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Colony Forming Cell (CFC) Assay for Human Hematopoietic Cells
Published on: December 19, 2010
Granulocyte colony-stimulating factor-responsive chronic neutropenia in cartilage-hair hypoplasia
Roland A Ammann1, Andrea Duppenthaler, Juergen Bux
1Department of Pediatrics, University of Bern, Bern, Switzerland.
Abstract:
: Chronic neutropenia is common in children with cartilage-hair hypoplasia (CHH). The authors describe a 6-year-old with severe CHH, moderate neutropenia associated with serum IgG antibodies directed against Fcgamma-RIIIb (NA1/2), and frequent bacterial infections. In this patient, long-term administration of granulocyte colony-stimulating factor increased peripheral neutrophil counts and prevented recurrent hospitalizations for bacterial lower respiratory tract infections.
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