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  • 1
    ISSN: 1432-1076
    Keywords: Pyruvate dehydrogenase deficiency ; Hyperammonaemia ; Peritoneal dialysis ; Lipoic acid ; Plasma amino acids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 2-day-old girl developed a severe lactic acidosis with a normal lactate/pyruvate ratio and hyperammonaemia. Plasma arginine and citrulline levels were below the limit of detection. In muscle total pyruvate dehydrogenase complex (PDHC) and pyruvate decarboxylase (E1) activities were reduced to a fraction of lower control values. The acute neonatal period was bridged with peritoneal dialysis, dichloroacetate therapy, supplements of arginine and branched chain amino acids, a complete vitamin B complex and lipoic acid. Lactate homeostasis responded to pharmacological supplements of lipoic acid. At age 1 year the child was hypotonic, showed severe developmental retardation, optic atrophy and cranial dysmorphism. She died aged 1 year 8 months with signs of respiratory paralysis but with normal lactate levels under assisted breathing. Pathological findings at autopsy were suggestive of Leigh syndrome, interstitial pneumonia and extensive fatty infiltration of hepatocytes. Regression analysis of data from 187 plasma amino acid determinations from the patient over a period of 1 year 8 months revealed a persistent imbalance involving alanine, glutamic acid, glutamine, proline, citrulline and branched chain amino acids. Aspects of acute and long-term therapy in this patient and some implications of the imbalances in plasma amino acids are discussed.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 152 (1993), S. 244-249 
    ISSN: 1432-1076
    Keywords: Nephropathic cystinosis ; Growth ; Puberty
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a retrospective investigation growth and pubertal development were evaluated in 30 patients with nephropathic cystinosis. Growth was investigated during the stage of chronic renal insufficiency as well as after successful kidney transplantation and growth rates were related to kidney function. Pubertal development was evaluated in 17 patients between 12 ans 25 years of age. Prepubertal growth rates were stable in a range between −2 and −3 height velocity SDS as long as glomerular filtration rate was above 20ml/min per 1.73m2. A decrease in glomerular filtration rate below this threshold was followed by further decrease in height velocity. After kidney transplantation a significant catch-up growth was seen if immunosuppression was performed with cyclosporine A and lowdose prednisolone. This did not occur if conventional therapy with azathioprine and high-dose prednisolone was used. Onset of puberty was delayed in all patients. Gonadotropin and oestradiol levels in female patients showed normal fluctuations according to ovulatory cycles. In male patients after puberty there was an increase in gonadotropin levels above the normal range for adult men while testosterone levels remained in the low normal range. These results indicate that adult men with nephropathic cystinosis may develop hypergonadotropic hypogonadism.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Clinica Chimica Acta 76 (1977), S. 187-191 
    ISSN: 0009-8981
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 0009-8981
    Keywords: Apolipoproteins ; Blood ; Lecithin-cholesterol acyltransferase ; Lipoproteins ; Reverse cholesterol transport
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Biochimica et Biophysica Acta (BBA)/Lipids and Lipid Metabolism 231 (1971), S. 535-536 
    ISSN: 0005-2760
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine , Physics
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Biochimica et Biophysica Acta (BBA)/Lipids and Lipid Metabolism 1127 (1992), S. 174-185 
    ISSN: 0005-2760
    Keywords: (Rat yolk sac) ; Apolipoprotein B ; Lipoprotein ; Mathematical model ; Perinatal period
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology , Chemistry and Pharmacology , Medicine , Physics
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    [S.l.] : American Institute of Physics (AIP)
    Review of Scientific Instruments 68 (1997), S. 4275-4281 
    ISSN: 1089-7623
    Source: AIP Digital Archive
    Topics: Physics , Electrical Engineering, Measurement and Control Technology
    Notes: A digital-imaging system based on a personal computer was tested for its ability to track in real time periodic motion with a frequency near 1 Hz. When imaging a vibrationally isolated stationary target, the system displayed a limitation in positional measurement of 0.3×10−6 m. Further tests indicate that this limitation arises from environmental vibrations driving the target, even though it is mounted on a vibration-isolation table. This work demonstrates the feasibility of using digital imaging to measure motions other than the (usually very low frequency) torsion motion of a torsion balance, where the ultimate goal is to remove the effects of these motions from the torsion signal of interest. © 1997 American Institute of Physics.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    [S.l.] : American Institute of Physics (AIP)
    Review of Scientific Instruments 63 (1992), S. 3556-3563 
    ISSN: 1089-7623
    Source: AIP Digital Archive
    Topics: Physics , Electrical Engineering, Measurement and Control Technology
    Notes: The torsion balance has been the experimental apparatus of choice for centuries, both in precision measurements of the Newtonian gravitational constant and in searches for weak anomalous interactions outside of gravity. If the form of the interaction is modeled, it is often possible to optimize the interacting bodies so that the apparatus has the greatest sensitivity to the interaction under study. Other researchers have applied this strategy in the case of the gravitational interaction between cylinders, and between a cylinder and sphere. Whereas their work focused on developing an analytical expression for the force between the masses, we present here a numerical method−Monte Carlo integration−which is general enough to aid in the design of bodies interacting under arbitrary potentials and with any desired geometric shape (as long as an accurate absolute value of the force is not needed). This numerical method is used to compute the gravitational torsion constant produced between an external hollow cylinder and sphere, and demonstrates the behavior studied previously through analysis. However, the main purpose for which we have used this numerical technique is in the design of interacting bodies used in a torsion-pendulum search for interactions that depend on net intrinsic spin. We demonstrate how the method may be used to determine the optimum aspect ratio (l/r) of the polarized test masses, as well as the most sensitive orientation of the masses. Two different interactions are considered: the dipole–dipole interaction between two polarized bodies, and the monopole–dipole interaction between a polarized and unpolarized body. In the case of the monopole–dipole interaction, we also show how the numerical method can indicate which orientation between test bodies is most susceptible to a false signal caused by gravity.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    [S.l.] : American Institute of Physics (AIP)
    Review of Scientific Instruments 57 (1986), S. 3019-3023 
    ISSN: 1089-7623
    Source: AIP Digital Archive
    Topics: Physics , Electrical Engineering, Measurement and Control Technology
    Notes: A method for measuring the force arising from the attraction between a charged electrode and another electrode on which charges have been induced has been developed using a torsion balance operated in the ac mode far above the resonance frequency of the balance. The movement of the balance is sensed by a capacitive position transducer which uses two lock-in amplifiers to demodulate the position signal. The balance itself and the position transducer are described. The sensitivity of the position transducer is given, and results from three electrode geometries are discussed.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Journal of Chromatography A 70 (1972), S. 164-167 
    ISSN: 0021-9673
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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