Alterations of panoramic radiomorphometric indices in children and adolescents with beta-thalassemia major: A fractal analysis study.

BACKGROUND: Beta-thalassemia major is an inherited disorder that can cause bone deformity and loss of bone mineral density. The objective of this study is to evaluate the cortical and trabecular mandibular bone morphology of children and adolescents who have beta-thalassemia major (ß-TM) using a fractal dimension (FD) analysis and different panoramic radiomorphometric indices with digital panoramic radiographic images (DPRIs). MATERIAL AND METHODS: The study included 80 patients (with 40 patients each of ß-TM and control). The mandibular cortical width (MCW), panoramic mandibular index (PMI), mandibular cortical index (MCI), and simple visual estimation (SVE) were evaluated, and an FD analysis of five regions of interest (ROIs) (ROI 1: in basal cortical bone; ROI 2: in premolar region; ROI 3: in molar region; ROI 4: in angulus mandible and ROI 5: in condyle region) was obtained in all DPRIs. Quantitative variables were analyzed using the student's t-test , Kruskal-Wallis and Mann-Whitney U tests. RESULTS: When the ß-TM groups were compared with controls, there were no statistically significant differences found in the mean FD values, the ROIs of the trabecular bone, or the SVE. There was a significant correlation in the mean MCW, PMI, ROI of cortical bone (ROI 1), and MCI between ß-TM and control groups (p < 0.001, p < 0.001, p = 0.047, and p = 0.046, respectively). The mean MCW values correlated with the SVE in both the ß-TM and control groups (p = 0.031 and p < 0.001, respectively). While the mean MCW values correlated with the MCI (p = 0.04) in the control group, the mean MCW values were not correlated with the MCI (p = 0.493) in ß-TM group. CONCLUSIONS: The current study revealed lower MCW and PMI values in the ß-TM group. While the mean FD values of trabecular bone is similar to the control groups, the mean FD value is lower in cortical bone in the ß-TM group. MCW, PMI, FD of cortical bone and MCI may be key indicators in individuals with beta-thalassemia major.

Screening first-degree relatives of patients with idiopathic dilated cardiomyopathy.

BACKGROUND: This study evaluated whether subclinical myocardial dysfunction occurs in first-degree relatives of patients with idiopathic dilated cardiomyopathy (IDCM), using strain echocardiographic imaging, before apparent left ventricular (LV) failure is observed. PATIENTS AND METHODS: The study comprised 77 subjects aged 16-63 years who had first-degree relatives with a previous or new diagnosis of IDCM. LV myocardial deformation parameters of the first-degree relatives with normal LVEF (≥55%) values, as assessed using 2D echocardiography, were evaluated. The findings of the first-degree relatives were compared with an age- and sex-matched control group (n = 86). RESULTS: No difference in terms of age, gender, and body surface area was detected between first-degree relatives and controls. First-degree relatives of IDCM patients had significantly lower LVEF (62.04 ± 5.8% vs. 65.65 ± 6.3%, p < 0.001) and FS values (39.4 ± 6.6 vs. 41.45 ± 5.5, p = 0.03) compared with the controls. Assessment of LV deformation parameters revealed that LV global longitudinal strain (-17.34 ± 2.19% vs. -19.21 ± 2.16%, p < 0.001) and strain rate (0.94 ± 0.14 s-1 vs. 1.03 ± 0.14 s-1, p < 0.001), radial strain (34.47 ± 9.14% vs 42.79 ± 11.91%, p < 0.001) and strain rate (1.6 ± 0.38 s-1 vs. 1.75 ± 0.29 s-1, p = 0.006), circumferential strain (-6.07 ± 2.83% vs. -18.29 ± 3.39%, p < 0.001) and strain rate (1.09 ± 0.24 s-1 vs. 1.2 ± 0.25 s-1, p = 0.004), and torsion (10.07 ± 5.18o/cm vs. 12.42 ± 5.78o/cm, p = 0.009) were significantly reduced in first-degree relatives compared with controls. CONCLUSION: LV deformation parameters are impaired in first-degree relatives of patients with IDCM. Screening of this population using standard 2D echocardiography and strain imaging may provide early detection of those with subclinical myocardial dysfunction.