An Effect of Pachydermoperiostosis Patients' Serum onthe Proliferation of Fibroblasts

Moon, Hong Joo; Kim, Seong Jin; Lee, Seung Chul; Chun, Inn Ki; Won, Young Ho

Abstract

BACKGROUND: Pachydermoperiostosis(PDP) is a rare genetic disease characterized by pachydermia, periostosis, arthralgia and finger clubbing. The pathogenesis of this disease is still unknown, but the concept that platelets and endothelial cells may play a major role in the developement of pachydermia is widely accepted nowadays, It is also suspected that several serum growth factors stimulate proliferation of soft tissue.
OBJECTIVE
The purpose of this study was to investigate the pathogenesis of pachydermia in patients with pachydermoperiostosis through evaluating whether the fibroblasts from these patients have a higher proliferation rate than those from controls or whether the proliferation rate of those cells are affected by certain serum growth factors. METHOD: At first, we evaluated the proliferation rate of fibroblasts from patients and corntrols by the MTT colorimetric assay, and then the proliferation rate of fibroblasts from the prepuce of newborn infants under several conditions of media containing uncentrifuged patients serum, centrifuged patients serum, uncentrifuged control serum, or centrifuged control serum.
RESULTS
The proliferation of fibroblasts from patients skin was slower than the control fibroblasts and fibroblasts derived from uninvolved skin of patients. The statistically significant highest proliferation rate was observed when fibroblasts were cultured in the uncentrifuged patients serum contained media and the order of proliferation was as follows: centrifuged patients serum, uncentrifuged control serum and centrifuged control serum condition at 20%, 10%, and 1% respectively.
CONCLUSION
These results suggest that patients fibroblasts do not proliferate in vitro at a higher rate than control firoblasts. Fibroblasts in PDP may only play a role as target cells and certain serum factors are responsible for the pathogenesis of PDP.