Advertisement

Zusammenfassung

Durch einen autosomal-rezessiv vererbten Defekt der Galaktose-1 -phosphat-uridyl-transferase (Häufigkeit: 1:35000/Geburten) kommt es zu einer Galaktose-1 -phosphat Kumulation mit toxischen Gehirn-, Leber-, Nieren- und Darmschädigungen.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Literatur

  1. 1.
    Brown BB, Watson PD, Taussig LM (1975) Congenital metabolic diseases of pediatric patients: anesthetic implications. Anesthesiology 43: 197PubMedCrossRefGoogle Scholar
  2. 2.
    Niessen KH (1987) Pädiatrie. VCH-Verlagsge-sellschaft, Weinheim, S 190Google Scholar
  3. 1.
    Brown BB, Watson PD, Taussig LM (1975) Congenital metabolic diseases of pediatric patients: anesthetic implications. Anesthesiology 43: 197PubMedCrossRefGoogle Scholar
  4. 2.
    Jones AEP, Pelton DA (1976) An index of syndromes and their anaesthetic implications. Can Anaesth Soc J 23: 206CrossRefGoogle Scholar
  5. 3.
    Niessen KH (1987) Pädiatrie. VCH-Verlagsge-sellschaft, Weinheim, S 254Google Scholar
  6. 4.
    Steward DJ (1985) Manual of Pediatric Anesthesia. 2nd edition Churchill Livingstone, New York Edinburgh London, p 304Google Scholar
  7. 1.
    Leichtling JJ (1971) Gardner’s syndrome. Mt Sinai J Med NY 38: 311Google Scholar
  8. 2.
    Jones EL et al. (1966) Gardner’s syndrome: Review of the literature and report on a family. Arch Surg 92: 287PubMedGoogle Scholar
  9. 3.
    Stauch GW et al. (1973) Das Gardner-Syndrom. Fortschr Röntgenstr 118: 603CrossRefGoogle Scholar
  10. 1.
    Coleman P (1984) McArdle’s disease. Problems of anaesthetic management for caesarean section. Anaesthesia 39: 784PubMedCrossRefGoogle Scholar
  11. 2.
    Cox JM (1968) Anesthesia and glycogen-storage disease. Anesthesiology 29: 1221PubMedCrossRefGoogle Scholar
  12. 3.
    Edelstein G, Hirschman CA (1980) Hyperthermia and ketoacidosis during anesthesia in a child with glycogen-storage disease. Anesthesiology 32: 90CrossRefGoogle Scholar
  13. 4.
    Grunfeld JP, Ganeval D, Chanard J et al (1972) Acute renal failure in McArdle’s disease. N Engl J Med 286: 1237PubMedCrossRefGoogle Scholar
  14. 5.
    Katz J, Steward D (1987) Anesthesia and uncommon pediatric diseases. WB Saunders, Philadelphia London Toronto, p 347Google Scholar
  15. 6.
    Stanbury JB, Wyngaarden JB, Fredrickson DS (1978) The Metabolic Basis of Inherited Disease. 4th edition. McGraw-Hill, New York, p 29Google Scholar
  16. 7.
    Steward DJ (1985) Manual of Pediatric Anesthesia. Appendix 1: Anesthetic implications of syndromes and unusual disorders. Churchill Livingstone, New York, p 289Google Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1989

Authors and Affiliations

  • Manfred Abel
    • 1
  1. 1.Anästhesie-AbteilungKinderkrankenhaus der Stadt KölnKöln 60Deutschland

Personalised recommendations