Evaluation of ProcollagenI N-Terminal Pro-Peptide As A Marker of Bone Remodeling in Beta Thalassemia Pediatric Patients

Abstract

T he management of patients with thalassemia has improved markedly over the past few decades with the use of optimized transfusion programs and chelating therapy. There has been a considerable improvement in the life expectancy and the quality of life of these patients. With prolongation in the life expectancy, it has been observed that this hemoglobinopathy is associated with a variety of bone disorders like deformities, bone pains, delayed bone age, growth failure, rickets, scoliosis, spinal deformities, nerve compression, pathologic fractures, osteopenia, and osteoporosis. High-dose iron chelating therapy may also contribute to osteopenia and osteoporosis. Osteoporosis is a significant cause of morbidity in these patients. It is characterized by low bone mass and disruption of bone architecture, resulting in reduced bone strength and increased risk of fractures. Dual Energy X-Ray Absorptiometry (DEXA) of lumbar spine and femoral neck is considered a very reliable and noninvasive technique to assess the degree of osteoporosis and total bone mass. The amino-terminal pro-peptide of type I procollagen (P1NP) is a recently introduced biochemical turnover marker (BTM) marker that is considered the most sensitive index of bone formation in patients with bone disease of varying origins.