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[Combination of a Shwachman syndrome and a complex granulocyte function disorder in a girl].

Abstract
We report the case of a 1-year-old girl with the typical symptoms of Shwachman syndrome: neutropenia, insufficiency of the exocrine pancreas, and metaphyseal dysostosis. The clinical course is complicated by recurrent infections caused by the neutropenia and an additional defect of granulocyte function. We demonstrate a severe defect of chemotactic activity as well as a disturbance of the intracellular oxidative metabolism leading to defective killing of Staphylococcus aureus and Candida albicans.
AuthorsR Dopfer, A Döring, D Niethammer
JournalHelvetica paediatrica acta (Helv Paediatr Acta) Vol. 38 Issue 4 Pg. 351-60 (Oct 1983) ISSN: 0018-022X [Print] Switzerland
Vernacular TitleKombination eines Shwachman-Syndroms mit einer komplexen Granulozytenfunktionsstörung bei einem Mädchen.
PMID6654686 (Publication Type: Case Reports, English Abstract, Journal Article, Research Support, Non-U.S. Gov't)
Topics
  • Bone Marrow Diseases (complications)
  • Chemotaxis
  • Exocrine Pancreatic Insufficiency (complications)
  • Female
  • Granulocytes (physiology, physiopathology)
  • Hematologic Diseases (complications)
  • Humans
  • Infant
  • Neutropenia (complications)
  • Phagocytosis
  • Syndrome

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