This study aims to analyze BMD at spinal level L1-L4 depending on the severity of anemic state in TDBT patients. Between May 2017 and June 2018 our team studied 62 adult patients with TDBT. The mean age of the subjects was 30.7±10.0 years for women and 32±12.9 years for men with TDBT. Mean pre-transfusion Hb level was 80.4±10.6 g/l for females and 79.1±10.9g/l for males. Mean spinal BMD at the L1-L4 level, calculated as Z-score for women and men were -2.5±1.07 and -2.7±1.1 respectively. In both sexes mean pre-transfusion Hb correlated significantly with L1-L4 Z-scores. For any increase in mean pre-transfusion Hb level by 1 g/l, Z-scores at L1-L4l increased by 0.050 for males and 0.048 for females with TDBT. Pre-transfusion hemoglobin levels below the limit of 90 g/l adversely affect BMD in TDBT patients over the age of 18 years. In this sense, adherence to hypertransfusional program even when somatic growth is completed is crucial for preventing excessive bone loss. The whole article you can read on the latest issue of our journal “Rare diseases and orphan drugs” here.
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