Due to the increasing survival of thalassemic patients, osteopathy is a mounting clinical problem. Low bone mass alone cannot account for the high fracture risk described; impaired bone quality has been speculated but so far it cannot be demonstrated noninvasively. We studied bone quality in thalassemia major using trabecular bone score (TBS), a novel texture measurement extracted from spine dual-energy X-ray absorptiometry (DXA), proposed in postmenopausal and secondary osteoporosis as an indirect index of microarchitecture. TBS was evaluated in 124 adult thalassemics (age range 19-56 years), followed-up with optimal transfusional and therapeutical regimens, and in 65 non-thalassemic patients (22-52 years) undergoing DXA for different bone diseases. TBS was lower in thalassemic patients (1.04 ± 0.12 [range 0.80-1.30]) versus controls (1.34 ± 0.11 [1.06-1.52]) (p < 0.001), and correlated with BMD. TBS and BMD values correlated with age, indicating that thalassemia negatively affects both bone quality and quantity, especially as the patient gets older. TBS was 1.02 ± 0.11 [0.80-1.28] in the osteoporotic thalassemic patients, 1.08 ± 0.12 [0.82-1.30] in the osteopenic ones and 1.15 ± 0.10 [0.96-1.26] in those with normal BMD. No gender differences were found (males: 1.02 ± 0.13 [0.80-1.30], females 1.05 ± 0.11 [0.80-1.30]), nor between patients with and without endocrine-metabolic disorders affecting bone metabolism. Our findings from a large population with thalassemia major show that TBS is a valuable tool to assess noninvasively bone quality, and it may be related to fragility fracture risk in thalassemic osteopathy.
Background: Acute ischemic stroke (AIS) is associated with autonomic dysfunction. We evaluated the prognostic value of heart rate variability (HRV) and the role of stroke localization and reperfusion treatment (RT) on autonomic control. Methods: Patients with AIS and sinus rhythm were enrolled in the emergency department. Autonomic parameters were recorded at the onset and after a potential RT. Neurological deficit was assessed using the National Institute of Health Stroke Scale (NIHSS) at the onset and residual disability with modified Rankin Scale (mRS) at 3 months. Two analyses were used to assess HRV. Low frequency (LF) and high frequency (HF) are, respectively, markers of sympathetic and respiratory vagal modulation in spectral analysis. Symbolic analysis provides pattern with no variation (0V%) as an index of sympathetic modulation and pattern with two like variations (2LV%) and pattern with two unlike variations (2UV%) as markers of vagal modulation. Results: We enrolled 41 patients. Twenty-seven underwent RT. A prevalent parasympathetic modulation was found in patients with NIHSS ≥14. The group with mRS 3–6 exhibited a higher 2UV% and lower 0V%. Right-sided strokes were associated with a higher respiratory vagal control. RT had no effects on HRV parameters. Conclusions: In the very early phases of AIS, a decreased 0V% and an increased 2UV% may reflect a loss of sympathetic oscillation, predicting a poorer 3 month-outcome.
Dear Editor, Poggi et al.(1) described two cases of thyroid cancer diagnosed in a population of more than 100 patients affected by β-thalassemia major (β-TM). The longer life expectancy produced by the modern treatment of this congenital anemia also had the effect of increasing the period of time for long-term complications to evolve. By analogy with liver disease, where an increased risk of hepatocellular carcinoma has emerged in recent years, the suspicion is now emerging of a possible carcinogenic role of the unavoidable iron overload in other tissues (2-4). Poggi et al.(1) focused on thyroid cancer, a malignancy with unknown prevalence in this population; the literature on this topic is sparse, except for the recent description of five cases of papillary carcinoma in the thalassemic patients followed at the Day Hospital for Thalassaemia and Haemoglobinopathies in Ferrara, Italy (5).In this scenario, we think that everyone's experience warrants consideration, and we would like to present our own experience in a tertiary outpatient clinic in Milan, Italy, where about 170 patients with β-TM have been followed. We prescribed thyroid scans, once or repeatedly, for 88 of the patients on the basis of one or more of the following indications: family history of thyroid disease, detection of thyroid or lymph-node enlargement at physical examination of the neck, functional alteration (generally hypothyroidism).Nodules were found in 31 patients; 25 lesions were smaller than 1 cm in diameter at discovery, and free of features suspicious for malignancy. These
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