Signature inversion caused by triaxiality and unpaired band crossings in 72Br

High-spin states in 72Br were studied with the EUROBALL III spectrometer using the 40Ca(40Ca,alpha3p1n) reaction. The negative-parity band observed in this experiment displays a signature inversion around spin I = 16. The interpretation within the cranked Nilsson-Strutinsky approach shows that this signature pattern is a signal of a substantial triaxial shape change with increasing spin where the nucleus evolves from a triaxial shape with rotation about the intermediate axis at low spin through a collective prolate shape to a triaxial shape but with rotation about the shortest principal axis at high spin.

Observation of proton radioactivity of the (21+) high-spin isomer in 94Ag.

We have observed direct one-proton decay of the (21+) isomer in the N=Z nuclide 94Ag into high-spin states in 93Pd by detecting protons in coincidence with gamma-gamma correlations and applying gamma gates based on known 93Pd levels. Two decay branches have been identified, with proton energies of 0.79(3) and 1.01(3) MeV and branching ratios of 1.9(5)% and 2.2(4)%, respectively. The corresponding partial half-life values are 21(6) and 18(4) s. The Q value of the direct proton decay of the (21+) isomer was found to be 5.78(3) MeV. The very small reduced widths of the observed proton decays might reflect dominating collective configurations in the (21+) isomer, and the fine structure of the proton spectrum might indicate a strong deformation of this state.

Evidence for nontermination of rotational bands in 74Kr.

Three rotational bands in 74Kr were studied up to (in one case one transition short of) the maximum spin I(max) of their respective single-particle configurations. Their lifetimes have been determined using the Doppler-shift attenuation method. The deduced transition quadrupole moments reveal a modest decrease, but far from a complete loss of collectivity at the maximum spin I(max). This feature, together with the results of mean field calculations, indicates that the observed bands do not terminate at I = I(max).

[Disaccharidase activity as a diagnostic criterion for the mucosa of the small intestine]. / Disaccharidasen-Aktivitäten als Beurteilungskriterium der Dünndarmschleimhaut.

Activities of lactase, sucrase, and maltase were determined in small intestinal biopsies of 125 children with coeliac disease, cow's milk protein intolerance, transient gluten intolerance, nonspecific enteropathies, and controls. Four cases of primary disaccharidase deficiencies could be identified. In the enteropathies, morphometric data were more closely correlated to the degree of the mucosal lesion (r = -0.92 for crypt depth) than were disaccharidase activities (r = 0.61 for lactase). In a stepwise discriminant analysis of the patient groups, based upon immunological, morphometric, and biochemical variables, lactase activity was a valuable secondary criterion, ranking third among the variables used.

Urinary excretion of aromatic acids in hyperphenylalaninemic states: response to a protein challenge.

A protein challenge (3g/kg) was given to 15 hyperphenylalaninemic patients for 4 days and urinary excretion of aromatic phenylalanine metabolites (e.g. phenylpyruvic acid; mandelic acid; ortho-hydroxy-phenylacetic acid) was studied by quantitative gas chromatographic analysis. Two groups of patients, different in their serum phenylalanine pattern, could be distinguished from the pattern of urinary aromatic acid excretion. In the first group, the aromatic acid excretion declined towards the end of the challenge whereas aromatic acid excretion increased throughout the challenge in the second group. The differences could be ascertained by statistical analysis.

Effects of growth hormone on protein metabolism. Acute changes in plasma amino acids in growth retarded patients with and without growth hormone deficiency.

39 patients with growth retardation were investigated: 21 (group H) were suffering from GH deficiency and 18 (group N) had no endocrine disease except for two adequately treated patients with mild hypothyroidism. Analysis of 15 plasma amino acid concentrations was carried out before and 1 and 2 hours after intravenous HGH injection at a dosage of 2 mg per m2. Except for one amino acid no significant difference between mean pre-treatment amino acid values was observed in the two groups of patients. In group H there was a highly significant decrease in plasma concentration of 14 amino acids already 1 hour after HGH injection and of all 15 amino acids after 2 hours. This response of plasma amino acids to HGH was less pronounced in group N. For 5 amino acids a moderate correlation was found in group H between acute metabolic response to HGH and growth response to long-term HGH treatment. Our results following HGH injection may reflect increased plasma amino acid transfer into cells due to HGH.

[Pancreatic enzyme replacement in mucoviscidosis (CF): clinical evaluation of a gastric acid-resistant pancreatin preparation in encapsulated microtablet form]. / Pankreasenzymsubstitution bei Mukoviszidose (CF): Klinische Prüfung eines magensäureresistenten Pankreatinpräparates in verkapselter Mikrotablettenform.

Pancreatic enzyme replacement therapy was tested in 17 patients with CF. Severe pancreatic insufficiency had been established by stool fat determination prior to therapy. Two enzyme preparations were tested that were equal in acid protection, but were different in the release of enzyme activities. Complaints, body weight, and stool frequency were not influenced by changing preparations. Stool weight and stool fat excretion did not show statistically significant differences, when the preparations were used in lipase equivalent doses. By using one of the preparations tested (Panzytrat 20,000), it was possible to reduce the number of capsules that had to be taken daily. Mild to moderate steatorrhoea was noted in spite of pancreatic enzyme replacement in all the CF patients. Massive steatorrhoea was seen in two patients with good nutritional status, due to high fat intake. It was proposed that moderate steatorrhoea may be tolerated in CF, as long as the nutritional status is adequate. If this is not the case, energy intake and, as a consequence, the dosage of pancreatic enzyme replacement therapy have to be raised.

Plasma amino acid pattern at noon in early treated hyperphenylalaninemic, phenylketonuric, and normal children.

In 41 hyperphenylalaninemic early treated children (classical phenylketonurics and hyperphenylalaninemic variants) on diet, aged 2 months to 15 years, the plasma amino acid pattern at noon was compared with that in 31 normal age-matched controls. In contrast to previously published results in infants, the mean levels of single amino acids deviated, with the exception of phenylalanine, only slightly from that of normal controls in patients of all five age groups investigated. Constant findings were a tendency towards low mean levels of arginine in younger patients and elevated levels of lysine, serine, and histidine which were found in patients of all age groups.

[Neonatal screening for mucoviscidosis using the BM-test-meconium]. / Neugeborenen-Screening auf Mucoviscidose mit dem BM-Test-Meconium.

In the Federal Republic of Germany screening for cystic fibrosis by the albumin content of meconium (BM-test) is performed on most newborns. In this paper arguments for and against this test are discussed. Arguments in favour of BM-test-screening are the possibility of early diagnosis, early treatment and genetic counseling. Drawbacks of the test are false positive and false negative results. The authors conclude that this screening test should not be performed.

[Intermittent branched--chain ketoacidurie in ketotic hypoglycemia: investigations to localize the biochemical defect (author's transl)]. / Intermittierende verzweigtkettige Ketoacidurie bei ketotischer Hypoglykämie: Untersuchungen zur Lokalisation des biochemischen Defekts

We are reporting a girl aged eight years with ketotic hypoglycemia, mental deficiency and retarded motor and somatic development. Investigation of plasma amino acid concentrations during a spontaneous hypoglycemia revealed an increase in the branched-chain amino acids valine (4.1), leucine (7.8) and isoleucine (1.7 mg/100 ml), while alanine was decreased (1.2 mg/100 ml) and ketonuria was present. The determination of the branched-chain ketoacid decarboxylase in leukocytes showed a decrease of approximately 50% of normal for alpha-ketoisocaproic acid (KIC) as substrate, whereas values for alpha-ketoisovaleric acid (KIVA) and alpha-keto-beta-methylvaleric acid (MEVA) were normal. In fibroblasts activities for all three substrates were in the normal range. Intermittend maple-syrup-urine disease was excluded by oral loading tests with the branched-chain amino acids and with an isocaloric, high-protein diet. Impairment of oxydative decarboxylation of leucine, valine, and isoleucine secondary to increased ketogenesis may play an etiologic role in ketotic hypoglycemia, since we observed, by gaschromatographic analysis, an increase in the urinary excretion of KIVA (5.5 mumol/h), KIC (29.4), and MEVA (47.9) after a provocative test with an isocaloric ketogenic diet for 36 hrs. The significance of branched-chain hyperaminoacidemia and branched chain alpha-ketoaciduria is discussed in this context.

[The phenylalanine stress test in the classification of patients with phenylketonuria]. / Der Phenylalanin-Belastungstest in der Klassifikation der Phenylketonurie-Patienten.

Classification of defects of phenylalanine hydroxylase is usually performed by a phenylalanine loading test according to Blaskovics. This loading test has some drawbacks, especially negative side effects for the patients. We found a correlation between the result of the loading test and the dietetic phenylalanine tolerance. Therefore the trouble of undergoing the loading rest may be spared many patients.

Screening of congenital hypothyroidism, phenylketonuria, galactosemia, homocystinuria, and maple syrup urine disease in moderate to severe mentally retarded Chinese children.

Five-hundred and fifty one mentally retarded children from seven institutes in Northern Taiwan were screened by dried blood spot for the detection of treatable congenital metabolic diseases, including congenital hypothyroidism, phenylketonuria, homocystinuria, maple syrup urine disease and galactosemia. We found 2 children (0.36%) with congenital hypothyroidism, 1 case (0.18%) of classical phenylketonuria and two cases (0.36%) of trisomy 21 associated with autoimmune thyroiditis. The results of our investigation suggest that congenital hypothyroidism and phenylketonuria can be the factors causing mental retardation among children in Taiwan and mass neonatal screening of these treatable inborn metabolic diseases is strongly indicated for efficiently circumventing mental retardation in our community.