM children exhibited pronounced lactic acidosis under stress (pH: group 2, 7.27 +/- 0.07; group 3, 7.28 +/- 0.05; group 4, 7.20 +/- 0.04, vs. group 1; 7.34 +/- 0.03). Baseline plasma norepinephrine and epinephrine levels showed a significant decrease parallel to the duration of IDDM. Stress induced an increase in the concentration of norepinephrine in each group, but the elevation was significantly higher in the IDDM children versus the controls. A significant negative correlation was found between pH and maximal plasma norepinephrine levels (y = 7.3-0.006x, r = -0.46, p < 0.02). Stress resulted in blood glucose elevation in 13 patients regardless of their pre-exercise blood glucose levels.(ABSTRACT TRUNCATED AT 250 WORDS)

[Biochemical, clinical and genetic analysis of various aminoacidopathies (non-ketotic hyperglycemia, maple syrup urine disease, histidinemia, tyrosinemia)]. / Néhány aminoacidopathia (non-ketotikus hyperglycinaemia, jávorfaszörp-betegség, histidinaemia, tyrosinaemia) biokémiai, klinikai és genetikai elemzése.

The genetical types were classified according to the clinical findings and biochemical results in cases of 13 newborn/children suffering from various aminoacidopathies. The genetical types were: 3 neonatal and 4 infantile types were found out of 7 non-ketotic disease (MSUD) patient was infantile type with 9.1 per cent keto acid decarboxylase activity in leukocyte homogenate. Among the 3 histidinemic patients 1 was severe neonatal type and 2 cases were chronic types. The 2 treated tyrosinemic children proved to be type III. (chronic with rickets).

The role of sympathetic-adrenergic activity in the regulation of acid-base homeostasis and renal sodium excretion under acute physical stress in type-I diabetic children (IDDM).

To evaluate the ability of stress response, plasma and urinary catecholamine concentrations, urinary electrolytes and acid-base status were measured in IDDM children (Group I: newly diagnoses 2-4 weeks, n = 7; Group II: duration 5-7 yr, n = 9; Group III: duration 10-13 yr, n = 12). The data were compared to healthy controls, n = 7, (mean +/- SD). Physical stress was induced by 2 W/kg/10 min bicycle ergometer. IDDM children exhibited metabolic acidosis under stress (pH: Group I = 7.24 +/- 0.08; Group II = 7.25 +/- 0.05; Group III = 7.19 +/- 0.03 vs C = 7.36 +/- 0.02). Stress induced an increase in the concentration of plasma norepinephrine (PNE in each group, the most pronounced elevation was seen in Group I (19.36 +/- 8.8 nmol/I vs C = 8.3 +/- 3.2, p < 0.2). Baseline PNE level showed a significant decrease with the duration of IDDM. Excretion of urinary NE (UNE) also increased under stress, however, the highest levels were measured in Group III (580 +/- 209 pmol/min/l. 73m2 vs 290 +/- 124 in Group I, p < .01). Baseline urinary dopamine (UDA) excretions were similar in each group. Stress caused an elevation in UDA only in C (2.05 +/- 1.8 vs 4.59 +/- 2.9 pmol/min.l. 73m2). The ratio of baseline UNE/UDA was similar in Groups I, II and C, but higher in Group III. Stress induced a shift towards NE excretion only in diabetic children which was most pronounced in children having IDDM more than 10 years. Stress did not affect urine output in Groups I. and II., but a decrease was observed in Group III (1.1 +/- 0.3 vs 0.7 +/- 0.3 ml/min.1.73 m2, p < .007). Urinary sodium excretion also decreased in Group III after physical loading (130 +/- 47 vs 66 +/- 33 umol/min/1.73 m2). The data show, that physical stress induces a severe lactic acidosis in IDDM. In spite of the decreased systemic sympathetic activity, the renal catecholamine response showed a shift towards vasoconstriction, sodium and fluid retention under physical stress in IDDM. These changes correlate with the duration of the underlying disease.

[Effect of somatostatin on kidney function]. / A somatostatin hatása a vese müködésére.

The renal effect of cyclic somatostatin was studied on healthy subjects. The somatostatin was used at therapeutical dose in intravenous infusion. Somatostatin decreases the renal plasma flow, glomerular filtration rate, osmotic and free water clearances, sodium and potassium excretion and the tubular reabsorption of phosphorus while urinary osmolality increases. Under somatostatin infusion the urinary excretion of catecholamines, PGE2, PGF2 alfa and the plasma renin activity and the plasma concentration of glucagon and growth hormone decrease. The antidiuretic activity of somatostatin is due to a) a direct haemodinamic effect, b) an influence on the renal tubular transport as well and also c) because of change the water handling in the collecting ducts.

Effect of somatostatin on kidney function and vasoactive hormone systems in health subjects.

The acute effects of i.v. somatostatin (250 mcg bolus followed by 250 mcg/h continuous infusion for two hours) on renal hemodynamics, renal electrolyte and water handling, and urinary excretion of catecholamines and prostaglandins, as well as on plasma concentrations of arginine vasopressin, atrial natriuretic factor, norepinephrine, epinephrine, dopamine, glucagon, and plasma renin activity were studied in seven normal subjects. Somatostatin decreased effective renal plasma flow and glomerular filtration rate, osmotic and free water clearances, urine volume, and sodium and potassium excretion, while urinary osmolality, fractional excretion of sodium, and phosphate excretion increased significantly. Plasma concentrations of arginine vasopressin, atrial natriuretic factor, norepinephrine, epinephrine, and dopamine remained unchanged, while plasma renin activity (3.0 +/- 0.25 vs 2.4 +/- 0.2 ng AngI/ml/h; p less than 0.01) and glucagon levels (40 +/- 11 vs 20 +/- 16 pg/ml; p less than 0.01) decreased. Urinary excretion of norepinephrine, epinephrine, dopamine, PGE2, and PGF2 alpha was suppressed under somatostatin. A significant positive correlation was found between urinary dopamine and sodium excretion (r = 0.7; p less than 0.001) and urinary prostaglandin E2 and glomerular filtration (r = 0.52; p less than 0.01). Without accompanying changes in plasma osmolality and vasopressin concentration significant antidiuresis occurred, suggesting a direct tubular effect of somatostatin. However, the hormone-induced changes are due mainly to the decrease in renal plasma flow. The results demonstrate that somatostatin at supraphysiological doses exerts significant effects on the kidney.

Changes in plasma and urinary amino acid levels during diabetic ketoacidosis in children.

Plasma and urinary concentrations of different amino acids were investigated during diabetic ketoacidosis (DKA) and 12, 24, 72 hours after initiation of therapy. In DKA, plasma concentration of glutamic acid, aspartic acid, valine, leucine and isoleucine significantly increased while that of asparagine and glutamine decreased compared to levels in well-controlled diabetic patients. The urinary excretion of branched-chain amino acids, histidine, serine and threonine was elevated while those of glutamic acid, glutamine, glycine and taurine were reduced. Among the different amino acids, histidine excretion had the highest variability. A strong correlation was found between the urinary excretion of several amino acids and that of the beta-2-microglobulin characterizing tubular dysfunction. Changes in the excretion of different amino acids reflect the altered metabolic state and renal function due to DKA.

Urinary catecholamine in children with diabetic ketoacidosis.

Urinary excretion of norepinephrine, epinephrine and dopamine was investigated in children with diabetic ketoacidosis. Besides pronounced hyperglycemia and metabolic acidosis, severe hypovolemia was also observed. At the onset of the study, urinary excretion of norepinephrine, epinephrine and dopamine was markedly elevated. Fluid replacement decreased urinary catecholamine excretion. Norepinephrine: 996 +/- 97 vs. 253 +/- 29; dopamine: 5,108 +/- 480 vs. 3,175 +/- 715; epinephrine: 402 +/- 81 vs. 77 +/- 200 pmol/min/1.73 m2. During ketoacidosis, there was a significant negative correlation between urinary norepinephrine excretion and endogenous creatinine clearance. Urinary output of both norepinephrine and dopamine correlated significantly with diuresis, while sodium excretion only correlated with dopamine excretion. Our data suggest that in diabetic ketoacidosis increased urinary excretion of norepinephrine may participate in renal hypoperfusion and hypofiltration. Elevated renal dopamine production contributes to sodium loss, characteristic for diabetic ketoacidosis.

[Follow-up of blood and urinary amino acid concentrations in diabetic ketoacidosis in children]. / A plazma és a vizelet aminosav koncentrációk nyomon követése gyermekkori diabeteses ketoacidózisban.

Plasma and urinary concentrations of amino acids were investigated during diabetic ketoacidosis (DKA) and 12, 24, 72 hours after initiation of therapy. In DKA plasma concentration of glutamic acid, asparaginic acid, valine, leucine and isoleucine significantly increased while that of asparagine and glutamine decreased compared to levels in well controlled diabetic patients. Despite the elevated urinary excretion of branched chain amino acids, histidine, serine and threonine, urinary excretion and clearance of glutamic acid, glutamine, glycine and taurine were reduced. Among the different amino acids histidine excretion had the highest variability. Strong correlation was found between the urinary excretion of several amino acids and that of the beta-2-microglobulin characterizing tubular dysfunction. Changes in the excretion of different amino acids reflect the altered metabolic state and renal function due to diabetic ketoacidosis.

Feeding preterm infants with L-carnitine supplemented formula.

A total of 29 preterm infants maintained on mixed enteral nutrition (50% pooled human milk, 50% formula daily) were studied over a 15 days period. 16 of them received L-carnitine supplemented formula during the first seven consecutive days (600 nmol/ml, as added supplement), 13 infants served as controls. In response to enhanced dietary intake, the plasma levels and urinary excretion rates of carnitines were increased by the 7th day of study. The plasma carnitines then returned to the initial values, whilst the urinary excretion remained elevated at the 14th day of study. The elevated daily urinary excretion of carnitines was accompanied by increased clearance and decreased relative reabsorption rates in the supplemented group. In the control group the plasma carnitine levels remained unchanged throughout the observations, while the daily excretion of free carnitine decreased by the end of the study. In the supplemented group statistically significant decrease was found in the daily excreted ammonia and urea with a decrease of plasma alanine and glutamine levels by the 7th day of study. The plasma levels of beta-hydroxybutyrate, glucose and creatinine remained unchanged in both groups.

[The significance of the determination of catecholamines and their metabolites in neuroblastomas]. / Die Bedeutung der Bestimmung von Katecholaminen und deren Metaboliten bei Neuroblastomen.

A method for the determination of catecholamines and their metabolites in the urine was established. The specimens were previously cleaned by ion exchangers and the components were separated by a 'reversed phase' ionic pair chromatography, determined by an electrochemical 'wall-jet'-detector. This method was applied in order to find a diagnostic marker in case of neuroblastoma diseases. In 85 per cent of the patients (n = 13) we stated an increased ejection of dopamine.

Changes of plasma free amino acids and renal clearances of carnitines in premature infants during L-carnitine-supplemented human milk feeding.

Eighteen breast-fed infants with a mean post-natal age of 26 days (range 16-41 days) were studied. Following 1 control day, the infants were fed for 7 consecutive days with pooled human milk supplemented with 300 nmol L-carnitine/ml milk. Both plasma fractions of acid-soluble carnitines increased as a consequence of carnitine application. The level of beta-hydroxybutyrate also increased significantly. Of the circulating free amino acids, the levels of alanine (p less than 0.025) and glutamine (p less than 0.01) were found to be lower, with a decreased urea level (p less than 0.005) by the last day of carnitine administration, compared with the control day. The urinary output of total nitrogen also decreased. There were no statistically significant changes in the level of free fatty acids and glucose. On the control day, the renal clearance rate of esterified carnitines significantly exceeded that of free fraction, thus the relative renal reabsorption calculated on the base of creatinine excretion rates was higher for free (mean 98.1%) than for acylated (mean 90.6%) carnitine. In response to enhanced carnitine intake, the clearance rates for each fractions of carnitines significantly exceeded the presupplementary values. The increased clearance rates was more pronounced for free (mean 13.2-fold) than for esterified (mean 8.08-fold) carnitines. Despite the increased clearance rates, considerable relative reabsorption was seen for free carnitines (mean 70.0%) as well as for acylcarnitines (mean 65.3%).

Vasoactive hormones in familial pheochromocytoma with predominant norepinephrine secretion.

A family was investigated with an increased occurrence of pheochromocytoma. Three males out of 7 family members showed biochemical and morphological evidence of catecholamine-secreting tumor. Plasma concentrations of norepinephrine, aldosterone, arginine vasopressin and renin activity, as well as urinary excretion of norepinephrine, were enhanced in all the 3 patients. The plasma level of atrial natriuretic peptide decreased. After surgical removal of the tumor the hormonal alterations tended to be normalized; however, plasma concentration of norepinephrine remained above normal. The elevated plasma norepinephrine was postoperatively accompanied by slightly increased urinary norepinephrine excretion in 1 patient without any detectable tumor. The severity of the disease was most pronounced in the youngest patient, while in the oldest one a 'silent' pheochromocytoma was found. The occurrence of familial pheochromocytoma in successive generations may suggest an autosomal-dominant inheritance.