Abstract
Because of the primary manifestations of GD are due to involvement of monocyte-derived cells, we studied functional activity of phagocytic cells from Gaucher patients GD type 1 was diagnosed in three patients (7 yr-old male/Pt.1/, 8 yr-old male/Pt.2/, 25 yr-old female /Pt.3/). Killing of S angeus by freshly isolated blood granulocytes (Gr) and monocytes (Mo), and monotyle-derived macrophages (MDM; obtained by culturing Mo for 5 d in Teflon beakeis in DMEM medium) was studied in suspensions of bacteria (5×104/ml) and phagocytic cells (5×104/ml) Incubations of phagncytie suspensions were performed in the presence of 10% human serum for 120 min, under slow rotation (4 rpm), bacterial killing was measured by colony counts after disrupting phagocytic cells in liquid nitrogen. The results shown below indicated a decreased capacity of Mo to kill S aureus, and the lack of any killing of bacteria by MDM, whereas killing of staphylococci by G1 were comparable to that of controls.
These in vitro studies provide new data to the pathophysiology of Mo and inactophages whose β-D-glucosidase activity has been reduced. Our results further elucidate host defense mechanism in patients with GD and indicate a decreased resistance to bacterial pathogens in these patients.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Marodi, L., Kúposzia, R., Shahak, H. et al. IMPAIRED MICROBICIDAL CAPACTIES OF MONOCYTES AND MACROPHAGES FROM PATIENTS WITH GAUCHER DISEASE (GD). Pediatr Res 35, 284 (1994). https://doi.org/10.1203/00006450-199402000-00177
Issue Date:
DOI: https://doi.org/10.1203/00006450-199402000-00177