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  • 1
    Digitale Medien
    Digitale Medien
    Springer
    The European physical journal 306 (1982), S. 315-322 
    ISSN: 1434-601X
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Physik
    Notizen: Abstract Eigensolutions of the Bohr collective Hamiltonian are obtained by expanding its eigenstates on a truncated basis which is adaptable to the most general potential energy and mass parameter surfaces. After a short survey of the method in use, its numerical accuracy is assessed from results obtained in some selected cases. The results of preliminary calculations for154, 156, 158Er and232Th nuclei (where the potential energy surfaces are deduced from self-consistent calculations using the Skyrme SIII force) are also briefly discussed.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    Digitale Medien
    Digitale Medien
    Springer
    Acta neuropathologica 45 (1979), S. 247-251 
    ISSN: 1432-0533
    Schlagwort(e): Infantile neuro-axonal dystrophy ; Skin ; Conjunctiva ; Spheroids
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary The diagnosis of infantile neuro-axonal dystrophy (INAD) in a 5-year-old patient was confirmed by the ultrastructural study of neuromuscular, skin and conjunctival biopsy specimens. Abnormal networks of smooth membranous, lamellar and tubular profiles were found in presynaptic terminals and in conjunctival and dermal axons. INAD is the first neurological disease outside the group of storage disorders in which skin and conjunctival biopsies contribute significantly to the diagnosis.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 3
    Digitale Medien
    Digitale Medien
    Springer
    Acta neuropathologica 53 (1981), S. 87-91 
    ISSN: 1432-0533
    Schlagwort(e): Fetal Krabbe leukodystrophy ; Galactosyl-ceramide-β-galactosidase ; Globoid cells ; Spinal cord ; Peripheral nerves
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Two new cases of Krabbe disease were diagnosed prenatally in a family with two previous affected children. The activity of galactosylceramide-β-galactosidase was virtually absent in cultured amniotic cells. The prenatal diagnosis was confirmed enzymatically in cultured fibroblasts, brain, and visceral organs. Light and electron microscopy studies in both fetuses, 20 and 23 weeks of gestational age respectively, revealed the presence of typical globoid cells in the white matter of the spinal cord. Specific inclusions were also found in the brain stem and in peripheral nerves of the second fetus. A comparison with other Krabbe disease fetuses described in the literature contributes to the consensus that abnormal morphological findings can be expected in particular in the most actively myelinating areas of the nervous system. Although most of the cells containing the specific melusions are probably non-glial in nature, some of them could represent myelination glia.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 4
    Digitale Medien
    Digitale Medien
    Springer
    Acta neuropathologica 38 (1977), S. 247-250 
    ISSN: 1432-0533
    Schlagwort(e): Conjunctival biopsy ; Skin biopsy ; Adrenoleukodystrophy ; Nerve bundles ; Vacuolated eccrine glands
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Conjunctival and skin biopsies were performed in an 111/2 year-old caucasian male affected by adrenoleukodystrophy (ALD). In Schwann cells surrounding myelinated axons in conjunctival and dermal nerve bundles, empty clefts and a few arrays of lamellae were discovered. The vacuolization in the eccrine glands of the skin, another striking feature, has not been reported previously in ALD. The obtained results suggest that ALD can be diagnosed in skin specimens precluding major surgery for biopsy. They provide support to the hypothesis of Schaumburg et al. (1975) that ALD is a generalized metabolic disorder.
    Materialart: Digitale Medien
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  • 5
    Digitale Medien
    Digitale Medien
    Springer
    Cellular and molecular life sciences 40 (1984), S. 425-429 
    ISSN: 1420-9071
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Biologie , Medizin
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 6
    ISSN: 1432-1076
    Schlagwort(e): Lysosomal storage disease ; Sialic acid ; Mucolipidosis
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract We describe two sibs with coarse facies, hepatosplenomegaly, prominent psychomotor retardation and unexpectedly fair complexion. Ultrastructural studies of conjunctival, skin, bone marrow and liver biopsies from these individuals showed generalized lysosomal storage of polysaccharidelike material, i.e., membrane bound inclusions containing sparse, fibrillo-granular material. Biochemical analyses of urine and cultured fibroblasts from these patients revealed increased levels of free (unbound) sialic acid. The ultrastructural and biochemical findings in these sibs are similar to those previously found in Salla disease, however, the clinical course is much more severe. It is concluded that these children represent a new pathogenetic entity whose basic defect is still to be defined.
    Materialart: Digitale Medien
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  • 7
    Digitale Medien
    Digitale Medien
    Springer
    European journal of clinical pharmacology 27 (1984), S. 173-179 
    ISSN: 1432-1041
    Schlagwort(e): benzodiazepine ; thermoregulation ; sleep ; rectal temperature ; mean skin temperature ; noise ; triazolam ; cortisol excretion ; urinary catecholamines
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Chemie und Pharmazie , Medizin
    Notizen: Summary The influence of a benzodiazepine (triazolam) on the body temperature of poor sleepers during nights disturbed by airplane noises has been examined. Subjects were divided into 3 groups each of 8 men. Following a double-blind design, Group A (controls) received a placebo for 6 consecutive nights, Group B received 0.25 mg triazolam, and Group C received 0.5 mg triazolam on nights N3, N4 and N5. On all other nights Group B and C subjects received placebo. For all 3 groups, nights N3 and N5 were disturbed by 32, semi-randomly distributed airplane noises. Air and wall temperatures (To=20°C), and air humidity (Tdp=10°C), were kept constant. Rectal temperature and 4 local skin temperatures were recorded from each subject. Urine samples were collected each morning for measurement of cortisol and catecholamine levels. Noise was found to cause an increase in body heat storage, but only in Group A. Both drug groups showed impairment of body heat balance. The hypothermic action of triazolam could be explained both by a central action of the drug on the thermoregulatory controller and by a peripheral action on blood vessels.
    Materialart: Digitale Medien
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  • 8
    Digitale Medien
    Digitale Medien
    Springer
    Journal of inherited metabolic disease 4 (1981), S. 103-104 
    ISSN: 1573-2665
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract This male infant was first brought to attention in the neonatal period because he presented clinical and radiological evidence of multiple bone deformities. He was readmitted at 2 1/2 months for hydrocephaly, hepatosplenomegaly and poor somatic and psychomotor development. In addition, coarse facies, corneal opacities and stiff joints were noticed. Bone X-ray anomalies and vacuolized lymphocytes supported the clinical presumption of lysosomal storage disorder. The diagnosis of multiple sulphatase deficiency rests on the presence of MPS and sulphatides in the urine, the finding of a mixed storage process in conjunctival biopsy and the demonstration of deficiencies in arylsulphatases A, B, C, iduronate sulphatase and heparan sulphatase in serum, leukocytes and cultured fibroblasts.
    Materialart: Digitale Medien
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  • 9
    Digitale Medien
    Digitale Medien
    Springer
    European journal of applied physiology 44 (1980), S. 123-133 
    ISSN: 1439-6327
    Schlagwort(e): Body drippage ; Sweating decrease ; Evaporative adjustments ; Active sweat glands
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Sweat rate and the rate of change in sweat drippage were studied during the acclimation of eight healthy male subject during exposure to heat during 10 consecutive days. During acclimation to hot humid conditions, the increase in total body sweat rate results in an increase in the rate of sweat drippage. We found, however, that on each day the drippage rate markedly decreased with time after the 1st h of heat exposure. This hidromeiosis was investigated as a function of the heat exposure time. No shortening of the onset time of hidromeiosis occurred with acclimation. With repeated heat exposures, the initial sweat rates in response to stress increased, and the subsequent decline became larger with higher sweat rates at the time of onset of hidromeiosis. Hidromeiosis appears to be a function of the degree of skin wettedness reached in the various local skin areas which determine the overall body skin wettedness upon which evaporative adjustments depend. Thus, the observed overshoot in total sweat rate as indicated by sweat drippage, and the subsequent hidromeiosis, result from initial oversweating in the poorly ventilated areas of skin. This sweat decline seems to be due to a reduction in output of the active sweat glands rather than to a reduction in active sweat gland number.
    Materialart: Digitale Medien
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  • 10
    Digitale Medien
    Digitale Medien
    Springer
    European journal of applied physiology 50 (1983), S. 235-246 
    ISSN: 1439-6327
    Schlagwort(e): Heat acclimation ; Transient thermal loads ; Local sweating response
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary The sweating response was studied before and after passive humid heat acclimation in four resting male subjects who were exposed to slow thermal transients increasing air and wall temperatures from 28
    Materialart: Digitale Medien
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