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Background: Finding the correlation between criteria for low bone mineral density (BMD) in adulthood and childhood is a matter of interest. This study aimed to find how many of thalassemic patients with Z-Score ≤-2 during childhood, will be found with T-Score ≤-2.5 or Z-Score ≤-2.0 in adulthood., Methods: The results of BMDs (one in childhood and one in adulthood) of 30 patients with beta thalassemia major (12 males and 18 females) with mean age of 17.63 y/o in childhood and 20.67 y/o in adulthood were studied, retrospectively. A Norland XR-46 device was used for measuring BMD., Results: Z-Score ≤-2 was found in 33% of children and T-Score ≤-2.5 and Z-Score ≤-2.0 was found in 43 and 66% of adults, respectively. Correlation of pediatric Z-scores was good with adult T-scores and Z-scores (0.699-0.833). Relation of low BMD diagnosis in pediatric and adults was significant, and Kappa showed a moderate to good correlation between criteria for low BMD in childhood and adulthood, both with special significance in femoral region (8.10-14.47 and 0.258-0.703, respectively). Z-score ≤-2 significantly increased risk of low BMD in adulthood only when T-Score ≤-2.5 was used as low BMD criteria in adulthood (6.2-15.7). More patients dropped to poorer diagnosis group when Z-Score ≤-2.0 was used as low BMD criteria in adulthood., Conclusions: There is a moderate to good agreement of these criteria, at least in femoral region but using T-Score ≤-2.5 as low BMD criteria in adulthood caused a better prediction power for Z-Score ≤-2 of childhood.

Objectives: Beta thalassemia major is a genetic hemoglobin disorder that affects bone density. The disease leads to deteriorating bone structure but can be treated with hematopoietic stem cell transplant. We aimed to assess bone mineral density changes in pediatric beta thalassemia major patients who had undergone a hematopoietic stem cell transplant compared with similarly affected patients who had not undergone a hematopoietic stem cell transplant., Materials and Methods: Forty beta thalassemia major patients, 20 transplant and 20 nontransplant, younger than 16 years of age were enrolled. The mean age of transplant patients was 8.15 years and nontransplant patients was 9.5 years (P = .242). The female:male ratio was 1:1 in both groups. None of the patients reached puberty during this study. Bone mineral density was evaluated in transplant patients before and 1 year after hematopoietic stem cell transplant. Bone mineral density of nontransplant patients also was evaluated 1 year after their initial bone mineral density test. A Norland XR-46 densitometer was used to make all bone mineral density measurements. None of the patients had a z score < -2., Results: Mean bone mineral density changes in the femur and spine during this study were 0.008 ± 0.075 g/cm2 and 0.048 ± 0.045 g/cm2 in transplant patients and 0.045 ± 0.072 g/cm2 and 0.036 ± 0.058 g/cm2 in nontransplant patients. No significant differences between bone mineral density changes in transplant and nontransplant patients were detected during the study., Conclusions: No significant effects on bone mineral density were detected in hematopoietic stem cell transplant pediatric beta thalassemia major patients compared with similarly affected nontransplant patients. Studies of longer duration may be required to identify significant changes in bone mineral density in hematopoietic stem cell transplant patients.

Unlabelled: Low BMDs, short stature, hypogonadism, subclinical hypothyroidism, and IFG are found in 3.3, 10, 33, 16.6, 6.6, and 26.6 % of 30 pediatric β thalassemia major patients, respectively. Age is related with low Z-scores. Short stature and hypogonadism patients were older. These patients' monitoring in late childhood and early teenage for these complications is recommended., Background: Beta-thalassemia major patients frequently have low BMD and increased fracture risk. We tried to determine the relation between BMD and biochemical, transfusion, and endocrinological parameters in pediatric patients., Methods: Thirty beta-thalassemia major patients entered the study. Male to female ratio was 14/16. Physicians collected demographic; anthropometric; menstrual; transfusion and treatments histories; and serum levels of calcium, phosphorus, alkalin phosphatase, FBS, Hb, Ferritin, T3, T4, TSH, LH, FSH, testosterone (for boys), and estradiol (for girls). BMD of the spine and femur was measured using a DXA Norland XR-46 device., Results: Prevalence of "low bone density" (Z-score < -2) in the spine and femur was 3.3 and 10 %, respectively. Short stature (Z-score < -2.5) and hypogonadism was seen in 33 and 16.6 % of patients, respectively. Hypogonadism was found only in boys. Subclinical hypothyroidism and impaired fasting glucose were found in 6.6 and 26.6 % of our patients, respectively. Age and transfusion duration was positively related with spinal BMD (P values, <0.001 and <0.001, respectively), and both related negatively with spinal Z-score (P values, 0.014 and 0.043, respectively). Age and transfusion duration related positively with femoral BMD (P values 0.030 and 0.017, respectively), and both related negatively with femoral Z-score (P values, 0.015 and 0.041, respectively). Mean age of short stature and hypogonadism patients were significantly higher than those who were normal (P values, 0.007 and 0.044, respectively)., Conclusion: The main factor related with lower Z-scores of the femur and spine was age. Short stature and hypogonadism patients were significantly older. So, we recommend early monitoring of thalassemia patients (in their late childhood and early teenage) for these complications.

Background: Thalassemia major and its treatment by stem cell transplantation can have deleterious effects on bone integrity. This study assesses the adverse effects of transplantation on growing bones of pediatric thalassemic patients., Methods: Bone mineral density (BMD) of 20 patients from three thalassemia classes whose mean (SD) age was 7.4 (3.8) years were tested with a Norland XR-46 device at baseline (before transplantation), 6 and 12 months after transplantation., Results: At 6 and 12 months after transplantation we observed no significant changes in mean BMD. There were no Z-scores less than -2 among patients. Class 3 thalassemia did not negatively impact BMD. Calcium (Ca), phosphorous (P) and ferritin levels were not significantly related to patients' BMD scores. Transfusion duration and chelation therapy showed positive significant relationships to BMD (g/cm(2)), but no significant relation with the BMD Z-score. The deleterious relation between corticosteroid use and changes in BMD was not significant. In contrast, patients who developed acute graft versus host disease (aGVHD) after transplantation showed significant adverse effects on BMD of their femur (P = 0.020) and spine (P = 0.027)., Conclusion: Stem cell transplantation in pediatric thalassemic patients who do not develop aGVHD does not appear to have any significant positive or negative effects on BMD.

Objectives: In this study, we sought the proper cutoff level for quantitative ultrasonography (QUS) of the heel in identifying bone mineral density (BMD) categories as determined by dual-energy x-ray absorptiometry (DXA) in postmenopausal women., Methods: With the use of DXA, BMD categories of the lumbar spine and different areas of the left femur of 420 healthy women according to World Health Organization definitions were determined. Quantitative ultrasonography of the heel was also performed in each subject. Receiver operating characteristic curves were plotted, and sensitivity and specificity of QUS to diagnose osteoporosis were examined at different points to identify the best cutoff level. The diagnostic agreement between the two techniques in identifying osteoporosis was assessed with kappa scores., Results: The kappa scores were 0.31 for the lumbar region and 0.5 for the femoral neck region. On the receiver operating characteristic study, a score of -1 was found to be the appropriate cutoff point for QUS studies, in which the sensitivity of QUS to diagnose BMD osteoporosis varied between 78% and 87.5% depending on the site of the DXA study. With the proposed cutoff point (-1), sensitivity and specificity of QUS in detecting osteoporosis at the lumbar spine were 83.9% and 51%, respectively, and at the femoral neck were 84% and 50%, respectively., Conclusions: Insufficient agreement between QUS and DXA led to uncertainty on expected BMD in people tested by QUS. The proposed cutoff value could achieve higher sensitivity but only by accepting higher rates of false-positive results.