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.
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Authors | R Dopfer, A Döring, D Niethammer |
Journal | Helvetica paediatrica acta
(Helv Paediatr Acta)
Vol. 38
Issue 4
Pg. 351-60
(Oct 1983)
ISSN: 0018-022X [Print] Switzerland |
Vernacular Title | Kombination eines Shwachman-Syndroms mit einer komplexen Granulozytenfunktionsstörung bei einem Mädchen. |
PMID | 6654686
(Publication Type: Case Reports, English Abstract, Journal Article, Research Support, Non-U.S. Gov't)
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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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