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  • 1
    Electronic Resource
    Electronic Resource
    Metabolism of D-ribose administered continuously to healthy persons and to patients with myoadenylate deaminase deficiency (1989)
    Springer
    Journal of molecular medicine 67 (1989), S. 1205-1213 
    Details
    ISSN: 1432-1440
    Keywords: (D-)ribose ; Metabolism ; Myoadenylate ; (adenylate-, AMP-)deaminase deficiency
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary D-ribose was administered orally or intravenously over at least 5 h to eight healthy volunteers and five patients with myoadenylate deaminase deficiency. Intravenous administration rates were 83, 167, and 222 mg/kg/h, which were well tolerated but oral administration of more than 200 mg/kg/h caused diarrhea. The average steady state serum ribose level ranged between 4.8 mg/100 ml (83 mg/kg/h, oral administration) and 81.7 mg/100 ml (222 mg/kg/h, intravenous administration). Serum glucose level decreased during ribose administration. The intestinal absorption rate of orally administered ribose was 87.8%–99.8% of the intake at doses up to 200 mg/kg/h without first pass effect. Urinary losses were 23% of the intravenously administered dose at 222 mg/kg/h. Ribose appeared to be excreted by glomerular filtration without active reabsorption; a renal threshold could not be demonstrated. The amount of ribose transported back from the tubular lumen depended on the serum ribose level. There was no difference in ribose turnover in healthy subjects and patients with MAD deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01716208
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Ribose administration during exercise: Effects on substrates and products of energy metabolism in healthy subjects and a patient with myoadenylate deaminase deficiency (1991)
    Springer
    Journal of molecular medicine 69 (1991), S. 151-155 
    Details
    ISSN: 1432-1440
    Keywords: Myoadenylate deaminase ; AMP-deaminase ; Deficiency ; Ribose ; Lactate ; Ammonia ; Hypoxanthine ; Muscle ; Metabolism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Nine healthy men and a patient with myoadenylate deaminase deficiency were exercised on a bicycle ergometer (30 minutes, 125 Watts) with and without oral ribose administration at a dose of 2 g every 5 minutes of exercise. Plasma or serum levels of glucose, free fatty acids, lactate, ammonia and hypoxanthine and the urinary hypoxanthine excretion were determined. After 30 minutes of exercise without ribose intake the healthy subjects showed significant increases in plasma lactate (p〈0.05), ammonia (p〈0.01) and hypoxanthine (p〈0.05) concentrations and a decrease in serum glucose concentration (p〈0.05). When ribose was administered, the plasma lactate concentration increased significantly higher (p〈 0.05) and the increase in plasma hypoxanthine concentration was no longer significant. The patient showed the same pattern of changes in serum or plasma concentrations with exercise with the exception of hypoxanthine in plasma which increased higher when ribose was administered.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01665856
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Datenschutz innerhalb des länderübergreifenden Deutschen Zentralregisters für kindliche Hörstörungen (1998)
    Springer
    HNO 46 (1998), S. 339-345 
    Details
    ISSN: 1433-0458
    Keywords: Schlüsselwörter Datenschutz ; Datensicherheit ; Medizinische Register ; Datenerhebung ; Kindliche Hörstörungen ; Key words Medical registries ; Data collection and security ; Hearing loss and childhood
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary The German Registry for Hearing Loss in Children (DZH) processes nationwide data from audiological centers. Coping with the accrued data and its subsequent management and analysis requires a high degree of security and control. To establish a nationwide registry it is necessary at an early stage to take into consideration the legal requirements of the participating states. Use of the DZH as an example demonstrates how a pragmatic solution can be reached. Special issues concerning data collection, transfer, storage and deletion, coding strategies to ensure anonymity, checking for duplicate entries, data separation, and automated data analysis and data protection are explained.
    Notes: Zusammenfassung Das Deutsche Zentralregister für kindliche Hörstörungen (DZH) verarbeitet bundesweit Daten von verschiedenen audiologischen Einrichtungen. Die Bewältigung der anfallenden Datenmengen, die nachfolgende Datenverwaltung und -analyse erfordern neben einer differenzierten und kontrollierbaren Verarbeitung ein Höchstmaß an Datensicherheit. Vor allem die länderübergreifende Struktur eines Registers erfordert schon bei der Planung engste Zusammenarbeit mit dem zuständigen Landesdatenschutzbeauftragten und auch mit den Landesdatenschutzbeauftragten anderer beteiligter Bundesländer. Am Beispiel des DZH wird demonstriert, wie eine kooperative Zusammenarbeit pragmatisch realisiert werden kann. Besonderheiten bei der Datenerhebung, Datentransfer, Speicherung und Löschung von Daten, technische Datenschutzmaßnahmen, Sicherstellung von Anonymität durch Codierungsstrategien, Duplikatsprüfung, Datentrennung und automatisierte Datenauswertung werden an Beispielen erläutert.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001060050249
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Wird die Diagnose bei persistierenden kindlichen Hörstörungen immer noch zu spät gestellt? (1998)
    Springer
    HNO 46 (1998), S. 598-602 
    Details
    ISSN: 1433-0458
    Keywords: Schlüsselwörter Diagnosezeitpunkt ; Verzögerte Diagnose ; Medizinische Register ; Datenerhebung ; Kindliche Hörstörungen ; Key words Age at diagnosis ; Delay of diagnosis ; Medical registries ; Data collection ; Hearing loss in childhood
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary Since 1994, the German Registry for Hearing Loss in Children has registered data of 1500 children and by now can present results concerning the age at diagnosis of permanent hearing loss in children in Germany. The mean age at diagnosis is still very high. There is a strong correlation between age at diagnosis and degree of hearing loss, i.e., severe and profound hearing loss, is diagnosed distinctly earlier than mild and moderate hearing loss. On average, mild hearing loss is diagnosed with 6.2 years, moderate h.l. with 4.4 years, severe hearing loss with 2.5 years and profound hearing loss with 1.9 years. This corresponds with the results of regional German studies. At least regionally, in other European countries the age at diagnosis is known to be distinctly lower. In 36% of the children registered in Germany the delay between first suspicion and diagnosis of permanent hearing loss is 1 year or more.
    Notes: Zusammenfassung Das Deutsche Zentralregister (DZH) für kindliche Hörstörungen hat seit 1994 Patientendatensätze von 1500 Kindern erfaßt und kann mittlerweile u.a. Aussagen und Ergebnisse zum Diagnosezeitpunkt persistierender kindlicher Hörstörungen in der Bundesrepublik Deutschland vorlegen. Nach wie vor ist das mittlere Alter bei der Diagnose persistierender kindlicher Hörstörungen sehr hoch. Das Diagnosealter korreliert stark mit dem Grad der Hörstörung, d.h. an Taubheit grenzende und hochgradige Hörstörungen werden deutlich früher diagnostiziert als leichte und mittlere. So werden leichte Hörstörungen im Durchschnitt erst mit 6;2 Jahren diagnostiziert, mittlere mit 4;4 Jahren, hochgradige mit 2;5 Jahren und an Taubheit grenzende mit 1;9 Jahren. Dies entspricht den Ergebnissen bereits vorliegender regionaler deutscher Studien [1–2]. Aus anderen europäischen Länderen sind zumindest regional deutlich frühere Diagnosezeitpunkte bekannt [3–5]. Bei 36% der im DZH erfaßten Kinder liegt zwischen dem ersten Verdacht auf Vorliegen einer persistierenden kindlichen Hörstörung und der Sicherstellung der Diagnose ein Jahr und mehr.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001060050279
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    Paper (German National Licenses)
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