Skip Navigation LinksHome > May 2010 - Volume 32 - Issue 4 > Iron Chelation Therapy in Upper Egyptian Transfusion-depende...
Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e3181d419d6
Original Articles

Iron Chelation Therapy in Upper Egyptian Transfusion-dependent Pediatric Homozygous β-Thalassemia Major: Impact on Serum L-Carnitine/Free Fatty Acids, Osteoprotegerin/The Soluble Receptor Activator of Nuclear Factor-κβ Ligand Systems, and Bone Mineral Density

Hamed, Enas A. MD*; Mohamed, Nagwa A. MD; EL-Metwally, Tarek H. PhD; Kamal, Manal M. MD*

Collapse Box

Abstract

Bone disease in β-thalassemia major (βTM) remains poorly understood. Receptor activator of nuclear factor-κβ ligand (RANKL) regulates osteoclast formation and function. RANKL activity is balanced by interaction with its receptor (RANK) and binding to osteoprotegerin (OPG). L-Carnitine (LC) enhances osteoblastic activity by furnishing fuel. This study hypothesized that abnormal bone metabolism in βTM involves imbalanced RANKL/OPG and LC/free fatty acids (FFAs) metabolism. Sixty-nine transfusion-dependent βTM patients and 15 healthy controls were enrolled. One group of patients (n=34) received desferrioxamine (DFO) and the other (n=35) did not. Serum OPG, soluble RANKL (sRANKL), FFAs, LC [total LC (TC), free LC (FC), and esterified LC (EC)], calcium, and inorganic phosphate were measured by specific immuno and colorimetric assays; bone mineral density was examined by dual x-ray absorptiometry. Patients showed lower levels of OPG, TC, FC, EC and higher levels of sRANKL, sRANKL/OPG ratio, and FFAs than controls. Patients on DFO showed lower levels of OPG, TC, FC and higher levels of sRANKL, sRANKL/OPG ratio, and FFAs than those without chelation. In patients, sRANKL correlated negatively with TC and OPG and FC correlated positively with OPG and negatively with sRANKL, sRANKL/OPG ratio, and FFAs. In conclusion, altered bone metabolism owing to imbalanced osteoclastic bone resorption versus constructive osteoblastic activities in βTM pediatric patients could be due to abnormal sRANKL-OPG and LC-FFAs systems that were worsened by DFO.

© 2010 Lippincott Williams & Wilkins, Inc.

Login

Article Tools

Share

Search for Similar Articles
You may search for similar articles that contain these same keywords or you may modify the keyword list to augment your search.

Connect With Us

Twitter
twitter.com/JPHOonline

For additional oncology content, visit LWW Oncology Journals on Facebook.