Low bone mineral density and high bone turnover in adult subjects with thalassemia major : Patients and methods

38 TM patients (recruited from the thalassemia centre of Palermo) were studied (14 male and 24 female), age range 20-40 years old. Osteoporosis was defined using the standard World Health Organization criteria (z-score of BMD lower than 25). The BMD of the lumbar spine (L1-L4), the femoral neck and the forearm was determined by Dual Energy X-Ray Absorptiometry (DEXA; Lunar DPX plus).The PTH was studied by ELISA (Biosource, Belgium), osteocalcin, C-telopeptide and bone alkaline phosphatase by Elisa (Beckmann-Coulter USA). Analisys of variance and U test of Mann-Whitney were used for TM patients and controls. The correlation was obtained with Pearson index. P value < 0.05 was considered statistically sig¬nificant. The calculated z-scores for males and females were evaluated by normal ranges.

Low bone mineral density and high bone turnover in adult subjects with thalassemia major

Introduction

Beta Thalassemia Major (TM) affects a significant number of the population in certain areas of the world. TM is an inherited blood disorder in which the body is unable to make adequate hemoglobin. This is due to an inborn error of metabolism that leads to absence or reduced synthesis of one or more types of globin polypeptide chains of the hemoglobin molecule; is a hereditary disorder of haemoglobin synthesis re­sulting in severe anemia.