Thalassemia Major

Seventeen patients (9 men, 8 women) with thalassemia major were studied to determine the effects of the disease process on BMC and BMD (99). The average age of the subjects was 24 years. Bone density at the distal radius was measured by Compton scattering and at the distal and midradius by SPA (Norland). Cortical indices of the third metacarpal were also measured. At the distal radius, BMC was found to be 34% lower than controls and at the midradius BMC was 24% lower than controls. The metacarpal cortical indices were 36% lower in the thalassemia subjects than controls. Higher BMC and cortical indices were seen in patients who had received more blood transfusions and longer treatment with desferrioxiamine but this difference was not statistically significant.

Lasco et al. (100) studied 40 patients with P-thalassemia major to determine the effects of sex-hormone replacement on bone density. The patients were 20 men and 20 women in their late teens and early 20s. They were divided into two groups, based on serum levels of testosterone in the men and 17 P-estradiol in the women. Men and women in group 1 were hormone replete from exogenous hormone replacement. Men and women in group

2 were hormone deficient and not receiving sex-steroid replacement. A control group consisting of 20 healthy subjects was matched for age, sex, height, and weight. Bone density was measured by DXA (SOPHOS L-XR-A) at the PA lumbar spine and proximal femur. In this cross-sectional study, bone density at the PA lumbar spine was reduced in groups 1 and 2 compared to the control group. The reduction was greater in group 2 than in group 1. Proximal femoral bone density was reduced in group 2 only in comparison to the control group. The authors suggested that the treatment of hypogonadism with sex hormone replacement therapy was beneficial in the prevention and treatment of osteoporosis in thalassemics.

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